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Dermatopathology
Volume 8
Issue 3
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Dermatopathology, Volume 8, Issue 3 (September 2021) – 20 articles

Cover Story (view full-size image): This review presents the clinical, pathological, and radiological features of the most common congenital cystic lesions of the neck, emphasizing their embryologic origin, the differential diagnosis, and the possible association with chromosomal defects. These lesions vary in prevalence from common (thyroglossal duct cysts, branchial cleft cysts, and lymphangioma) to very rare (thymic cysts and cervical bronchogenic cysts).The age of the child (infant or child) and the localization of the mass (median, lateral, or parotid) are extremely important elements of orientation. Most of the time, clinical examination and US are sufficient for clear identification and correct treatment of the cervical cyst. Large predominance of benign cystic lesions does not rule out the possibility of any rare malignancies associated with a cystic presentation. View this paper.
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4 pages, 1795 KiB  
Case Report
Eccrine Nevus in the Forearm of a 16-Year-Old Presenting as Unilateral Hyperhidrosis: A Clinicopathological Correlation Paradigm
by Alejandro Martin-Gorgojo, Ignacio Sanchez-Carpintero, Ricardo Ruiz-Rodriguez and Ana-Belen Enguita-Valls
Dermatopathology 2021, 8(3), 446-449; https://doi.org/10.3390/dermatopathology8030047 - 18 Sep 2021
Cited by 2 | Viewed by 4975
Abstract
A case of a purely eccrine nevus in an adolescent patient presenting with focal hyperhidrosis on an area comprising the left forearm and the dorsal aspect of the left hand is described. No clinically evident lesions were identifiable. Dermatopathologic findings were subtle, showing [...] Read more.
A case of a purely eccrine nevus in an adolescent patient presenting with focal hyperhidrosis on an area comprising the left forearm and the dorsal aspect of the left hand is described. No clinically evident lesions were identifiable. Dermatopathologic findings were subtle, showing only a slight increase in the number of eccrine glands. Clinicopathological correlation was paramount to achieve the diagnosis. Full article
(This article belongs to the Section Clinico-Pathological Correlation in Dermatopathology)
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Figure 1

Figure 1
<p>(<b>a</b>): Dorsal aspect of the patient’s left forearm, showing no clinically evident abnormalities. (<b>b</b>): Iodine-starch test (Minor’s test) highlighting the hyperhidrosis area.</p> Full article ">Figure 2
<p>Punch biopsy sample, hematoxylin-eosin staining. (<b>a</b>): 10× magnification. (<b>b</b>): 40× magnification.</p> Full article ">
4 pages, 527 KiB  
Case Report
A New Case of Hybrid Epidermoid and Apocrine Cyst
by Fulvia Serra and Gürkan Kaya
Dermatopathology 2021, 8(3), 442-445; https://doi.org/10.3390/dermatopathology8030046 - 1 Sep 2021
Cited by 2 | Viewed by 3271
Abstract
We described a new case of a hybrid epidermoid and apocrine cyst, known to be a rare histopathological entity. The cyst was located in the axillary region and completely excised, without complication. The diagnosis was made at the histological analysis, where we found [...] Read more.
We described a new case of a hybrid epidermoid and apocrine cyst, known to be a rare histopathological entity. The cyst was located in the axillary region and completely excised, without complication. The diagnosis was made at the histological analysis, where we found a cystic lesion in the dermis, lined with both epidermoid and apocrine epithelium. Full article
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<p>Histology. (<b>A</b>) HE ×10. The whole cyst. (<b>B</b>) HE Magnification ×20 (inset). Transition between epidermoid and apocrine epithelium. (<b>C</b>). HE ×40 Magnification. The apocrine epithelium.</p> Full article ">
16 pages, 3195 KiB  
Article
Immunohistopathological Analysis of Immunoglobulin E-Positive Epidermal Dendritic Cells with House Dust Mite Antigens in Naturally Occurring Skin Lesions of Adult and Elderly Patients with Atopic Dermatitis
by Ryoji Tanei and Yasuko Hasegawa
Dermatopathology 2021, 8(3), 426-441; https://doi.org/10.3390/dermatopathology8030045 - 1 Sep 2021
Cited by 4 | Viewed by 3695
Abstract
The immunopathogenic role of house dust mite (HDM) allergens in the development of skin lesions in atopic dermatitis (AD) has not yet been precisely clarified. We immunohistopathologically evaluated the localization of immunoglobulin E (IgE)-positive epidermal dendritic cells with HDM antigens in the skin [...] Read more.
The immunopathogenic role of house dust mite (HDM) allergens in the development of skin lesions in atopic dermatitis (AD) has not yet been precisely clarified. We immunohistopathologically evaluated the localization of immunoglobulin E (IgE)-positive epidermal dendritic cells with HDM antigens in the skin lesions of patients with IgE-allergic AD. Using double-immunofluorescence and single-immunochemical staining methods, we analyzed biopsy specimens from the skin lesions of six patients with IgE-allergic AD and HDM allergy and 11 control subjects with inflammatory skin disorders. Inflammatory dendritic epidermal cells (IDECs; CD11c+ and CD206+ cells) were markedly observed in the central area of the spongiotic epidermis of skin lesions in all AD patients. Furthermore, IgE-positive IDECs with HDM antigens in the central areas of the spongiosis were found in four of the six (66.7%) AD patients. Langerhans cells (LCs; CD207+ cells) with HDM antigens were also observed in the peripheral areas of the spongiosis. Infiltration of CD4+ and CD8+ T cells in association with IgE-positive IDECs and LCs with HDM antigens was seen in the spongiotic epidermis. An IgE-mediated delayed-type hypersensitivity reaction, in combination with IgE-bearing dendritic cells, specific T cells, keratinocytes, and HDM antigens, may lead to spongiotic tissue formation in eczematous dermatitis in AD. Full article
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Figure 1
<p>Routine and double-immunofluorescence staining for active lesions of chronic AD in patients with IgE-allergic AD and HDM allergy. (<b>a</b>) Chronic eczematous reactions with a scaly crust, epidermal hyperplasia, and obvious spongiosis with mononuclear cell infiltration were observed in the epidermis. (<b>b</b>) Double-positive IgE+ CD206+ cells (yellow) markedly infiltrated into the spongiotic epidermis of the active lesions of chronic AD. (<b>c</b>) Double-positive IgE+ Der f1+ cells (yellow) were observed among the infiltrating cells in the same spongiotic epidermis. (<b>d</b>) Double-positive CD206+ Der f1+ cells (yellow) were also found among the infiltrating cells in the same spongiotic epidermis. (<b>e</b>) Double-positive IgE+ CD206+ cells (yellow) were observed in the spongiotic epidermis of active lesions of chronic AD. (<b>f</b>) Double-positive CD206+ Der f1+ cells (yellow) were found among the infiltrating cells in the same spongiotic epidermis. (<b>g</b>) Both double-positive CD3+ CD8+ cells (yellow) and single-positive CD3+ CD8−cells (green) were also observed in the spongiotic epidermis. (<b>h</b>) CD207+ cells (red) were less localized to the central area, and some double-positive CD207+ Der f1+ cells (yellow) were present in the peripheral area of the spongiotic epidermis. (<b>a</b>): Case 1; hematoxylin-eosin staining. (<b>b</b>–<b>d</b>,<b>g</b>,<b>h</b>): Case 1; double-immunofluorescence staining. (<b>e</b>,<b>f</b>): Case 6; double-immunofluorescence staining. In the double-immunofluorescence staining, nuclei were labeled with 4′,6-diamidino-2-phenylindole (DAPI; blue). Sets of figures, i.e., (<b>b</b>–<b>h</b>), represent serial sections. Original magnifications: 100×, (<b>a</b>); 200×, (<b>b</b>–<b>h</b>).</p> Full article ">Figure 1 Cont.
<p>Routine and double-immunofluorescence staining for active lesions of chronic AD in patients with IgE-allergic AD and HDM allergy. (<b>a</b>) Chronic eczematous reactions with a scaly crust, epidermal hyperplasia, and obvious spongiosis with mononuclear cell infiltration were observed in the epidermis. (<b>b</b>) Double-positive IgE+ CD206+ cells (yellow) markedly infiltrated into the spongiotic epidermis of the active lesions of chronic AD. (<b>c</b>) Double-positive IgE+ Der f1+ cells (yellow) were observed among the infiltrating cells in the same spongiotic epidermis. (<b>d</b>) Double-positive CD206+ Der f1+ cells (yellow) were also found among the infiltrating cells in the same spongiotic epidermis. (<b>e</b>) Double-positive IgE+ CD206+ cells (yellow) were observed in the spongiotic epidermis of active lesions of chronic AD. (<b>f</b>) Double-positive CD206+ Der f1+ cells (yellow) were found among the infiltrating cells in the same spongiotic epidermis. (<b>g</b>) Both double-positive CD3+ CD8+ cells (yellow) and single-positive CD3+ CD8−cells (green) were also observed in the spongiotic epidermis. (<b>h</b>) CD207+ cells (red) were less localized to the central area, and some double-positive CD207+ Der f1+ cells (yellow) were present in the peripheral area of the spongiotic epidermis. (<b>a</b>): Case 1; hematoxylin-eosin staining. (<b>b</b>–<b>d</b>,<b>g</b>,<b>h</b>): Case 1; double-immunofluorescence staining. (<b>e</b>,<b>f</b>): Case 6; double-immunofluorescence staining. In the double-immunofluorescence staining, nuclei were labeled with 4′,6-diamidino-2-phenylindole (DAPI; blue). Sets of figures, i.e., (<b>b</b>–<b>h</b>), represent serial sections. Original magnifications: 100×, (<b>a</b>); 200×, (<b>b</b>–<b>h</b>).</p> Full article ">Figure 2
<p>Double-immunofluorescence staining for active lesions of chronic AD in patients with IgE-allergic AD and HDM allergy. (<b>a</b>) Double-positive IgE+ CD11c+ cells (yellow) markedly infiltrated into the spongiotic epidermis of active lesions of chronic AD. (<b>b</b>) Double-positive IgE+ CD206+ cells (yellow) were also observed in the spongiotic epidermis. Note: almost all CD206+ cells in the spongiotic epidermis were IgE-positive. (<b>c</b>) Double-positive IgE+ Mite Extract antigen+ cells (yellow) were observed among the infiltrating cells in the same spongiotic epidermis. (<b>d</b>) Double-positive CD206+ Mite Extract antigen+ cells (yellow) were also observed among the infiltrating cells in the same spongiotic epidermis. (<b>a</b>–<b>d</b>): Case 2; double-immunofluorescence staining. Sets of (<b>a</b>–<b>d</b>) represent serial sections. Original magnifications: 200×, (<b>a</b>–<b>d</b>).</p> Full article ">Figure 3
<p>Double-immunofluorescence staining for skin lesions in control subjects. (<b>a</b>) In a control case of non-eczematous inflammatory skin disorders and serum hyper-IgE (bullous pemphigoid), double-positive IgE+ CD11c+ cells (yellow) were seen in small numbers among the infiltrated cells in the upper dermis, and they were scarcely detected in the epidermis. (<b>b</b>) In the same control case, double-positive IgE+ Der f1+ cells were not observed in the epidermis or the upper dermis. Note: in (<b>a</b>,<b>b</b>), linear depositions of IgE at the basal layer (red) were characteristically observed. (<b>c</b>) In a control case of inflammatory skin disorders and spongiotic tissue within the epidermis (nummular eczema), single-positive IgE-CD206+ cells (green) were observed in the spongiotic epidermis. (<b>d</b>) In the same control case, infiltrating CD206+ cells were scarcely detected with Mite Extract antigens in the spongiotic epidermis. (<b>a</b>,<b>b</b>): Case 11. (<b>c</b>,<b>d</b>): Case 13. Note: in (<b>a</b>,<b>b</b>), the single-staining images of DAPI were omitted from the figures. Sets of figures, i.e., (<b>a</b>–<b>d</b>), represent serial sections. Original magnifications: 200×, (<b>a</b>–<b>d</b>).</p> Full article ">Figure 4
<p>Routine and single-immunohistochemical staining for active lesions of chronic AD in a patient with IgE-allergic AD and HDM allergy. (<b>a</b>) An early lesion of spongiosis with the infiltration of mononuclear cells in the epidermis. (<b>b</b>) CD207+ cells were mainly seen in the peripheral area of the spongiosis. (<b>c</b>) Infiltrating IgE+ cells were observed to aggregate in the central area of the spongiosis. (<b>d</b>) Infiltrating CD11c+ cells that show similar localization as the infiltrating IgE+ cells were observed in the central area of the spongiosis. (<b>e</b>) Infiltrating CD4+ cells that showed similar localization as the infiltrating IgE+ cells and CD11c+ cells were observed in the central area of the spongiosis. (<b>f</b>) Infiltrating CD8+ cells were mainly seen in the peripheral area of the spongiosis. (<b>a</b>): Case 1; hematoxylin-eosin staining. (<b>b</b>–<b>f</b>): Case 1; single-immunohistochemical staining. Sets of (<b>a</b>–<b>f</b>) represent serial sections. Original magnification: 200×, (<b>a</b>–<b>f</b>).</p> Full article ">
8 pages, 1172 KiB  
Review
Artificial Intelligence in Dermatopathology: New Insights and Perspectives
by Gerardo Cazzato, Anna Colagrande, Antonietta Cimmino, Francesca Arezzo, Vera Loizzi, Concetta Caporusso, Marco Marangio, Caterina Foti, Paolo Romita, Lucia Lospalluti, Francesco Mazzotta, Sebastiano Cicco, Gennaro Cormio, Teresa Lettini, Leonardo Resta, Angelo Vacca and Giuseppe Ingravallo
Dermatopathology 2021, 8(3), 418-425; https://doi.org/10.3390/dermatopathology8030044 - 1 Sep 2021
Cited by 23 | Viewed by 4815
Abstract
In recent years, an increasing enthusiasm has been observed towards artificial intelligence and machine learning, involving different areas of medicine. Among these, although still in the embryonic stage, the dermatopathological field has also been partially involved, with the attempt to develop and train [...] Read more.
