ABSTRACT
A female newborn presented with emerging skin lesions, systemic eosinophilia, and eosinophilic reaction in the skin, liver, lungs, spleen, lymphatic nodes, porencephalia, convulsions, and disorders of thermoregulation. In addition to that, respiratory and heart failure, as well as brain edema were progressing. The suspected diagnosis of incontinentia pigmenti (Bloch-Sulzberger syndrome) was confirmed postmortem by skin biopsy.
Subject(s)
Incontinentia Pigmenti , Autopsy , Eosinophilia/complications , Eosinophilia/diagnosis , Female , Humans , Incontinentia Pigmenti/complications , Incontinentia Pigmenti/diagnosis , Incontinentia Pigmenti/mortality , Incontinentia Pigmenti/pathology , Infant, Newborn , Skin/pathologyABSTRACT
Incontinentia Pigmenti is a rare X-linked multisystem neuroectodermic disorder with signs and symptonms related mainly to the dermatologic, dental, ocular and central nervous systems, and characterized by death in the majority of male embryos. Affected children do not appear sick, in spite of the skin eruption, the peripheral leucocytosis and marked eosinophilia. Most of the cases are reported in caucasians, although there are description in black children, orientals, north, central and south american indians, and in our mestizos. The name of Incontinentia Pigmeti describes the incontinence of the melain pigment from the basal layer of the epidemermis into the superficial dermis
