[Cutaneous mastocytosis].
Cutaneous mastocytosis represent the most frequent form of mastocytosis, rare diseases, defined by an abnormal accumulation and proliferation of mastocytes in one or more organs. Cutaneous mastocytosis more often appear early in childhood and usually resolve spontaneously by the time of puberty. In adult, cutaneous mastocytosis rarely involute and are frequently associated to extracutaneous involvement and so, are in fact systemic mastocytosis. Clinical presentation of cutaneous mastocytosis includes polymorphous cutaneous lesions linked to mastocytes skin infiltration often associated to acute episodes (lesional or systemic flush) due to mast cells degranulation. The cause of mastocytosis is unknown. Several mutations of the c-kit proto-oncogen coding for the transmembrane receptor kit of the stem groth factor, factor of maturation, proliferation and activation of mastocytes, are often observed. Currently, the treatment of cutaneous mastocytosis is mainly symptomatic.
Flageul
Béatrice
B
Service de dermatologie, hôpital Saint-Louis, 75475, Paris Cedex 10. beatrice.flageul@sls.aphp.fr
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008407:Mast Cells / Q000473:pathology; D034701:Mastocytosis, Cutaneous / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D009365:Neoplasm Regression, Spontaneous; D015995:Prevalence; D011379:Prognosis; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics; D012737:Sex Factors; D012867:Skin / Q000473:pathology; D014582:Urticaria Pigmentosa / Q000453:epidemiology
D019009:Proto-Oncogene Proteins c-kit