Mastocytosis

Mastocytosis implies involvement by excessive numbers of mast cells, of skin and/or other organs. Symptoms may be absent to severe, even occasionally life-threatening. H1- and H2-blocking agents potentially may relieve symptoms, but do nothing to prevent systemization. At present no specific combination chemotherapy regimen has been shown to be markedly effective in a large controlled study. Mithramycin may be of benefit in relieving localized bone pain, although confirmation of this observation in large numbers of patients is needed. PUVA may be a viable approach to symptomatic treatment of otherwise poorly controlled urticaria pigmentosa. In children, urticaria pigmentosa is a relatively benign disease without an increased risk of later systemic involvement. In adults, however, other organs by be involved, with resultant increased morbidity and mortality. Without specific symptoms to a particular organ system, elaborate and expensive staging work-up is not indicated, at least until there is evidence that a reproducibly effective therapy for systemic disease exists.