In recent years, an increasing enthusiasm has been observed towards artificial intelligence and machine learning, involving different areas of medicine. Among these, although still in the embryonic stage, the dermatopathological field has also been partially involved, with the attempt to develop and train algorithms that could assist the pathologist in the differential diagnosis of complex melanocytic lesions. In this article, we face this new challenge of the modern era, carry out a review of the literature regarding the state of the art and try to determine promising future perspectives. Full article
(This article belongs to the Section Artificial Intelligence in Dermatopathology)
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Figure 1

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<p>Literature search and article selection.</p> Full article ">Figure 2
<p>Examples of images of lesions used in various studies in the literature. (<b>A</b>) Basal cell carcinoma, superficial variant (hematoxylin–eosin, original magnification: 4×). (<b>B</b>) Seborrheic keratosis, hyperkeratotic variant (hematoxylin–eosin, original magnification: 10×). (<b>C</b>) Squamous cell carcinoma (hematoxylin–eosin, original magnification: 20×). (<b>D</b>) Intradermic nevus (hematoxylin–eosin, original magnification: 10×). (<b>E</b>) Amelanotic malignant melanoma (hematoxylin–eosin, original magnification: 20×).</p> Full article ">Figure 2 Cont.
<p>Examples of images of lesions used in various studies in the literature. (<b>A</b>) Basal cell carcinoma, superficial variant (hematoxylin–eosin, original magnification: 4×). (<b>B</b>) Seborrheic keratosis, hyperkeratotic variant (hematoxylin–eosin, original magnification: 10×). (<b>C</b>) Squamous cell carcinoma (hematoxylin–eosin, original magnification: 20×). (<b>D</b>) Intradermic nevus (hematoxylin–eosin, original magnification: 10×). (<b>E</b>) Amelanotic malignant melanoma (hematoxylin–eosin, original magnification: 20×).</p> Full article ">
28 pages, 66904 KiB  
Article
What to Look Out for in a Newborn with Multiple Papulonodular Skin Lesions at Birth
by Sylvie Fraitag and Olivia Boccara
Dermatopathology 2021, 8(3), 390-417; https://doi.org/10.3390/dermatopathology8030043 - 17 Aug 2021
Cited by 6 | Viewed by 7964
Abstract
Multiple papulonodular skin lesions at birth can indicate the presence of various benign and malignant disorders. Although the lesions’ clinical aspect (color and consistency, in particular) may steer the clinician towards one disorder or another (infantile myofibromatosis, xanthogranuloma, or metastatic neuroblastoma), the diagnosis [...] Read more.
Multiple papulonodular skin lesions at birth can indicate the presence of various benign and malignant disorders. Although the lesions’ clinical aspect (color and consistency, in particular) may steer the clinician towards one disorder or another (infantile myofibromatosis, xanthogranuloma, or metastatic neuroblastoma), the diagnosis can only be confirmed by the histopathologic assessment of a biopsy. In neonates, a rapid but accurate diagnosis is critical because skin lesions may be the first manifestation of a malignant disorder like leukemia cutis or metastatic neuroblastoma. Here, we review the various disorders that may manifest themselves as multiple skin lesions at birth. Full article
(This article belongs to the Special Issue New Insights in Pediatric Dermatopathology)
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Figure 1

Figure 1
<p>Blueberry muffin rash.</p> Full article ">Figure 2
<p>Congenital syphilis. (<b>a</b>) Multiple small erythematous lesions (by courtesy S Mallet). (<b>b</b>) 4× superficial and deep cellular infiltrate with ulcerated overlying epidermis (by courtesy N Macagno). (<b>c</b>) 25× polymorphic infiltrate containing plasma cells. (<b>d</b>) Positivity of anti-treponema showing numerous spirochetes between cells.</p> Full article ">Figure 2 Cont.
<p>Congenital syphilis. (<b>a</b>) Multiple small erythematous lesions (by courtesy S Mallet). (<b>b</b>) 4× superficial and deep cellular infiltrate with ulcerated overlying epidermis (by courtesy N Macagno). (<b>c</b>) 25× polymorphic infiltrate containing plasma cells. (<b>d</b>) Positivity of anti-treponema showing numerous spirochetes between cells.</p> Full article ">Figure 3
<p>Dermal erythropoiesis (<b>a</b>,<b>b</b>) 2.5× and 25× clusters of erythroblasts in the dermis. They are highlighted by anti-glycophorin antibody (<b>c</b>).</p> Full article ">Figure 3 Cont.
<p>Dermal erythropoiesis (<b>a</b>,<b>b</b>) 2.5× and 25× clusters of erythroblasts in the dermis. They are highlighted by anti-glycophorin antibody (<b>c</b>).</p> Full article ">Figure 4
<p>Congenital leukemia cutis: (<b>a</b>) blueberry muffin rash; (<b>b</b>) 4× dense dermal infiltration separated from the epidermis by a grenz-zone; (<b>c</b>) 10× medium-sized blastic cells arranged in single-file between collagen bundles and with a peri-vascular and peri-adnexal arrangement as well; (<b>d</b>) 25× apoptotic cells and mitotic figures; (<b>e</b>) CD68 intense positivity; (<b>f</b>) Ki67 immunostaining: almost 100% of nuclei are positive.</p> Full article ">Figure 4 Cont.
<p>Congenital leukemia cutis: (<b>a</b>) blueberry muffin rash; (<b>b</b>) 4× dense dermal infiltration separated from the epidermis by a grenz-zone; (<b>c</b>) 10× medium-sized blastic cells arranged in single-file between collagen bundles and with a peri-vascular and peri-adnexal arrangement as well; (<b>d</b>) 25× apoptotic cells and mitotic figures; (<b>e</b>) CD68 intense positivity; (<b>f</b>) Ki67 immunostaining: almost 100% of nuclei are positive.</p> Full article ">Figure 5
<p>Metastatic neuroblastoma. (<b>a</b>) Pink lesion surrounded by a whitish halo. (<b>b</b>) 2.5× tumoral nodules in the reticular dermis and the subcutis. (<b>c</b>) 25× blue round cells. (<b>d</b>) Anti-synaptophysin immunostaining.</p> Full article ">Figure 6
<p>40× Rhaboid tumor. Very atypical large cells.</p> Full article ">Figure 7
<p>Langerhans cell histiocytosis (<b>a</b>) disseminated congenital lesions with erosive and purpuric lesions. (<b>b</b>) nodular and crusted lesion (Hashimoto-Pritzker). (<b>c</b>) 2.5× usual aspect of LCH with Langerhans cell infiltrate filling the papillary dermis. (<b>d</b>) 2.5× anti-CD1a. (<b>e</b>) 2.5× Nodular spontaneously regressive lesion. Dense infiltrate throughout the dermis with very few epidermotropism.</p> Full article ">Figure 7 Cont.
<p>Langerhans cell histiocytosis (<b>a</b>) disseminated congenital lesions with erosive and purpuric lesions. (<b>b</b>) nodular and crusted lesion (Hashimoto-Pritzker). (<b>c</b>) 2.5× usual aspect of LCH with Langerhans cell infiltrate filling the papillary dermis. (<b>d</b>) 2.5× anti-CD1a. (<b>e</b>) 2.5× Nodular spontaneously regressive lesion. Dense infiltrate throughout the dermis with very few epidermotropism.</p> Full article ">Figure 8
<p>Juvenile xanthogranuloma. (<b>a</b>) Presenting as blueberry muffin rash (by courtesy E Puzenat); (<b>b</b>) multiple yellowish lesions. (<b>c</b>) 4× rather dense infiltrate in the dermis extending into the superficial subcutis. (<b>d</b>) 10× non xanthomized histiocytes with no giant cells (mononuclear-vacuolated early variant). (<b>e</b>) 25× Histiocytes are accompanied by inflammatory cells, in particular here, neutrophils. (<b>f</b>) Anti-Factor XIIIa positivity.</p> Full article ">Figure 8 Cont.
