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Anhidrotic ectodermal dysplasia with eruptive vellus hair cysts
Author(s) -
Köse Osman,
Tastan Halis Bülent,
Deveci Salih,
Gür Ali Riza
Publication year - 2001
Publication title -
international journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 93
eISSN - 1365-4632
pISSN - 0011-9059
DOI - 10.1046/j.1365-4362.2001.01246.x
Subject(s) - vellus hair , forehead , frontal bossing , medicine , anatomy , scalp , papule , canthus , dermatology , pathology , eyelid , ophthalmology , lesion
A 21‐year‐old white man complained of heat intolerance, absence of sweating, and papular lesions on the forehead. Facial features included a saddle nose, prominent supraorbital edge, and frontal bossing. The scalp hair was scanty, fine, and brittle. Eyelashes and eyebrows were also thin. Subungual hyperkeratosis and dystrophic changes were observed on the finger and toe nails. Hypodontic teeth were detected on oral examination. He was otherwise healthy. Biochemical and hematologic analyses were also normal. Cutaneous examination revealed smooth and dry skin. Multiple, 4–5 mm, brown–black, comedo‐like, soft papules were observed on the forehead ( Fig. 1). A biopsy specimen from a papule on the forehead revealed cystic structures lined by squamous epithelium and containing laminated keratinous material and scattered obliquely and transversely cut vellus hairs ( Fig. 2). Topical application of 0.1% tretinoin for 2 months produced no clinical improvement. 1Scanty and fine scalp hair, prominent supraorbital edge and frontal bossing on the face, and brown–black comedo‐like soft papules (arrows) on the forehead2Luminal vellus hairs and laminated keratinous material (hematoxylin and eosin, × 200)