Segmental pigmented purpura.

Abstract

A segmental distribution of pigmented purpura (PP) is rare. Our patient presented at 5 years of age with red-brown petechial macules and telangiectases on her chest, the inner and dorsal aspects of the right upper extremity, and the dorsal aspect of the hand, which had developed slowly over the previous 7 months. The lesions were intermittently pruritic. A biopsy from the right arm revealed discrete hydropic degeneration of the basal layer of the epidermis, dilation of the blood vessels, numerous extravasated erythrocytes and lymphocytes, and lymphocytes in the lower part of the epidermis. The skin disease resolved almost completely within 18 months. The general physical examination revealed an open foramen ovale, slight hypogammaglobulinemia (G and A), and enhanced fragility of the blood vessels.