Korean J Dermatol.  1998 Oct;36(5):946-949.

Two Familial Cases of Acrokeratoelastoidosis of Costa with Autosomal Dominant Inheritance

Abstract

Acrokeratoelastoidosis of Costa is a rare palmoplantar keratoderma with autosomal dominant inheritance. It is clinically charaeterized by small, firm, yellowish, shiny, translucent papules occumng over the dorsal hands, the knuckles, and the lateral margine of the palms and soles. Histologically, the characteristic features are hyperkeratosis, aeanthosis, and most strikingly, fragmentation of coarse elastic fibers within the dermis. The lesions usually begin in early childhood and progress slowly. We herein report two familial cases of acrokeratoelastoidosis of Costa showing typical clinic1 and histopathological features.


MeSH Terms

Dermis
Elastic Tissue
Hand
Keratoderma, Palmoplantar
Wills*
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