Hyperkeratosis in Flegel's disease

Autor: Veronika Burmeister, Rajagopala Swaminathan
Rok vydání: 1985
Předmět:
Zdroj: JAMA. 254(6)
ISSN: 0098-7484
Popis: Hyperkeratosis lenticularis perstans was first described by Flegel1in 1958. It may rightly be redefined as an autosomal dominant mucocutaneous syndrome of variable expressivity, characterized by an aberration in the keratinization process. A 67-year-old woman presented with numerous asymptomatic keratotic lesions, confined to the anterior aspect of the legs and the dorsa of the feet. These skin-colored papules varied in size from 1 to 4 mm in diameter. Other manifestations were palmar pits and mucosal lesions on the tongue. The patient was treated with vitamin A (Aquasol A) and topical tretinoin 0.01% gel (Retin-A), with some response. The lack of patient compliance ruled out the use of isotretinoin (Accutane). A specimen from a skin biopsy was examined under a light microscope and displayed massive hyperkeratosis and spotty parakeratosis, with the granular layer being absent in most areas except in the relatively normal margin. There was some irregular acanthosis in
Databáze: OpenAIRE