dermatology
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Clinical features •Extremely rare cutaneous tumor •Variably considered a variant of squamous cell carcinoma or an entity sui generis representing the cutaneous equivalent of nasopharyngeal lymphoepithelioma-like carcinoma; lastly is the possibility of an adnexal carcinoma •Head & neck with predilection for sun-damaged skin; solitary flesh colored or erythematous, firm nodule or plaque •Exceptionally presents elsewhere e.g., trunk & arm •Elderly (39-96 years; mean 70 years) •M=F…
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Currently classified as a variant of mycosis fungoides overlapping with granulomatous mycosis fungoides. It has been postulated by some that the entity is a reflection of the site of involvement (axiallae & groins) rather than indicating a separate disease. Due to its rarity, further larger series are needed to substantiate this hypophysis. Clinical features •Exceedingly…
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Dystrophic epidermolysis bullosa (DEB) develops as a consequence of mutations in COL7A1. This results in defective or absent anchoring fibrils. As a consequence the skin is susceptible to shearing blisters with even minor trauma. Repeated blistering and ulceration complicated by severe scarring results. Squamous cell carcinoma (SCC) is most often encountered on the extremities. Current…
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Clinical features •Rare & may present in the dermis, subcutaneous fat or soft tissue •Cutaneous lesions present mostly in adolescents & young adults with a predilection for males •Firm to hard nodule 0.5-2.5 cm •Age range newborn-93 years •Soft tissue tumors are often much larger & present most often limbs & limb girdles with an equal sex incidence •Malignant myoepithelioma in the soft tissues is exceedingly rare and has a…
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Papillary eccrine adenoma & tubular apocrine adenoma are very similar and some authors combine the two entities under the umbrella term “tubular adenoma”. While this has merit, there are clinical differences. Papillary eccrine adenoma arises most often on the extremities and shows a marked predilection for black females (9F:1M). Tubular apocrine adenoma most commonly arises…
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Clinical features •Trichoblastic carcinoma, sarcoma & carcinosarcoma •Exceedingly rare, most cases have arisen on the face & scalp of the elderly but may present at a wide variety of sites •Exceptional complicating nevus sebaceus •Average age 65 •M>F •Exceptionally familial/syndromic e.g. Brooke-Spiegler syndrome •Some authors divide tumors into subtypes- low-grade presenting predominantly on the face (with risk of recurrence but no metastatic potential) & high-grade presenting at a wide…
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Rare sweat gland carcinoma which may be confused with a papillary metastasis and should be differentiated from papillary eccrine adenoma. Recently has been shown to be associated with HPV42 in the majority of cases Leiendecker et al 2023). A small subset is associated with mutations in BRAF V600E. Clinical features •Very rare, often aggressive •Volar fingers,…
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This tumor occurs exclusively in females and is thought to represent an external genital equivalent to breast intraduct papilloma. It is probably derived from the ano-genital mammary-like glands Clinical features •Almost all cases have arisen in females, generally white & young-middle-aged •Occasionally symptomatic- pruritus, pain etc •Male cases represent misdiagnosed cystic apocrine hidradenoma •Small, asymptomatic solid/cystic papule…
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Clinical features •Very rare benign tumor that shows a predilection for the distal extremities •4F:1M •Black> White races •Slowly growing, asymptomatic, flesh colored or pink nodule (0.5-4 cm diameter) Histological features •Circumscribed nodule composed of ducts & cysts embedded in a dense fibrous stroma sometimes associated with lymphoid aggregates/germinal centers •Ducts lined by double-layered epithelium- small inner, uniform cells with eosinophilic…
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Clinical features •Tender or painful, sharply delineated typically blue 1-3 cm dermal nodule nodule •Ventral skin, most on the upper half of the integument (head, neck & trunk) •Rarely multiple lesions 2nd-4th decade •Giant variant •Linear, Blaschkoid, variants •Autosomal dominant familial variant •May occur as part of the Brooke-Spiegler syndrome Histological features •Nodule/multiple blue encapsulated nodules in the…
McKee Derm 