<p>Juvenile xanthogranuloma. (<b>a</b>) Presenting as blueberry muffin rash (by courtesy E Puzenat); (<b>b</b>) multiple yellowish lesions. (<b>c</b>) 4× rather dense infiltrate in the dermis extending into the superficial subcutis. (<b>d</b>) 10× non xanthomized histiocytes with no giant cells (mononuclear-vacuolated early variant). (<b>e</b>) 25× Histiocytes are accompanied by inflammatory cells, in particular here, neutrophils. (<b>f</b>) Anti-Factor XIIIa positivity.</p> Full article ">Figure 9
<p>Metastatic choriocarcinoma. (<b>a</b>) 4× cellular and hemorrhagic areas in the reticular dermis arranged in lobules. (<b>b</b>) 25× very large typical cells of syncytiotrophoblast.</p> Full article ">Figure 10
<p>Infantile myofibromatosis. (<b>a</b>) This baby presented with multiple small blueish lesions. (<b>b</b>) 4× dermal rounded well-circumscribed lesion. (<b>c</b>) 10× antler-shaped vascular proliferation associated with spindle-shaped cells in the center of the lesion. (<b>d</b>) 25× spindle-shaped cells arranged in fascicles. (<b>e</b>) 10× round lobules made up of elongated cells with poorly seen cytoplasmic borders at the periphery of the lesion. (<b>f</b>) 4× anti-SMA.</p> Full article ">Figure 10 Cont.
<p>Infantile myofibromatosis. (<b>a</b>) This baby presented with multiple small blueish lesions. (<b>b</b>) 4× dermal rounded well-circumscribed lesion. (<b>c</b>) 10× antler-shaped vascular proliferation associated with spindle-shaped cells in the center of the lesion. (<b>d</b>) 25× spindle-shaped cells arranged in fascicles. (<b>e</b>) 10× round lobules made up of elongated cells with poorly seen cytoplasmic borders at the periphery of the lesion. (<b>f</b>) 4× anti-SMA.</p> Full article ">Figure 11
<p>Subcutaneous fat necrosis of the newborn. (<b>a</b>) Multiple skin-colored nodular lesions at the upper back. (<b>b</b>) 10× lobular panniculitis with inflammatory cells and typical radially arranged needle-shaped cleft into the fatty tissue.</p> Full article ">Figure 12
<p>Multifocal infantile hemangioma. (<b>a</b>) Multiple small red lesions disseminated over the body. (<b>b</b>) 10× Well-differentiated small vessels in the superficial dermis. (<b>c</b>) Anti-Glut1 immunostaining showing positivity of all the endothelia.</p> Full article ">Figure 13
<p>Multifocal lymphangiomatosis with thrombocytopenia. (<b>a</b>) Red-brown papules and plaques (by courtesy C Droitcourt). (<b>b</b>) 2.5× Thin-walled vascular channels dissecting the superficial and mid-dermis. (<b>c</b>) 4× These vessels are lined by endothelial cells with a plump, sometimes hobnail, nucleus. (<b>d</b>) These cells are positive for Lyve 1.</p> Full article ">Figure 14
<p>Neonatal pyogenic granulomas. (<b>a</b>) Small red typical pyogenic granuloma. (<b>b</b>) 4× lobulated vascular lesion in the dermis. The overlying epidermis is normal whereas in (<b>c</b>), it is ulcerated and the lesion contains inflammatory cells.</p> Full article ">Figure 14 Cont.
<p>Neonatal pyogenic granulomas. (<b>a</b>) Small red typical pyogenic granuloma. (<b>b</b>) 4× lobulated vascular lesion in the dermis. The overlying epidermis is normal whereas in (<b>c</b>), it is ulcerated and the lesion contains inflammatory cells.</p> Full article ">Figure 15
<p>Tufted angioma and kaposiform hemangioendotheliomatosis. (<b>a</b>) 25× numerous very small and well-limited lobules throughout the dermis. (<b>b</b>) 25× packed small vessels and crescent-shaped vascular channel surrounding it. (<b>c</b>) Anti-podoplanin staining the lobules partially. (<b>d</b>) Poorly limited and infiltrative vascular tumor in the dermis extending into the subcutis. (<b>e</b>) Spindle-shaped cells with red blood cells in between. (<b>f</b>) Partial staining of the vessels with anti-podoplanin.</p> Full article ">Figure 15 Cont.
<p>Tufted angioma and kaposiform hemangioendotheliomatosis. (<b>a</b>) 25× numerous very small and well-limited lobules throughout the dermis. (<b>b</b>) 25× packed small vessels and crescent-shaped vascular channel surrounding it. (<b>c</b>) Anti-podoplanin staining the lobules partially. (<b>d</b>) Poorly limited and infiltrative vascular tumor in the dermis extending into the subcutis. (<b>e</b>) Spindle-shaped cells with red blood cells in between. (<b>f</b>) Partial staining of the vessels with anti-podoplanin.</p> Full article ">Figure 16
<p>Glomuvenous malformation. (<b>a</b>) Bluish to violet small nodules in the arm. (<b>b</b>) 10× multiples veinous channels in the dermis. (<b>c</b>) 25× these vessels are surrounded by round glomus cells. (<b>d</b>) These cells are highlighted by anti-SMA.</p> Full article ">Figure 17
<p>A blue rubber bleb nevus syndrome. (<b>a</b>) Blue nodules on the foot. (<b>b</b>) 4× dilated venous vessels, some of them containing thrombi.</p> Full article ">Figure 18
<p>Cutaneous mastocytosis. (<b>a</b>) Multiple maculopapular lesions present at birth. (<b>b</b>) Rather dense band-like infiltrations at the upper dermis. (<b>c</b>) 40× large mast cells containing granules in their cytoplasm. (<b>d</b>) anti-CD117 stains mast cells in bullous mastocytosis.</p> Full article ">Figure 18 Cont.
<p>Cutaneous mastocytosis. (<b>a</b>) Multiple maculopapular lesions present at birth. (<b>b</b>) Rather dense band-like infiltrations at the upper dermis. (<b>c</b>) 40× large mast cells containing granules in their cytoplasm. (<b>d</b>) anti-CD117 stains mast cells in bullous mastocytosis.</p> Full article ">
14 pages, 6906 KiB  
Review
Pseudomalignancies in Children: Histological Clues, and Pitfalls to Be Avoided
by Sébastien Menzinger and Sylvie Fraitag
Dermatopathology 2021, 8(3), 376-389; https://doi.org/10.3390/dermatopathology8030042 - 14 Aug 2021
Cited by 2 | Viewed by 4547
Abstract
The term “pseudomalignancy” covers a large, heterogenous group of diseases characterized by a benign cellular proliferation, hyperplasia, or infiltrate that resembles a true malignancy clinically or histologically. Here, we (i) provide a non-exhaustive review of several inflammatory skin diseases and benign skin proliferations [...] Read more.
The term “pseudomalignancy” covers a large, heterogenous group of diseases characterized by a benign cellular proliferation, hyperplasia, or infiltrate that resembles a true malignancy clinically or histologically. Here, we (i) provide a non-exhaustive review of several inflammatory skin diseases and benign skin proliferations that can mimic a malignant neoplasm in children, (ii) give pathologists some helpful clues to guide their diagnosis, and (iii) highlight pitfalls to be avoided. The observation of clinical–pathological correlations is often important in this situation and can sometimes be the only means (along with careful monitoring of the disease’s clinical course) of reaching a firm diagnosis. Full article
(This article belongs to the Special Issue New Insights in Pediatric Dermatopathology)
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Figure 1

Figure 1
<p>An example of vitiligo (inflammatory phase): parakeratosis, epidermal hyperplasia, slight spongiosis, lymphocyte exocytosis, and a discrete lymphocytic infiltrate in the superficial dermis. HE ×200.</p> Full article ">Figure 2
<p>IPEX syndrome: Epidermal hyperplasia, marked lymphocytic exocytosis, scattered apoptotic keratinocytes (arrowhead), and a moderate lymphocytic infiltrate in the superficial dermis. HE ×400.</p> Full article ">Figure 3
<p>Mycosis fungoides-like pityriasis lichenoides. In this example there is a marked lymphocytic exocytosis, with lymphocytes displaying a clear halo. Note the few necrotic keratinocytes (arrowhead). HE ×250.</p> Full article ">Figure 4
<p>Lymphoplasmacytic plaque: parakeratotic scale, epidermal hyperplasia, a dense lymphoplasmacytic infiltrate in the whole dermis. Inset: note the numerous plasma cells. HE ×40 (inset: ×200).</p> Full article ">Figure 5
<p>Left panel: Langerhans cell histiocytosis. (<b>A</b>): HE ×40. Superficial infiltrate of histiocytes with epidermotropism and edema. (<b>B</b>): CD1a immunostaining. (<b>C</b>): CD207/Langerin immunostaining. The whole infiltrate express CD207. Right panel: spongiotic dermatitis with CD1a+ dendritic cell hyperplasia. (<b>D</b>): HE ×40. (<b>E</b>): CD1a immunostaining. (<b>F</b>): CD207/Langerin immunostaining. The true Langerhans cells in the epidermal vesicles express CD207. The dermal infiltrate is almost completely negative.</p> Full article ">Figure 6
<p>Junctional component of a congenital nevus in a newborn. Irregularly distributed melanocytes with pagetoid spread. HE ×250.</p> Full article ">Figure 7
<p>(<b>A</b>): (HE ×40) Proliferative nodule in a congenital melanocytic lesion. (<b>B</b>): (HE ×250) The melanocytes of the proliferative nodule are epithelioid and with a dusty cytoplasm, without nuclear atypia. Note the delicate transition (blending), which may be very focal and inconspicuous.</p> Full article ">Figure 8
<p>Pagetoid Spitz nevus (HE ×250). Epithelioid or spindle melanocytes with large nuclei and abundant “ground glass” cytoplasm, and pagetoid spread.</p> Full article ">
5 pages, 888 KiB  
Communication
Granular Cell Dermatofibroma: When Morphology Still Matters
by Gerardo Cazzato, Anna Colagrande, Antonietta Cimmino, Maricla Marrone, Alessandra Stellacci, Francesca Arezzo, Teresa Lettini, Leonardo Resta and Giuseppe Ingravallo
Dermatopathology 2021, 8(3), 371-375; https://doi.org/10.3390/dermatopathology8030041 - 13 Aug 2021
Cited by 8 | Viewed by 4294
Abstract
Dermatofibroma, also known as “fibrous histiocytoma”, is one of the most common cutaneous soft-tissue tumors. Many variants of dermatofibromas have been described, and knowledge of these variations is important to avoid misdiagnosis of a possibly more aggressive tumor. Histological features of different variants [...] Read more.
Dermatofibroma, also known as “fibrous histiocytoma”, is one of the most common cutaneous soft-tissue tumors. Many variants of dermatofibromas have been described, and knowledge of these variations is important to avoid misdiagnosis of a possibly more aggressive tumor. Histological features of different variants can coexist in the same lesion, but typical common fibrous histiocytoma features are generally found, at least focally, in all cases. However, when cellular changes make up the majority of the lesion, the histopathological diagnosis can become more complex and requires immunohistochemical investigations for correct nosographic classification. We present the case of a cutaneous fibrous histiocytoma, “granular cell” variant, found on the left leg of a 74-year-old woman. Full article
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<p>A grayish crusty and scaling plaque with a 30 mm of diameter was detected on the anterior middle third of the left leg.</p> Full article ">Figure 2
<p>(<b>A</b>) The lesion was composed of a variable admixture of fibroblast-like cells and histiocytes that showed in more than 85% a large eosinophilic cytoplasm filled of granules or microvacuoles (hematoxylin and eosin, original magnification 40×). (<b>B</b>) Cytological details of the lesion with granular cytoplasm (original magnification 60×). Box: Histological details of the granular cytoplasm of the GCD constituent cells (original magnification 60×). (<b>C</b>) The neoplastic cells were strongly immunoreactive for CD68 (original magnification 40×).</p> Full article ">
12 pages, 7084 KiB  
Review
Aberrant Expression of Immunohistochemical Markers in Malignant Melanoma: A Review
by Elie Saliba and Jag Bhawan
Dermatopathology 2021, 8(3), 359-370; https://doi.org/10.3390/dermatopathology8030040 - 3 Aug 2021
Cited by 24 | Viewed by 10580
Abstract
Immunohistochemical stains are increasingly used to aid in the diagnosis of malignant melanoma, especially when the differentiation of the tumor is unclear based on examination with hematoxylin and eosin. However, aberrant expression of non-melanocytic markers has been reported in melanomas, which can sometimes [...] Read more.
