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.
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D001847:Bone Diseases / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005767:Gastrointestinal Diseases / Q000209:etiology; D006402:Hematologic Diseases / Q000209:etiology; D006636:Histamine Release; D006801:Humans; D007223:Infant; D008407:Mast Cells / Q000032:analysis / Q000166:cytology; D012515:Mast-Cell Sarcoma / Q000473:pathology; D008875:Middle Aged; D011379:Prognosis; D011537:Pruritus / Q000209:etiology; D012867:Skin / Q000473:pathology; D014582:Urticaria Pigmentosa / Q000145:classification / Q000150:complications / Q000473:pathology* / Q000628:therapy