Congenital poikiloderma with unusual hypopigmentation and acral blisteringat birth

Congenital poikiloderma is an uncommon hereditary disorder. It has been rep orted in association with various syndromes. No case has previously been re ported from this environment. We report a case of congenital poikiloderma i n a two and a half year old female Nigerian associated with unusual general ised hypopigmentation and acral blisters at birth. The child subsequently d eveloped macular hyperpigmentation on an erythematous background and atroph y of the skin. Although she had some features which were suggestive of Roth mund-Thomson syndrome (RTS), the presence of hypopigmentation at birth, alo ng with acral blistering, was noted to be peculiar to this child. We, there fore, feel that this case presents a distinct variant of congenital poikilo derma that has not been described previously. (C) 1999 Elsevier Science B.V . All rights reserved.