Immunohistochemical stains are increasingly used to aid in the diagnosis of malignant melanoma, especially when the differentiation of the tumor is unclear based on examination with hematoxylin and eosin. However, aberrant expression of non-melanocytic markers has been reported in melanomas, which can sometimes be further complicated by the loss of conventional melanocytic markers. This review aims to summarize available data regarding unusual staining patterns in primary and metastatic malignant melanoma. It also raises awareness of the potential pitfalls and highlights the importance of appropriate use and interpretation of broad immunohistochemical markers in the context of clinical and histopathologic findings to facilitate the diagnosis of atypical cases of malignant melanoma. Full article
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<p>Spindle cell melanoma ((<b>A</b>), hematoxylin and eosin (H &amp; E) stain ×10. Lesional cells are negative for Mart-1 ((<b>B</b>), immunohistochemical stain ×10) and positive for SOX10 ((<b>C</b>), immunohistochemical stain ×10) and NGFR ((<b>D</b>), immunohistochemical stain ×10).</p> Full article ">Figure 2
<p>Desmoplastic melanoma. Lesional cells are negative for Mart-1 ((<b>A</b>), immunohistochemical stain ×10) and positive for S100 ((<b>B</b>), immunohistochemical stain ×4) and NGFR ((<b>C</b>), immunohistochemical stain ×10).</p> Full article ">Figure 3
<p>Desmoplastic and neurotropic melanoma. Lesional cells are positive for NGFR ((<b>A</b>), immunohistochemical stain ×20) with aberrant expression of actin ((<b>B</b>), immunohistochemical stain ×20), CD34 ((<b>C</b>), immunohistochemical stain ×20), and CD68 ((<b>D</b>), immunohistochemical stain ×20).</p> Full article ">Figure 4
<p>Malignant melanoma ((<b>A</b>), H &amp; E ×20). Lesional cells are positive for S100 ((<b>B</b>), immunohistochemical stain ×20) and low molecular weight keratin ((<b>C</b>), immunohistochemical stain ×20).</p> Full article ">Figure 5
<p>Malignant melanoma ((<b>A</b>), H &amp; E ×10) with aberrant expression of CD31 ((<b>B</b>), immunohistochemical stain ×10).</p> Full article ">Figure 6
<p>Malignant melanoma. Lesional cells are positive for Mart-1 ((<b>A</b>), immunohistochemical stain ×20) with aberrant expression of CEA ((<b>B</b>), immunohistochemical stain ×20).</p> Full article ">
17 pages, 5447 KiB  
Review
Diagnostic Approach to Congenital Cystic Masses of the Neck from a Clinical and Pathological Perspective
by Amanda Fanous, Guillaume Morcrette, Monique Fabre, Vincent Couloigner and Louise Galmiche-Rolland
Dermatopathology 2021, 8(3), 342-358; https://doi.org/10.3390/dermatopathology8030039 - 1 Aug 2021
Cited by 17 | Viewed by 10403
Abstract
Background: neck cysts are frequently encountered in pediatric medicine and can present a diagnostic dilemma for clinicians and pathologists. Several clinical items enable to subclassify neck cyst as age at presentation, anatomical location, including compartments and fascia of the neck, and radiological presentation. [...] Read more.
Background: neck cysts are frequently encountered in pediatric medicine and can present a diagnostic dilemma for clinicians and pathologists. Several clinical items enable to subclassify neck cyst as age at presentation, anatomical location, including compartments and fascia of the neck, and radiological presentation. Summary: this review will briefly describe the clinical, imaging, pathological and management features of (I) congenital and developmental pathologies, including thyroglossal duct cyst, branchial cleft cysts, dermoid cyst, thymic cyst, and ectopic thymus; (II) vascular malformations, including lymphangioma. Key Messages: pathologists should be familiar with the diagnostic features and clinicopathologic entities of these neck lesions in order to correctly diagnose them and to provide proper clinical management. Full article
(This article belongs to the Special Issue New Insights in Pediatric Dermatopathology)
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<p>Diagnostic algorithm with the different steps depending on clinical examination and imaging.</p> Full article ">Figure 2
<p>Thyroglossal duct cyst. (<b>A</b>) Clinical examination: typical picture of a non-infected thyroglossal duct cyst presenting as a midline neck mass. (<b>B</b>) Gross examination: cystic lumen filled with gelatinous and inflammatory material. (<b>C</b>) (H.E.S). Small cystic structure lined by ciliated pseudostratified columnar epithelium; mucous glands (*) on the left; hyoid bone (+) on the right, in close relation to the cyst. (<b>D</b>) (H.E.S), ectopic thyroid follicles in the cystic wall.</p> Full article ">Figure 3
<p>Branchial cleft cysts. First branchial cleft cyst (<b>A</b>), operating view. Periauricular type I cyst (<b>B</b>), gross examination. Cartilage (*) and necrosis (+) are visible. Periparotid type II with inflammatory changes (<b>C</b>) (H.E.S). lined by squamous epithelium and containing cartilage (<b>D</b>) (H.E.S). Lined by squamous epithelium and ciliated columnar epithelium (*). The lumen contains macrophages and squamous debris (+) (<b>E</b>) (H.E.S), located within the parotid gland (at the upper part, arrow), mainly denuded due to inflammatory changes (<b>F</b>) (H.E.S). The epithelial border is mainly replaced by foreign body giant cells (*). Lipid-laden macrophages are observed in the cyst wall and squamous debris within the cyst lumen. Second branchial fistula (<b>G</b>) (H.E.S), lined by respiratory epithelium and confluent lymphoid follicles (*). Fourth branchial fistula (<b>H</b>) (H.E.S). The wall of the fistula contains thyroid tissue (*).</p> Full article ">Figure 3 Cont.
<p>Branchial cleft cysts. First branchial cleft cyst (<b>A</b>), operating view. Periauricular type I cyst (<b>B</b>), gross examination. Cartilage (*) and necrosis (+) are visible. Periparotid type II with inflammatory changes (<b>C</b>) (H.E.S). lined by squamous epithelium and containing cartilage (<b>D</b>) (H.E.S). Lined by squamous epithelium and ciliated columnar epithelium (*). The lumen contains macrophages and squamous debris (+) (<b>E</b>) (H.E.S), located within the parotid gland (at the upper part, arrow), mainly denuded due to inflammatory changes (<b>F</b>) (H.E.S). The epithelial border is mainly replaced by foreign body giant cells (*). Lipid-laden macrophages are observed in the cyst wall and squamous debris within the cyst lumen. Second branchial fistula (<b>G</b>) (H.E.S), lined by respiratory epithelium and confluent lymphoid follicles (*). Fourth branchial fistula (<b>H</b>) (H.E.S). The wall of the fistula contains thyroid tissue (*).</p> Full article ">Figure 4
<p>Lymphatic malformation. (<b>A</b>), (H.E.S). Collection of large interconnected lymphatic cisterns. Small lymphoid aggregates are also present (at the upper left quarter). (<b>B</b>), (D2-40). Positive cytoplasmic immunostaining of endothelial cells. (<b>C</b>), (smooth muscle actin). Positive cytoplasmic immunostaining of disorganized smooth muscle in the wall of larger channels.</p> Full article ">Figure 5
<p>Dermoid cyst. (<b>A</b>) (H.E.S). The cyst is lined by keratinizing stratified squamous epithelium. (<b>B</b>) (H.E.S). The cyst is deeply located in the subcutis. Small hair follicles and sebaceous glands are attached to the epithelium. (<b>C</b>) (H.E.S). The lumen contains numerous vellus hair shafts and lamellated keratin, and the epithelium has a granular layer. (<b>D</b>), (H.E.S). Several vellus hairs (arrowheads) are observed within the granuloma.</p> Full article ">Figure 5 Cont.
<p>Dermoid cyst. (<b>A</b>) (H.E.S). The cyst is lined by keratinizing stratified squamous epithelium. (<b>B</b>) (H.E.S). The cyst is deeply located in the subcutis. Small hair follicles and sebaceous glands are attached to the epithelium. (<b>C</b>) (H.E.S). The lumen contains numerous vellus hair shafts and lamellated keratin, and the epithelium has a granular layer. (<b>D</b>), (H.E.S). Several vellus hairs (arrowheads) are observed within the granuloma.</p> Full article ">Figure 6
<p>Thymic cyst. (<b>A</b>), (H.E.S). Mature thymic tissue showing Hassall’s corpuscles (arrow) and cortico-medullary differentiation and arranged in lobules at the hypodermis. The overlying dermis is devoid of adnexa and replaced by scar-like fibrous tissue. (<b>B</b>), (H.E.S). Ductal-like structures (arrow) opening at the surface of the skin, lined by a columnar, pseudostratified epithelium (at the left, half part).</p> Full article ">Figure 7
<p>Bronchogenic cyst. (<b>A</b>), (H.E.S). unilocular cyst lined by respiratory epithelium overlying circular muscular tunic (arrow). (<b>B</b>), (H.E.S.). In the wall, seromucous glands (arrowheads) and cartilage plates (*).</p> Full article ">
5 pages, 2859 KiB  
Case Report
Plaque-Like Dermatofibroma: Case Report of a Rare Entity
by Sara Moradi, Laila Mnayer, Jonathan Earle, Alex C. Cech and Torsten Ehrig
Dermatopathology 2021, 8(3), 337-341; https://doi.org/10.3390/dermatopathology8030038 - 1 Aug 2021
Cited by 3 | Viewed by 3571
Abstract
A case of a well-demarcated plaque measuring 11 cm without satellites of several years’ duration is presented. It showed typical histologic findings of dermatofibroma, prompting a diagnosis of plaque-like dermatofibroma. The relationship to multiple clustered dermatofibromas and plaque-like myofibroblastic tumor is discussed. Full article
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<p>A plaque with irregular brown pigmentation.</p> Full article ">Figure 2
<p>Histopathology of plaque-like dermatofibroma. Well-demarcated infiltrate of spindle cells in the dermis and upper subcutis (<b>a</b>,<b>b</b>) with overlying epidermal hyperplasia ((<b>b</b>), scale bar: 400 µm), multinucleated cells ((<b>c</b>), scale bar: 60 µm), and foam cells ((<b>d</b>), scale bar: 100 µm).</p> Full article ">Figure 2 Cont.
