Mastocytosis (Urticaria Pigmentosa)

Abstract

Mastocytosis first came to the attention of physicians at the end of the last century when the speckled brown pigmentation of the skin and the associated whealing after irritation was described (Nettleship 1869). Several years later, Unna observed a local proliferation of mast cells in the dermis underlying the brown spots (Unna 1887). The disease was called urticaria pigmentosa until the 1930ies when it became apparent that other organs than the skin could be involved, and the more general term mastocytosis was introduced at that time by Sézary (see Chapter 1, p. 3 for details).