A Case of Incontinentia Pigmenti / 대한피부과학회지

ABSTRACT

Incontinentia pigmenti is an uncommon genodermatosis. It affects female infants predominantly, described first by Bardach in l925, with the diagnosis of systematized nevus and Bloch in 1926, and Sulzberger in 1928 rnore compIetely. Skin lesions are characterized by 3 stages such as vesicobullous, verucous and finally pigmentary lesions and leave brownish pigmentation on the extremities and trunk. Hesides skin lesions some ectodermaI and mesodermal organs are affected and show developmental abnormalities. We experienced a case of incontinentia pigmenti in a 45-day-old female infant and present it with the review of literature. She showed extensive vesicobullopustular eruption with linear and reticular pigmentation on the extremities and trunk. Clinical and histopathologic findings of these lesions are compatible with Bloch-Sulzberger type of incontinentia pigmenti.