Photochemotherapy (PUVA) in the treatment of urticaria pigmentosa

摘要: SUMMARY Eight patients received PUVA for mastocytosis. Five women had typical adult-onset urticaria pigmentosa, without evidence of systemic disease. Another woman suspected hepatic involvement while the remaining female early-onset familial pigmentosa with morphologically atypical mast cells. The only male patient cirrhosis deposits cells in addition to polycythaemia rubra vera. In all patients, except man disease, there was reduced pruritus and wealing partial almost complete fading macules. manifestations recurred after treatment discontinued. both lesional uninvolved skin no significant change either mean cell counts or ultrastructure an average twenty-seven exposures. addition, did not cause a alteration histamine content skin. The beneficial effect therefore does appear be directly related numbers morphology, concentration skin. Urticaria usually presents as generalized maculo-papular rash which urticates on rubbing (Darier's sign). Many are troubled by unsightliness some complain pruritus, flushing. These symptoms attributed release characteristically occur increased dermis. Symptomatic is often unrewarding, but favourable results have been claimed cimetidine Hi blockers (Hirschowitz & Groarke, 1979; O'Laughlin Bredfeldt, 1980) oral disodium cromoglycate (Soter, Austen Wasserman, Czarnetski Behrendt, 1981). 1978, Christophers colleagues reported that photochemotherapy (PUVA) produced symptomatic relief ten adult pigmentosa. Similarly encouraging were subsequently from other centres (Ortonne et al., 1980; Allevato, Donatti Cordero, Granerus, Roupe Swanbeck, 1981; Vaatainen, Hannuksela Karvonen, 1981). In this study we examined effects eight Although moderately good clinical response seven out these (reduced faded macules) quantitative studies failed reveal consistent population density clinically skin. findings arc discussed relation existing information concerning ultraviolet radiation (U VR) constituents