<p>Histopathology of plaque-like dermatofibroma. Well-demarcated infiltrate of spindle cells in the dermis and upper subcutis (<b>a</b>,<b>b</b>) with overlying epidermal hyperplasia ((<b>b</b>), scale bar: 400 µm), multinucleated cells ((<b>c</b>), scale bar: 60 µm), and foam cells ((<b>d</b>), scale bar: 100 µm).</p> Full article ">
22 pages, 4989 KiB  
Review
Panniculitis in Children
by Isabelle Moulonguet and Sylvie Fraitag
Dermatopathology 2021, 8(3), 315-336; https://doi.org/10.3390/dermatopathology8030037 - 1 Aug 2021
Cited by 10 | Viewed by 8762
Abstract
Panniculitides form a heterogenous group of inflammatory diseases that involve the subcutaneous adipose tissue. These disorders are rare in children and have many aetiologies. As in adults, the panniculitis can be the primary process in a systemic disorder or a secondary process that [...] Read more.
Panniculitides form a heterogenous group of inflammatory diseases that involve the subcutaneous adipose tissue. These disorders are rare in children and have many aetiologies. As in adults, the panniculitis can be the primary process in a systemic disorder or a secondary process that results from infection, trauma or exposure to medication. Some types of panniculitis are seen more commonly or exclusively in children, and several new entities have been described in recent years. Most types of panniculitis have the same clinical presentation (regardless of the aetiology), with tender, erythematous subcutaneous nodules. Although the patient’s age and the lesion site provide information, a histopathological assessment is sometimes required for a definitive diagnosis and classification of the disorder. In children, most panniculitides are lobular. At present, autoimmune inflammatory diseases and primary immunodeficiencies have been better characterised; panniculitis can be the presenting symptom in some of these settings. Unexplained panniculitis in a young child should prompt a detailed screen for monogenic immune disorders because the latter usually manifest themselves early in life. Here, we review forms of panniculitis that occur primarily in children, with a focus on newly described entities. Full article
(This article belongs to the Special Issue New Insights in Pediatric Dermatopathology)
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<p>Subcutaneous fat necrosis of the newborn. (<b>A</b>) Indurated plaques on the neck and on the back of a newborn. (<b>B</b>) Significant purulent material of the shoulder <span class="html-italic">Photo Dermatology Department Hopital Necker Enfants Malades. Paris France.</span> (<b>C</b>) Lobular panniculitis without involvement of the dermis (original magnification ×20) (<b>D</b>) Many adipocytes are replaced by cells with finely eosinophilic granular cytoplasm that contain narrow needle-shaped clefts (original magnification ×100). (<b>E</b>) Needle-shaped crystals in radial fashion surrounded by histiocytes (original magnification ×400).</p> Full article ">Figure 2
<p>Post-steroid panniculitis. (<b>A</b>) Clinical presentation: subcutaneous nodules with overlying erythema. (<b>B</b>) Histopathologic features of post-steroid panniculitis: mostly lobular panniculitis with an inflammatory infiltrate of foamy histiocytes involving the fat lobules (original magnification ×40). (<b>C</b>) Needle-shaped clefts (original magnification ×400).</p> Full article ">Figure 3
<p>Panniculitis associated with inherited immunodeficiency. (<b>A</b>,<b>B</b>) Clinical presentation: Erythematous nodules of the leg of a 20 month-old boy <span class="html-italic">Photo Dermatology Department Hopital Necker Enfants Malades. Paris France</span> (<b>C</b>,<b>D</b>) Histopathology: inflammation of the deep dermis and the subcutis localised in the septa and the lobules composed of mixed infiltrate. (<b>C</b>) Original magnification ×80, (<b>D</b>) original magnification ×200.</p> Full article ">Figure 3 Cont.
<p>Panniculitis associated with inherited immunodeficiency. (<b>A</b>,<b>B</b>) Clinical presentation: Erythematous nodules of the leg of a 20 month-old boy <span class="html-italic">Photo Dermatology Department Hopital Necker Enfants Malades. Paris France</span> (<b>C</b>,<b>D</b>) Histopathology: inflammation of the deep dermis and the subcutis localised in the septa and the lobules composed of mixed infiltrate. (<b>C</b>) Original magnification ×80, (<b>D</b>) original magnification ×200.</p> Full article ">Figure 4
<p>Panniculitis associated with inherited immunodeficiency. (<b>A</b>) Lobular panniculitis with dense neutrophilic infiltrate replacing part of the subcutis (original magnification ×80). (<b>B</b>) Closer view of showing entrapped adipocytes within the neutrophilic infiltrate (original magnification ×200).</p> Full article ">Figure 5
<p>A Recurrent lipoatrophic panniculitis of children. (<b>A</b>) Areas of lipoatrophy in the leg <span class="html-italic">Photo Dermatology Department Hopital Necker Enfants Malades. Paris France</span>. (<b>B</b>,<b>C</b>) Lobular panniculitis with a mixed infiltrate, including neutrophils, as well as lymphocytes and macrophages. (<b>B</b>) Original magnification ×10, (<b>C</b>) original magnification ×40. <span class="html-italic">Photo Courtesy of Christina Mittledorf Dermatologie, Venerologie und Allergologie Göttingen Germany.</span></p> Full article ">Figure 6
<p>(<b>A</b>,<b>B</b>) Self-healing juvenile cutaneous mucinosis. Painful nodules of the limbs. <span class="html-italic">Photo Courtesy of Christine Bodemer Dermatology Department Hopital Necker Enfants Malades. Paris France</span>. (<b>C</b>,<b>D</b>) Chronic lobular panniculitis with features of proliferative fasciitis. (<b>C</b>) Original magnification ×20, (<b>D</b>) original magnification ×100.</p> Full article ">Figure 7
<p>Erythema nodosum. (<b>A</b>) Nodules of the legs in an 8-year-old child. <span class="html-italic">Photo Dermatology Department Hopital Necker Enfants Malades. Paris France</span>. (<b>B</b>) Fibrosing septal pattern with involvement of the periphery of the fat lobules. The process extends into the fat lobules (original magnification ×40). (<b>C</b>) Thickened septa of subcutaneous fat with granulomatous inflammation (original magnification ×100). (<b>D</b>) Miescher granuloma consisting of small nodular aggregation of histiocytes around a central stellate-shaped cleft located near the junction of the adipocytes and the septum (original magnification ×200).</p> Full article ">Figure 8
<p>Subcutaneous aluminium granuloma. (<b>A</b>) Dense, deep dermal and subcutaneous nodular infiltrate surrounding areas of necrosis (original magnification ×80). (<b>B</b>) Mixed infiltrate of lymphocytes, histiocytes, and eosinophils (original magnification ×250). (<b>C</b>) Histiocytes containing violaceous granular within their cytoplasm (original magnification ×400). (<b>D</b>) The granules stain positive with aluminon staining (Morin, (original magnification ×200)), which reveals the existence of aluminium.</p> Full article ">Figure 9
<p>Lupus panniculitis. (<b>A</b>) Dense dermal infiltrate with extension into subcutaneous fat (original magnification ×30). (<b>B</b>) Septae appear fibrotic (original magnification ×100). (<b>C</b>) Lymphoid follicles (original magnification ×120). (<b>D</b>) Necrosis of adipocytes with hyalinisation of the fat lobular panniculitis with lymphocytes among necrotic adipocytes (original magnification ×120).</p> Full article ">Figure 10
<p>Cytotoxic T clonal panniculitis. (<b>A</b>) Lobular panniculitis with lymphoid cell infiltration (original magnification ×30). (<b>B</b>) Atypical lymphoid cells with adipocyte rimming and adipocyte necrosis showing infiltration by cells expressing (<b>C</b>) CD3, (<b>D</b>) Tia1, and (<b>E</b>) granzyme (original magnification ×400).</p> Full article ">
14 pages, 5599 KiB  
Review
Cutaneous Melanomas Arising during Childhood: An Overview of the Main Entities
by Arnaud de la Fouchardière, Felix Boivin, Heather C. Etchevers and Nicolas Macagno
Dermatopathology 2021, 8(3), 301-314; https://doi.org/10.3390/dermatopathology8030036 - 1 Aug 2021
Cited by 4 | Viewed by 3508
Abstract
Cutaneous melanomas are exceptional in children and represent a variety of clinical situations, each with a different prognosis. In congenital nevi, the risk of transformation is correlated with the size of the nevus. The most frequent type is lateral transformation, extremely rare before [...] Read more.
