All issues > Volume 36(3); 1993

Case Report

J Korean Pediatr Soc. 1993;36(3):428-433. Published online March 15, 1993.

A Case of Incontinentia Pigmenti

Soo Am SA Chung¹, Won Rae WR Kim¹, Hyung Kun HK Nam¹, Jin Sam JS No¹, Jung Hee JH Park²

¹Department of Pediatrics, St. Francisco General Hospital, Seoul, Korea
²Department of Anatomical Pathology, St. Francisco General Hospital, Seoul, Korea

Abstract

Incontinentia pigmenti (Bloch-Sulzberger syndrome)is a genetic disease of the skin with generalized ectodermal and mesodermal dysplasia. Skin lesions are uually present at birth or shortly after in the form of eythermatous eruptions with linear vesiculations. The final stage is characterised by irregular macular streaks of brown to slate-grey pigmentation. We experienced five cases of incontinentia pigmenti maternal female relative of a three generation.

Keywords :Incontinentia pigmenti, Bloch-Sulzberger syndrome