Cowden’s Syndrome: Dermatological Features

Abstract

The skin manifestations of Cowden’s syndrome include three common lesions:

Facial trichilemmomas: 1–5 mm diameter, flesh-colored, flat-topped or elongated verrucous papules in periorofacial and centrofacial distribution

Papillomatous papules: small, smooth, flesh-colored papules on labial, gingival, palatal surfaces leading to a cobblestone appearance; also occur on face and pressure-points (palmar/plantar surfaces) where they tend to be more translucent, hard papules

Acral keratoses: flesh-colored, flat-topped, rough papules on the dorsum of the hands or feet that resemble common warts; can be pits if small or microplaques/papules if larger

Less common skin manifestations include lipomas, hemangiomas (associated with related Bannayan-Riley-Ruvalcaba syndrome), scrotal tongue, neuromas