Cutaneous melanomas are exceptional in children and represent a variety of clinical situations, each with a different prognosis. In congenital nevi, the risk of transformation is correlated with the size of the nevus. The most frequent type is lateral transformation, extremely rare before puberty, reminiscent of a superficial spreading melanoma (SSM) ex-nevus. Deep nodular transformation is much rarer, can occur before puberty, and must be distinguished from benign proliferative nodules. Superficial spreading melanoma can also arise within small nevi, which were not visible at birth, usually after puberty, and can reveal a cancer predisposition syndrome (CDKN2A or CDK4 germline mutations). Prognosis is correlated with classical histoprognostic features (mainly Breslow thickness). Spitz tumors are frequent in adolescents and encompass benign (Spitz nevus), intermediate (atypical Spitz tumor), and malignant forms (malignant Spitz tumor). The whole spectrum is characterized by specific morphology with spindled and epithelioid cells, genetic features, and an overall favorable outcome even if a regional lymph node is involved. Nevoid melanomas are rare and difficult to diagnose clinically and histologically. They can arise in late adolescence. Their prognosis is currently not very well ascertained. A small group of melanomas remains unclassified after histological and molecular assessment. Full article
(This article belongs to the Special Issue New Insights in Pediatric Dermatopathology)
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<p>SSM melanoma and pagetoid Spitz nevus (hematoxylin, phloxin, safranin stain). (<b>A</b>): Low-magnification silhouette of SSM, 14-year-old: mainly junctional disorganized melanocytic proliferation. (<b>B</b>): Close-up view showing dispersed junctional nests. Ascent of isolated cell. Numerous lymphocytes and melanophages clutter the upper dermis. (<b>C</b>): Invasive melanoma with “pseudo-maturation” and mitotic figure (arrow). (<b>D</b>): Large, junctional, foamy melanocytes. (<b>E</b>): Low-magnification silhouette of a pagetoid Spitz nevus, 6-year-old. Epidermal hyperplasia encasing numerous small nests with a regular distribution. (<b>F</b>,<b>G</b>): Close-up view with isolated cells and small nests. An important pericellular retraction is seen. Ascent of small, isolated cells. Arborescent vascular structure in the upper dermis surrounded by small lymphocytes.</p> Full article ">Figure 2
<p>Malignant Spitz tumor, with <span class="html-italic">MYO5A-RET</span> fusion, 4-year-old (hematoxylin, phloxin, safranin stain). (<b>A</b>): Low-magnification silhouette: massive, sheet-like, destructive dermal expansion elevating the epidermis and invading the subcutis. Central dermal tumoral necrosis patch. (<b>B</b>): Close-up view of junction with a thinned epidermis covered by a crust. Obscuration of the grenz zone by dense fascicules. (<b>C</b>,<b>D</b>): High-power view of the dermal fascicules made of large spindled atypical melanocytes with mitotic activity (arrow).</p> Full article ">Figure 3
<p>Spitzoid melanoma, 9-year-old (hematoxylin, phloxin, safranin stain). (<b>A</b>): Low-magnification silhouette: mainly dermal clonal proliferation, without pigmentation, elevating a slightly verrucous epidermis. Central vertical periadnexial expansion. (<b>B</b>): Confluent nests are present in the junction. Abrupt cytological hiatus and lymphocytes are seen in the dermis below. (<b>C</b>): Close-up view of the dermal component with spindled and epithelioid large melanocytes with mitotic figure (arrow).</p> Full article ">Figure 4
<p>Superficial spreading melanoma ex-congenital nevus, 13-year-old (hematoxylin, phloxin, safranin stain). (<b>A</b>): Low-magnification silhouette: asymmetrical lesion with on the left side a compound, mainly dermal, congenital nevus with a loose horizontal band of melanocytes under a slightly verrucous epidermis, and on the right side a mainly junctional melanocytic proliferation underlined by a pigmented inflammatory reaction. (<b>B</b>): Close-up view on the transition between the nevus and melanoma. (<b>C</b>): Close-up view disorganized confluent junctional nests of large spindled hyperpigmented melanocytes in a slightly atrophic epidermis with numerous.</p> Full article ">Figure 5
<p>Melanoma ex-giant congenital nevus, 8-year-old (hematoxylin, phloxin, safranin stain). (<b>A</b>): Low-magnification silhouette: loose horizontal band of melanocytes under a verrucous epidermis elevated by a large cellular dermal nodule with hyperpigmented areas and patch of tumoral necrosis. (<b>B</b>): Close-up view on transition area with the congenital nevus made of loose bland melanocytes in the upper dermis separated by fibrous collagen from the dense nests of the melanoma underneath. (<b>C</b>,<b>D</b>): High-power view of the melanoma with confluent nests of large epithelioid and nevoid melanocytes; mitotic figure (arrow).</p> Full article ">Figure 6
<p>Nevoid melanoma, 17-year-old (hematoxylin, phloxin, safranin stain). (<b>A</b>): Low-magnification silhouette: massive dermal invasion by a pigmented clonal proliferation with the destruction of hair follicles. The grenz zone is obscured, and the epidermis is elevated. (<b>B</b>): Close-up view displaying sheets of large epithelioid melanocytes with mitotic figures (arrows) and dispersed melanophages.</p> Full article ">Figure 7
<p>Melanoma <span class="html-italic">ex</span> BAP1 inactivated nevus; relapse, initial lesion at age 11(hematoxylin, phloxin, safranin stain). (<b>A</b>): Low-magnification silhouette: compound melanocytic proliferation with dense dermal nodular areas including pigmented clones. (<b>B</b>): Intermediate view of a cellular area with admixed small and large cells. (<b>C</b>): Close-up view of a deep dermal nodule with mixed small nevocytoid and large epithelioid melanocytes. A few of them show a pigmented cytoplasm; mitotic figure (arrow). (<b>D</b>): BAP1 immunohistochemistry (clone C4; 1/50): loss of nuclear staining with positive controls of endothelial cells. (<b>E</b>): Ki67 immunohistochemistry: variable dermal staining with clonal areas showing a 10% positivity.</p> Full article ">
16 pages, 152907 KiB  
Review
Update on Superficial Spindle Cell Mesenchymal Tumors in Children
by Philippe Drabent and Sylvie Fraitag
Dermatopathology 2021, 8(3), 285-300; https://doi.org/10.3390/dermatopathology8030035 - 21 Jul 2021
Cited by 6 | Viewed by 8442
Abstract
The diagnosis of cutaneous and subcutaneous spindle cell neoplasms in children is often challenging and has potential therapeutic and prognostic implications. Although correctly diagnosing dermatofibrosarcoma protuberans and infantile fibrosarcoma is paramount, pathologists should not ignore a number of diagnostic pitfalls linked to mostly [...] Read more.
The diagnosis of cutaneous and subcutaneous spindle cell neoplasms in children is often challenging and has potential therapeutic and prognostic implications. Although correctly diagnosing dermatofibrosarcoma protuberans and infantile fibrosarcoma is paramount, pathologists should not ignore a number of diagnostic pitfalls linked to mostly rare tumors with completely different clinical outcomes. In the last decade, a spectrum of novel entities has been described; information from molecular biology has helped to shape this new landscape for spindle cell tumors. Here, we review the most noteworthy neoplasms in this spectrum, with a focus on their histological similarities: fibroblastic connective tissue nevus, medallion-like dermal dendrocyte hamartoma, or plaque-like CD34-positive dermal fibroma, which share features with fibrous hamartoma of infancy; lipofibromatosis and lipofibromatosis-like neural tumor; and plexiform myofibroblastoma, a recently described neoplasm that should be distinguished from plexiform fibrohistiocytic tumor. These tumors also have genetic similarities, particularly gene rearrangements involving NTRK3 or NTRK1. These genetic features are not only essential for the differential diagnosis of infantile fibrosarcoma but are also of diagnostic value for lipofibromatosis-like neural tumors. The more recently described RET, RAF1, and BRAF gene fusions are also discussed. Full article
(This article belongs to the Special Issue New Insights in Pediatric Dermatopathology)
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<p>Fibroblastic connective tissue nevus. (<b>A</b>) Clinical presentation on the scalp of an infant (courtesy of the Department of Maxillofacial Surgery, Necker-Enfants Malades hospital, Paris, France). (<b>B</b>) Low-power view of the lesion showing infiltration of the dermis and subcutis (HE ×50). (<b>C</b>) Short intersecting fascicles surrounding the appendages and epidermal hyperplasia (HE ×100). (<b>D</b>) Extension into the subcutis (HE ×100). (<b>E</b>) High-power view showing the bland morphology of the spindle cells (HE ×200). (<b>F</b>) Diffuse positivity for CD34 (×50). (<b>G</b>) In this case, smooth muscle actin was negative, with internal controls on vessels and smooth muscles (×50).</p> Full article ">Figure 2
<p>Medallion-like dermal dendrocyte hamartoma (MLDDH)/plaque-like CD34-positive dermal fibroma (PDF). (<b>A</b>) Clinical presentation as a medallion-like lesion on the upper back of an infant (courtesy of the Department of Maxillofacial Surgery, Necker-Enfants Malades hospital, Paris, France). (<b>B</b>) Low-power view of the lesion showing dermal location and slight extension into the subcutis (HES ×50). (<b>C</b>) Venules with dilated lumens (HES ×100). (<b>D</b>) Presence of both spindle cells and more ovoid cells (HES ×200). (<b>E</b>) High-power view showing the bland morphology of the spindle cells and a mast cell in the center of the picture (HES ×400). (<b>F</b>) Diffuse positivity for CD34 (×50).</p> Full article ">Figure 3
<p>Fibrous hamartoma of infancy (FHI). (<b>A</b>) Typical clinical presentation as an axillary mass (courtesy of the Department of Maxillofacial Surgery, Necker-Enfants Malades hospital, Paris, France). (<b>B</b>) Low-power view of the lesion showing location in the subcutis and extension of the spindle-cell component into the dermis (HE ×50). (<b>C</b>) Presence of the three typical components (mature fibrous tissue, mature adipose tissue, immature mesenchymal tissue) (HE ×100). (<b>D</b>) In some cases, the immature mesenchymal tissue is not visible (HE ×100). (<b>E</b>) Peri-eccrine extension of the lesion (HES ×100). (<b>F</b>) Hyalinized zone reminiscent of giant cell fibroblastoma (HES ×100).</p> Full article ">Figure 4
<p>Lipofibromatosis (LPF). (<b>A</b>) Clinical presentation as a large mass of the right lower leg in an infant (courtesy of the Department of Maxillofacial Surgery, Necker-Enfants Malades hospital, Paris, France). (<b>B</b>) Low-power view of the lesion showing location in the subcutis (HES ×50). (<b>C</b>) Long fascicles of spindle cells admixed with adipose tissue (HES ×100). (<b>D</b>) High-power view showing the bland morphology of the cells (HES ×200). (<b>E</b>) Focus on cells with a single vacuole at the interface between the fibroblastic and the adipose components (HES ×200).</p> Full article ">Figure 5
<p>Lipofibromatosis-like neural tumor (LPF-NT). (<b>A</b>) Low-power view showing extension of the tumor in both the dermis and subcutis (HE ×50). (<b>B</b>) Spindle cells in fascicles admixed with adipose tissue (HE ×100). (<b>C</b>) Some areas can lack the typical fascicular growth (HE ×100). (<b>D</b>) Heterogeneous positivity for CD34 (×400). (<b>E</b>) Positivity for S100 (×400). (<b>F</b>) Diffuse positivity for NTRK1 (×200). (<b>G</b>) FISH showing fusion transcripts between <span class="html-italic">NTRK</span>1 and <span class="html-italic">LMNA</span>. (Courtesy of Marie Karanian, Department of Pathology, Centre Léon Bérard, Lyon, France).</p> Full article ">Figure 6
<p>Plexiform myofibroblastoma (PM). (<b>A</b>) Clinical presentation as a sub-scapular subcutaneous mass in an infant (courtesy of the Department of Maxillofacial Surgery, Necker-Enfants Malades hospital, Paris, France). (<b>B</b>) Low-power view of the lesion showing location in the subcutis (HES ×50). (<b>C</b>) Typical plexiform architecture with extension into the subcutaneous septa (HES ×100). (<b>D</b>) High-power view showing the bland morphology of the cells and the characteristic collagenous stroma (HES ×200). (<b>E</b>) Positivity for smooth muscle actin in the spindle cells (SMA ×200).</p> Full article ">
8 pages, 1719 KiB  
Article
Evaluation of Melanocyte Loss in Mycosis Fungoides Using SOX10 Immunohistochemistry
by Cynthia Reyes Barron and Bruce R. Smoller
Dermatopathology 2021, 8(3), 277-284; https://doi.org/10.3390/dermatopathology8030034 - 8 Jul 2021
Cited by 1 | Viewed by 4214
Abstract
Mycosis fungoides (MF) is a subtype of primary cutaneous T-cell lymphoma (CTCL) with an indolent course that rarely progresses. Histologically, the lesions display a superficial lymphocytic infiltrate with epidermotropism of neoplastic T-cells. Hypopigmented MF is a rare variant that presents with hypopigmented lesions [...] Read more.
