Denouement and Discussion
Incontinentia Pigmenti (Bloch-Sulzberger Syndrome)
Major manifestations involve the skin, eyes, teeth, and central nervous system. There may be four different types of skin lesions. The first is vesicular or bullous often in clusters with an erythematous base and most frequently located on the extremities. They are present during the first weeks of life and are usually gone by the fourth month. On microscopic examination, the bullous lesions contain a large number of eosinophils. The second stage, which does not always occur, consists of warty hyperkeratotic lesions, usually arranged in longitudinal verrucous ridges and are also present on the limbs, mainly the fingers, knuckles, and joints. These lesions may persist for months. The classical brownish-gray areas of pigmentation are present at birth or shortly thereafter. They appear in a reticular pattern or as whorls, usually on the trunks and limbs, although they may be present on other parts of the body. By about 2
Gellis SS, Feingold M. Picture of the Month. Am J Dis Child. 1972;123(2):137–138. doi:10.1001/archpedi.1972.02110080115012
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