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Which of the following is not associated with diabetes?

A. Xanthomas

B. Syringomas

C. Dermatofibromas

D. Lipomas

E. Rubeosis

C. Diabetes is associated with eruptive xanthomas, clear cell syringomas, lipomas, and rubeosis (flushed appearance improved by dietary diabetic control). Multiple eruptive dermatofibromas have been reported in patients with systemic lupus erythematous, atopic dermatitis, and immunosuppression.

Which of the following is not associated with steatocystomas?

A. Hidrotic ectodermal dysplasia

B. Anhidrotic ectodermal dysplasia

C. Pachonychia congenita (type 1)

D. Pachonychia congenita (type 2)

E. All are associated

C. Pachyonychia congenital type 1 (Jadassohn-Lewandowsky) is an ectodermal dysplasia characterized by nail dystrophy, follicular hyperkeratosis, and angular cheilitis. It is distinguished from pachyonychia congenital type 2 (Jackson-Lawler) at puberty, when type 2 patients develop steatocystoma multiplex and eruptive vellus hair cysts.

A patient presents with multiple firm, rubbery pink nodules on the face, some are coalescing into a plaque covering the scalp. Which is the most likely gene defect?

A. BRAF

B. CYLD

C. NF1

D. NFKB

E. P53

B. Multiple cylindromas and some coalescing into a large plaque covering the scalp (turban tumor) is a characteristic of Brooke-Spiegler syndrome (familial cylindromatosis). Both this Multiple Familial Trichoepitheliomas are autosomal dominant disorders of the CYLD gene (chromosome 16q), which encodes a deubiquitinating enzyme.

Which is not a feature of microcystic adnexal carcinoma?

A. Subtle clinical presentation

B. Large plaque on nose or upper lip

C. Biphasic histologic appearance

D. Perineural involvement

E. Marked cytologic atypia

E. The lack of cytologic atypia sometimes makes differentiation from desmoplastic trochiepithelioma difficult. Infiltrative plaques on the midface tend to be larger than they appear clinically, histologically appears as basaloid strands in the upper dermis but as ductal structures in the deeper dermis, and frequently have perineural involvement.

A patient presents with brightly erythematous eczematous patches on the perineal ...

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