Mycosis fungoides (MF) is a subtype of primary cutaneous T-cell lymphoma (CTCL) with an indolent course that rarely progresses. Histologically, the lesions display a superficial lymphocytic infiltrate with epidermotropism of neoplastic T-cells. Hypopigmented MF is a rare variant that presents with hypopigmented lesions and is more likely to affect young patients. The etiology of the hypopigmentation is unclear. The aim of this study was to assess melanocyte loss in MF through immunohistochemistry (IHC) with SOX10. Twenty cases were evaluated, including seven of the hypopigmented subtype. The neoplastic epidermotropic infiltrate consisted predominantly of CD4+ T-cells in 65% of cases; CD8+ T-cells were present in moderate to abundant numbers in most cases. SOX10 IHC showed a decrease or focal complete loss of melanocytes in 50% of the cases. The predominant neoplastic cell type (CD4+/CD8+), age, race, gender, histologic features, and reported clinical pigmentation of the lesions were not predictive of melanocyte loss. A significant loss of melanocytes was observed in 43% of hypopigmented cases and 54% of conventional cases. Additional studies will increase our understanding of the relationship between observed pigmentation and the loss of melanocytes in MF. Full article
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<p>Mycosis fungoides presenting as a scaly, erythematous patch in a Caucasian patient.</p> Full article ">Figure 2
<p>Mycosis fungoides presenting as hypopigmented macules and patches in the upper extremity of an African American patient.</p> Full article ">Figure 3
<p>Ten cases displayed melanocyte loss by SOX10 IHC. The loss was observed in both conventional and hypopigmented cases (<b>a</b>) and in CD8 or CD4 predominant cases (<b>b</b>).</p> Full article ">Figure 4
<p>Sample case with a predominant CD8+ epidermotropic T-cell infiltrate with melanocytes in normal quantities and distribution highlighted by SOX10 IHC. The patient was a 40-year-old male with persistent pink to hyperpigmented patches in sun-protected areas. (<b>a</b>) Hematoxylin and eosin stained section, original magnification 100×. (<b>b</b>) T-cells highlighted by a CD4 IHC stain, original magnification 100×. (<b>c</b>) T-cells highlighted by a CD8 IHC stain, original magnification 100×. (<b>d</b>) SOX10 IHC highlighting melanocytes, original magnification 100×.</p> Full article ">Figure 5
<p>Sample case with a predominant CD4+ epidermotropic T-cell infiltrate with melanocyte loss shown by complete focal loss of SOX10 IHC staining. The patient was an 86-year-old male with a long history of hyperpigmented rash in sun-protected areas. (<b>a</b>) Hematoxylin and eosin-stained section, original magnification 100×. (<b>b</b>) T-cells highlighted by a CD4 IHC stain, original magnification 100×. (<b>c</b>) T-cells highlighted by a CD8 IHC stain, original magnification 100×. (<b>d</b>) Negative SOX10 IHC staining, original magnification 100×.</p> Full article ">
6 pages, 1950 KiB  
Review
“Animal-Type Melanoma/Pigmented Epithelioid Melanocytoma”: History and Features of a Controversial Entity
by Gerardo Cazzato, Francesca Arezzo, Anna Colagrande, Antonietta Cimmino, Teresa Lettini, Sara Sablone, Leonardo Resta and Giuseppe Ingravallo
Dermatopathology 2021, 8(3), 271-276; https://doi.org/10.3390/dermatopathology8030033 - 5 Jul 2021
Cited by 8 | Viewed by 3187
Abstract
Animal-type melanoma (ATM) was first described in the literature by Levene in 1979 in relation to a patient with a characteristic clinical presentation, and only later, rare and anecdotal case series have tried to shed light on an entity that has undergone several [...] Read more.
Animal-type melanoma (ATM) was first described in the literature by Levene in 1979 in relation to a patient with a characteristic clinical presentation, and only later, rare and anecdotal case series have tried to shed light on an entity that has undergone several nosographic classification changes, and which, since 2018, is classified under the term “pigmented epithelioid melanocytoma”. Here, we conduct a brief review of the current literature on ATM and present a new clinical case with histopathological, immunophenotypic, and molecular investigations. Full article
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<p>(<b>A</b>) Skin and subcutis including intensely pigmented melanocyte proliferation. (Hematoxylin-Eosin, Original Magnification: 4×). (<b>B</b>) Histological detail of melanocyte proliferation consisting of variously intertwined cells, with evident presence of melanic pigment. (Hematoxylin-Eosin, Original Magnification: 10×). (<b>C</b>) Immunohistochemical preparation with anti-Melan-A antibody which highlights the melanocyte proliferation in question (Immunohistochemistry, Original Magnification: 4×). (<b>D</b>) Detail of micrometastases in the sentinel lymph node (Hematoxylin-Eosin, Original Magnification: 4×).</p> Full article ">
6 pages, 1386 KiB  
Review
The Multiple Faces of Nodular Trichoblastoma: Review of the Literature with Case Presentation
by Gerardo Cazzato, Antonietta Cimmino, Anna Colagrande, Francesca Arezzo, Lucia Lospalluti, Sara Sablone, Teresa Lettini, Leonardo Resta and Giuseppe Ingravallo
Dermatopathology 2021, 8(3), 265-270; https://doi.org/10.3390/dermatopathology8030032 - 5 Jul 2021
Cited by 8 | Viewed by 6421
Abstract
Trichoblastoma (TB) is a rare biphasic benign adnexal neoplasm originating from follicular germ cells but clinically, it can simulate basal cell carcinoma (BCC), making the diagnosis more difficult. There are several variants of Trichoblastoma and a good knowledge of these is essential for [...] Read more.
Trichoblastoma (TB) is a rare biphasic benign adnexal neoplasm originating from follicular germ cells but clinically, it can simulate basal cell carcinoma (BCC), making the diagnosis more difficult. There are several variants of Trichoblastoma and a good knowledge of these is essential for correct diagnosis and management. We report two new cases observed in the last year at our Pathological Anatomy Operative Unit, and conduct a careful review of the literature, from the first description of this lesion by Headington in 1970 to the most recent classifications. Full article
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<p>Clinical features of the “Case 1” lesion.</p> Full article ">Figure 2
<p>(<b>A</b>) Clinical features of the “Case 2” lesion, well circumscribed, symmetrical, smooth bordered, skin-colored pinkish or brown, at the level of the passage from the tip to the right nasal wing. (<b>B</b>) Dermoscopic features of TB with arborizing vessels and teangiectasias.</p> Full article ">Figure 3
<p>Proliferation of numerous small and irregular nests of basaloid cells with no obvious connection to the epidermis (hematoxylin and eosin, original magnification, (<b>A</b>) 40× (<b>B</b>) 100×).</p> Full article ">Figure 4
<p>Proliferation of large nests of basaloid cells without connection to the epidermis (hematoxylin and eosin, original magnification, (<b>A</b>) 40× (<b>B</b>) 100×).</p> Full article ">
7 pages, 3243 KiB  
Article
GLUT1 Expression in Cutaneous Sebaceous Lesions Determined by Immunohistochemical Staining Patterns
by Cynthia Reyes Barron and Bruce R. Smoller
Dermatopathology 2021, 8(3), 258-264; https://doi.org/10.3390/dermatopathology8030031 - 5 Jul 2021
Cited by 1 | Viewed by 3036
Abstract
GLUT1 is a membrane associated carrier protein that functions in the physiologic transport of glucose across cell membranes. Multiple studies have shown an increased GLUT1 expression in various tumor types and a role in cancer prognosis. The aim of this study was to [...] Read more.
GLUT1 is a membrane associated carrier protein that functions in the physiologic transport of glucose across cell membranes. Multiple studies have shown an increased GLUT1 expression in various tumor types and a role in cancer prognosis. The aim of this study was to determine whether cutaneous sebaceous lesions have a differential expression of GLUT1 by immunohistochemistry (IHC). GLUT1 IHC was performed on excision specimens of ten cases of sebaceous carcinoma, nine of sebaceoma, ten of sebaceous adenoma, and ten of sebaceous hyperplasia. Intense, diffuse cytoplasmic staining was observed in sebaceous carcinoma. The pattern of GLUT1 staining in sebaceomas and sebaceous adenomas consisted of a gradient of intense cytoplasmic staining in the basaloid cells with a decreased intensity to membranous staining only and absent staining in mature sebaceous cells. In lesions of sebaceous hyperplasia, GLUT1 staining outlined the basal layer of each gland; cytoplasmic staining was minimal to absent. Increased cytoplasmic staining of GLUT1 may correlate with cellular metabolic and proliferative activity. GLUT1 has potential utility in differentiating sebaceous lesions. Full article
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<p>Sebaceous carcinoma, sample case. (<b>a</b>) Hematoxylin and eosin-stained section showing an infiltrative proliferation of basaloid cells with focal sebaceous differentiation and abundant mitoses, original magnification 100×; (<b>b</b>) GLUT1 immunohistochemical stain of the same lesion showing strong diffuse cytoplasmic and membranous staining throughout the tumor, original magnification 100×.</p> Full article ">Figure 2
<p>Sebaceoma, sample case. (<b>a</b>) Hematoxylin and eosin-stained section showing a well-circumscribed dermal tumor consisting predominantly of basaloid cells with minimal cytologic atypia and mitoses, original magnification 100×; (<b>b</b>) GLUT1 immunohistochemical stain of the same tumor showing diffuse strong cytoplasmic and membranous staining in the basaloid cells with weaker staining in cells with greater maturation, original magnification 100×.</p> Full article ">Figure 3
<p>Sebaceous adenoma, sample case. (<b>a</b>) Hematoxylin and eosin-stained section showing a well-circumscribed dermal-based tumor consisting predominantly of mature sebocytes with a basaloid component comprising less than 50% of the tumor cells. Cytologic atypia and mitoses are not prominent, original magnification 100×; (<b>b</b>) GLUT1 immunohistochemical stain of the same tumor showing cytoplasmic and membranous staining in the basaloid component of the tumor with weak to absent staining in mature sebocytes, original magnification 100×.</p> Full article ">Figure 4
<p>Sebaceous hyperplasia, sample case. (<b>a</b>) Hematoxylin and eosin-stained section showing a benign proliferation of sebaceous glands with a single layer of basaloid cells surrounding each lobule, original magnification 100×; (<b>b</b>) GLUT1 immunohistochemical stain of the same tumor showing primarily membranous staining only in the basaloid cells at the periphery of each lobule with staining becoming weaker to completely absent in the central mature sebocytes, original magnification 100×.</p> Full article ">
5 pages, 10532 KiB  
Case Report
A Wolf in Sheep’s Clothing: Collision of Melanoma and Keratoacanthoma
by Matthias Walther, Sandra Falkvoll and Sebastian Leibl
Dermatopathology 2021, 8(3), 253-257; https://doi.org/10.3390/dermatopathology8030030 - 4 Jul 2021
Viewed by 5136
Abstract
Collision tumors consisting of melanoma and squamous cell carcinoma are very rare. We present the case of a deceptive hyperkeratotic nodule on the forearm of a 72-year-old woman, which clinically appeared to be a squamous cell carcinoma, keratoacanthoma type. Histological examination surprisingly revealed [...] Read more.
Collision tumors consisting of melanoma and squamous cell carcinoma are very rare. We present the case of a deceptive hyperkeratotic nodule on the forearm of a 72-year-old woman, which clinically appeared to be a squamous cell carcinoma, keratoacanthoma type. Histological examination surprisingly revealed a coexisting epithelioid melanoma. Thus, this case report shows the importance of an early histopathological and immunohistochemical workup to prevent unnecessary diagnostic and therapeutic delay with negative effects on prognosis. Full article
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<p>Macroscopy, hyperkeratotic nodule on forearm.</p> Full article ">Figure 2
<p>Collision tumor consisting of keratoacanthoma and melanoma, Hematoxylin and Eosin stain (H&amp;E). The small insert on the upper right side shows the edge of the keratoacanthoma with characteristic architecture and large pale keratinocytes.</p> Full article ">Figure 3
<p>Detail of the invasion front: Large pale keratinocytes and epithelial nests with micro abscesses in a background of spindle-shaped melanoma cells (H&amp;E stain).</p> Full article ">Figure 4
<p>High molecular weight cytokeratin (clone 34βE12) stain highlighting the epithelial component of the collision tumor.</p> Full article ">Figure 5
<p>Melan-A immunohistochemical stain highlighting the melanoma component of the collision tumor.</p> Full article ">
17 pages, 10399 KiB  
Review
Histological Patterns of Skin Lesions in Tuberous Sclerosis Complex: A Panorama
by Marine Cascarino and Stéphanie Leclerc-Mercier
Dermatopathology 2021, 8(3), 236-252; https://doi.org/10.3390/dermatopathology8030029 - 4 Jul 2021
Cited by 10 | Viewed by 11155
Abstract
Tuberous Sclerosis Complex (TSC) is a multisystem genetic disease characterized by cutaneous and extracutaneous hamartomas. The diagnosis is based on the association of major and minor criteria, defined by a consensus conference updated in 2012. The clinical examination of the skin is crucial [...] Read more.
