Urticaria pigmentosa; mastocytosis; a consideration of various manifestations.

Abstract

As a graduate student it was a happy day when one first saw a case of urticaria pigmentosa. Classically it would be a child with scattered macular pigmentation which would urticate when the lesions were irritated (Darier's sign). This was satisfying and for the moment represented all there was to know of the clinical manifestations of this condition. However, with increasing clinical experience, it became increasingly clear that urticaria pigmentosa was not a single simple condition. The concept of a typical case of urticating macular pigmentation (Fig. 1), beginning usually in the first six months of life (congenital) and tending to disappear at puberty, had to be expanded. This broadened concept had to include solitary lesions which had the appearance of some unusual type of nevus (Fig. 2) or xanthoma (Fig. 3); a bullous type (Fig. 4) with solitary or disseminated lesions; a nodular or