Congenital Poikiloderma With Features of Hereditary Acrokeratotic Poikiloderma

Abstract

An adolescent boy with a bizarre progressive poikiloderma had episodic acral blistering and the development of palmar and plantar keratosis suggestive of hereditary acrokeratotic poikiloderma. Growth and development had been normal. Vesicobullous lesions were said to be present at birth and during early infancy. No family members were similarly affected. Results of laboratory studies were normal. Histological sections of skin demonstrated epidermal atrophy in some areas, increased epidermal melanin fibrosis of the papillary dermis, and modest capillary proliferation.