Tuberous Sclerosis Complex (TSC) is a multisystem genetic disease characterized by cutaneous and extracutaneous hamartomas. The diagnosis is based on the association of major and minor criteria, defined by a consensus conference updated in 2012. The clinical examination of the skin is crucial because seven diagnostic criteria are dermatological: four major (hypomelanotic macules, angiofibroma or fibrous cephalic plaques, ungual fibromas, shagreen patches) and three minor criteria (confetti skin lesions, dental enamel pits, intraoral fibromas). Skin biopsy is commonly performed to assert the diagnosis of TSC when the clinical aspect is atypical. Histopathology of TSC cutaneous lesions have been poorly reported until now. In this article, we review the histologic features described in the literature and share our experience of TSC skin biopsies in our pediatric hospital specialized in genetic disorders. Both hypomelanotic lesions and cutaneous hamartomas (angiofibroma/fibrous cephalic plaques, ungual fibromas, shagreen patches) are discussed, including the recent entity called folliculocystic and collagen hamartoma, with a special emphasis on helpful clues for TSC in such lesions. Full article
(This article belongs to the Special Issue New Insights in Pediatric Dermatopathology)
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<p>Hypomelanotic macules. (<b>a</b>): Ash leaf spot hypomelanotic lesion in a 7 year-old boy’s arm; (<b>b</b>): lance-ovate hypomelanotic macules in a 4 months boy’s back.</p> Full article ">Figure 2
<p>Angiofibroma. (<b>a</b>) Face’s angiofibromas in a 14 year-old patient; (<b>b</b>) Hematein eosin ×40: hypertrophy of the collagen bundles and of the vascular elements represented by dilated venules; (<b>c</b>). Orcein staining ×40: decreased and fragmented elastic fibers.</p> Full article ">Figure 3
<p>Periungual fibroma. (<b>a</b>). Clinical aspect of a toe periungual fibroma; (<b>b</b>). Hematein eosin ×5: exophytic dome-shaped lesion with hyperplastic epidermis; (<b>c</b>). Hematein eosin ×20: collagen bundles are vertically oriented; increased number of dilated venules; (<b>d</b>). Orcein staining × 40: decreased elastic fibers; (<b>e</b>). Immuno-histochemistry with anti-FXIIIa antibody showing positivity of stellate cells.</p> Full article ">Figure 3 Cont.
<p>Periungual fibroma. (<b>a</b>). Clinical aspect of a toe periungual fibroma; (<b>b</b>). Hematein eosin ×5: exophytic dome-shaped lesion with hyperplastic epidermis; (<b>c</b>). Hematein eosin ×20: collagen bundles are vertically oriented; increased number of dilated venules; (<b>d</b>). Orcein staining × 40: decreased elastic fibers; (<b>e</b>). Immuno-histochemistry with anti-FXIIIa antibody showing positivity of stellate cells.</p> Full article ">Figure 4
<p>Fibrous cephalic plaque. (<b>a</b>): Clinical aspect of a fibrous cephalic plaque of the forehead; (<b>b</b>): Hematein eosin ×10: dense proliferation of collagen bundles in the dermis and the hypodermis; (<b>c</b>). Concentric perifollicular fibrosis leading to atrophy and compression of the follicle.</p> Full article ">Figure 5
<p>Shagreen patch. (<b>a</b>). Clinical aspect of a lumbar shagreen patch; (<b>b</b>). Hematein eosin ×20: extensive fibrosis with collagen bundles into the dermis and the hypodermis; (<b>c</b>). Hematein eosin ×20: concentric perifollicular fibrosis; (<b>d</b>). Orcein staining ×40: lack of elastic fibers.</p> Full article ">Figure 5 Cont.
<p>Shagreen patch. (<b>a</b>). Clinical aspect of a lumbar shagreen patch; (<b>b</b>). Hematein eosin ×20: extensive fibrosis with collagen bundles into the dermis and the hypodermis; (<b>c</b>). Hematein eosin ×20: concentric perifollicular fibrosis; (<b>d</b>). Orcein staining ×40: lack of elastic fibers.</p> Full article ">Figure 6
<p>Folliculocystic Hamartoma. (<b>a</b>): clinical aspect of an occipital FCCH; (<b>b</b>): Hematein eosin ×10: extensive fibrosis and concentric perifollicular fibrosis; (<b>c</b>): Hematein eosin ×10: comedo openings; (<b>d</b>): Hematein eosin ×10: large infundibular cyst.</p> Full article ">Figure 6 Cont.
<p>Folliculocystic Hamartoma. (<b>a</b>): clinical aspect of an occipital FCCH; (<b>b</b>): Hematein eosin ×10: extensive fibrosis and concentric perifollicular fibrosis; (<b>c</b>): Hematein eosin ×10: comedo openings; (<b>d</b>): Hematein eosin ×10: large infundibular cyst.</p> Full article ">Figure 7
<p>Common and distinctive histologic findings in TSC cutaneous hamartoma. (<b>a</b>): Hematein eosin ×10: fibrosis component: extensive fibrosis composed of thick collagen bundles; (<b>b</b>): Hematein eosin ×20: vascular component made of dilated vessels and thick collagen bundles; (<b>c</b>): Hematein eosin ×40: cellular component: stellate cells; (<b>d</b>): Hematein eosin ×10: Concentric perifollicular fibrosis; (<b>e</b>): Hematein eosin ×10: Dilated infundibular cyst; (<b>f</b>): Orcein staining: decreased or lack of elastic fibers.</p> Full article ">Figure 7 Cont.
<p>Common and distinctive histologic findings in TSC cutaneous hamartoma. (<b>a</b>): Hematein eosin ×10: fibrosis component: extensive fibrosis composed of thick collagen bundles; (<b>b</b>): Hematein eosin ×20: vascular component made of dilated vessels and thick collagen bundles; (<b>c</b>): Hematein eosin ×40: cellular component: stellate cells; (<b>d</b>): Hematein eosin ×10: Concentric perifollicular fibrosis; (<b>e</b>): Hematein eosin ×10: Dilated infundibular cyst; (<b>f</b>): Orcein staining: decreased or lack of elastic fibers.</p> Full article ">Figure 7 Cont.
<p>Common and distinctive histologic findings in TSC cutaneous hamartoma. (<b>a</b>): Hematein eosin ×10: fibrosis component: extensive fibrosis composed of thick collagen bundles; (<b>b</b>): Hematein eosin ×20: vascular component made of dilated vessels and thick collagen bundles; (<b>c</b>): Hematein eosin ×40: cellular component: stellate cells; (<b>d</b>): Hematein eosin ×10: Concentric perifollicular fibrosis; (<b>e</b>): Hematein eosin ×10: Dilated infundibular cyst; (<b>f</b>): Orcein staining: decreased or lack of elastic fibers.</p> Full article ">
7 pages, 2211 KiB  
Case Report
Primary Localized Cutaneous Nodular Amyloidosis and Limited Cutaneous Systemic Sclerosis: Additional Cases with Dermatoscopic and Histopathological Correlation of Amyloid Deposition
by Laura Atzori, Caterina Ferreli, Caterina Matucci-Cerinic, Luca Pilloni and Franco Rongioletti
Dermatopathology 2021, 8(3), 229-235; https://doi.org/10.3390/dermatopathology8030028 - 2 Jul 2021
Cited by 7 | Viewed by 5651
Abstract
Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare condition due to the plasma cell proliferation and skin deposition of immunoglobulin light chains, without systemic amyloidosis or hematological dyscrasias. The association with autoimmune connective tissue diseases has been reported, especially with Sjogren’s syndrome, [...] Read more.
Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare condition due to the plasma cell proliferation and skin deposition of immunoglobulin light chains, without systemic amyloidosis or hematological dyscrasias. The association with autoimmune connective tissue diseases has been reported, especially with Sjogren’s syndrome, and in a few cases with systemic sclerosis. Herein, we describe three cases of PLCNA occurring in women with a diagnosis of limited cutaneous systemic sclerosis and review the literature on the topic to highlight a stereotypical presentation. Moreover, we support the usefulness of dermoscopy, characterized by a yellow–orange waxy pattern surrounded by telangiectasias, for a rapid and non-invasive diagnostic assessment. Thus, when asymptomatic nodules occur on lower limbs of women affected with limited systemic sclerosis, and dermoscopy identifies yellow–orange blotches, a diagnosis of PLCNA can be considered and further confirmed by histopathology. Monitoring for systemic amyloidosis development is advisable, although the risk of progression is considered very low. Full article
(This article belongs to the Special Issue In Memory of Raffaele Gianotti)
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<p>Clinical examination. (<b>A</b>) Case 1: residual well demarcated, linear orange-yellow plaque on the left leg with a white scar from a previous diagnostic biopsy; (<b>B</b>) Case 2: dome-shaped, yellow–pinkish nodule, and isolated papules of her left leg; (<b>C</b>) Case 3: multiple yellow–purple nodular lesions of the left leg.</p> Full article ">Figure 2
<p>Dermoscopy. (<b>A</b>) Case 1: a structureless yellow background interspersed with whitish scar-like strikes in Case 1; (<b>B</b>) Case 2: roundish yellow waxy blotches on a hemorrhagic background, interspersed with fine telangiectasias and hemorrhagic spots; (<b>C</b>) Case 3: structureless yellow background interspersed with whitish spots, surrounded by a hemorrhagic halo with elongated serpentine vessels.</p> Full article ">Figure 3
<p>Histopathological findings of Case 2. (<b>A</b>). Nodular deposits of amorphous eosinophilic material in the dermis and subcutis (original magnification ×40) (<b>B</b>). Focal microcalcification inside the hyaline material (original magnification ×100) (<b>C</b>). Peripheral patchy focal infiltrate of lymphocytes with many plasma cells (original magnification ×400) (<b>D</b>). Amorphous material intensely stained with Congo red (original magnification ×100) (<b>E</b>). Amorphous material intensely stained with crystal violet (original magnification ×100) (<b>F</b>). Positive apple green birefringence under polarized light (original magnification ×200).</p> Full article ">Figure 4
<p>Histopathological findings of Case 3. (<b>A</b>) presence of a hyaline-like, amorphous eosinophilic material in the dermis surrounding and involving dermal vessels. (HE original staining ×40); (<b>B</b>) A perivascular and interstitial lymphocytic infiltrate (original magnification ×100) with (<b>C</b>) focal plasma cells component (original magnification ×400). (<b>D</b>) Congo red stained positively the amorphous eosinophilic deposits of amyloid in the dermis, surrounding deep dermal vessels (original magnification ×100). Immunohistochemical studies for (<b>E</b>) lambda and (<b>F</b>) kappa light chains showed evidence of lambda light-chain restriction, consistent with a monoclonal plasma cell proliferation.</p> Full article ">
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