[Urticaria pigmentosa: a current approach].
The term urticaria pigmentosa (UP) denotes a heterogeneous group of disorders characterized by abnormal growth and accumulation of mast cells (MC) in the skin. Symptoms result from MC chemical mediator's release, pathologic infiltration of neoplastic MC in tissues or both. Multiple molecular, genetic and chromosomal defects seem contribute to an autonomous growth, but somatic c-kit D816V mutation is more frequently found, especially in systemic disease. The aim of this paper is to provide a current overview for a better understanding of the symptoms associated with this disease, to describe its classification, recent advances in its pathophysiology and its treatment.
Pérez-Elizondo
Antonio David
AD
Servicio de Dermatología, Hospital para el Niño IMIEM, Toluca, Estado de México. apederma@yahoo.com.mx
Zepeda-Ortega
Benjamín
B
del Pino-Rojas
Gladys Teresa
GT
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D003937:Diagnosis, Differential; D006633:Histamine Antagonists / Q000627:therapeutic use; D006801:Humans; D006967:Hypersensitivity / Q000453:epidemiology; D007223:Infant; D007231:Infant, Newborn; D008407:Mast Cells / Q000378:metabolism / Q000473:pathology; D008415:Mastocytosis / Q000145:classification; D020125:Mutation, Missense; D017354:Point Mutation; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics / Q000502:physiology; D019089:Stem Cell Factor / Q000502:physiology; D014582:Urticaria Pigmentosa / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics / Q000473:pathology / Q000503:physiopathology
D000305:Adrenal Cortex Hormones; D006633:Histamine Antagonists; D019089:Stem Cell Factor; D019009:Proto-Oncogene Proteins c-kit