In recent years increased recognition of internal medical counterparts of urticaria pigmentosa (mastocytosis) has been recorded. Nickel's 1 comprehensive review summarized the frequency with which visceral and osseous lesions of mastocytosis are encountered, if searched for. To be sure, a purely cutaneous form of urticaria pigmentosa is still identified, but the possibility is not excluded that even these cases may have subclinical, inconspicuous, and hidden foci of the disease in locations elsewhere than the skin. Proof of the latter conjecture is not readily available, as opportunities for autopsy examination of this benign disease are extremely rare. Simply to authenticate the fact that the skin may indeed be the organ exclusively involved in urticaria pigmentosa, even when of severe degree, is the purpose of this paper. We wish to report a case of congenital urticaria pigmentosa in an infant, studied clinically and at autopsy. Report

Urticaria Pigmentosa

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