Two Familial Cases of Acrokeratoelastoidosis of Costa with Autosomal Dominant Inheritance.
- Author:
Sang Hee HAM
;
Seog Jun HA
;
Young Min PARK
;
Sang Hyun CHO
;
Baik Kee CHO
- Publication Type:Case Report
- MeSH:
Dermis;
Elastic Tissue;
Hand;
Keratoderma, Palmoplantar;
Wills*
- From:Korean Journal of Dermatology
1998;36(5):946-949
- CountryRepublic of Korea
- Language:Korean
-
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.
