Bone lesions of urticaria pigmentosa in childhood.

Urticaria pigmentosa is a dermatosis characterized clinically by areas of pigmented skin which show marked urtication when a stimulus such as a scratch or a pinprick is applied. Initially they occur as pink urticarial lesions, but instead of fading they persist as oval or round, light or dark brown macules or, less commonly, papules or nodules. Histologically, the skin lesions show an increased number of mast cells in the dermis, and probably it is their high histamine content which accounts for the facility with which urtication occurs. The mast cells contain not only an abnormally high content of histamine (Riley, 1953; Riley and West, 1953), but also a great excess of heparin (Oliver, Bloom and Mangieri, 1947). In recent years the concept of a generalized disease due to mast cell proliferation has been gaining ground. Mast cell infiltration of parenchymatous organs has been reported (Ellis, 1949; Reilly, Shintani and Goodman, 1955; Berlin, 1955; Brodeur and Gardner, 1956; Loewenthal, Schen, Berlin and Wechsler, 1957; Rider, Stein and Abbuhl, 1957), and the appearance of mast cells in the peripheral blood has been called a mast cell leukaemia (Hissard, Moncourier and Jacquet, 1950; Waters and Lacson, 1957). The discovery by Sagher, Cohen and Schorr (1952) that bone lesions could be associated with urticaria pigmentosa has led others to investigate the condition from this aspect (Clyman and Rein, 1952; Calnan, 1953; Asboe-Hansen, 1953; Grupper, 1954; Parmentier, 1954; Bluefarb and Salk, 1954; Cordero, 1954; Degos, 1955; Reilly et al., 1955; Stark, Van Buskirk and Daly, 1956; Deutsch, Ellegast and Nosko, 1956; Schorr, Sagher and Liban, 1956; Sagher, Liban, Ungar and Schorr, 1956; Zak, Covey and Snodgrass, 1957). These accounts show a striking variability in the incidence of osseous abnormalities and also in the form and characteristics of the lesions. The admission of an 8-year-old girl with urticaria pigmentosa, paraplegia and bone changes in the spine (Case 1) led us to re-examine 31 other children, who had been patients at this hospital, with a diagnosis of urticaria pigmentosa.