Pediatric mastocytosis.
Pediatric mastocytosis presents with heterogeneous cutaneous lesions and symptoms that are caused by increased numbers of tissue mast cells. In contrast with adult patients with mastocytosis, the course of pediatric patients is usually transient. Therefore, it has long been speculated that pediatric and adult mastocytosis may be based on different pathogenetic mechanisms. Indeed, new genetic findings now indicate differences in the pathogenesis. Adult patients usually express activating mutations of the growth factor receptor c-kit. Most children lack these mutations but sometimes carry other inactivating mutations of c-kit. Only children with progressive mastocytosis seem to express the activating mutations seen in adults. Causal treatment is not yet available, but H1 and H2 antihistamines may provide relief of symptoms. It is important to counsel patients and their parents carefully to avoid triggers that induce systemic mast cell degranulation.
Hartmann
K
K
Department of Dermatology, University of Cologne, Germany.
Metcalfe
D D
DD
D000293:Adolescent; D000328:Adult; D017209:Apoptosis; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007378:Interleukins / Q000235:genetics; D008407:Mast Cells / Q000378:metabolism / Q000473:pathology; D012515:Mast-Cell Sarcoma / Q000453:epidemiology / Q000473:pathology; D008415:Mastocytosis / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000473:pathology / Q000628:therapy; D011379:Prognosis; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics; D012983:Soft Tissue Neoplasms / Q000453:epidemiology / Q000473:pathology; D019089:Stem Cell Factor / Q000235:genetics; D014582:Urticaria Pigmentosa / Q000453:epidemiology / Q000473:pathology
D007378:Interleukins; D019089:Stem Cell Factor; D019009:Proto-Oncogene Proteins c-kit