선단각화 유탄력섬유증 1예

Abstract

Acrokeratoelastoidosis (AKE) is a rare skin disorder initially described by Costa, which is inherited by autosomal dominant, but also may be sporadic. Clinically, it consists of small, firm papules with occasional keratosis or umbilication, characteristically along the margins of hands and feet. Histopathologically, it shows hyperkeratosis, acanthosis in the epidermis and fragmentation and rarefaction of elastic fibers-elastorrhexis-in the dermis. A 32-year-old man presented with multiple papules along the border of the hands and feet, and from histopathology, AKE was diagnosed. Herein we report a case of acrokeratoelastoidosis and review the clinical and histopathologic features, etiology, differential diagnosis and treatment.