Incontinentia pigmenti. A case report.

Abstract

: Incontinentia pigmenti (IP) is an uncommon X-linked dominant genodermatosis, can affects the skin, eyes, teeth, and may be associated with neurological defects. Changes of skin that are always present are usually combined with anomalies in skin appendages and in other organs. A 2 month female baby, with chief complaint brown patches on her body. Her mother said that brown patches on her body since 1 month, at first it was red patch with blister, then dry and left rough mark. Dermatological examination on regio thoracalis anterior et posterior, brachii dextra et sinistra, femoralis dextra et sinistra found linear hiperpigmentation macule, along blaschko line, no scale, no verucosa. Histopathological examination on epidermis there were hiperkeratosis basal cell, no spongiosis and on dermis there were melanin within macrophage along superficial dermis. In this case, there was a skin anomali but with no other organ involvement. This patient is female, with history of her mother miscarriage in second child. Although the skin lesions of IP appear impressive, little treatment is needed as they will gradually clear on their own. Parents should be appropriately counseled about the expected course of the disease. A bland emollient can be applied to inflammatory lesions to prevent ulceration.