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Cutaneous mastocytosis

Orpha number ORPHA66646
Synonym(s) -
Prevalence Unknown
Inheritance
  • Sporadic
Age of onset Childhood
ICD-10
  • Q82.2
OMIM -
UMLS
  • C1136033
MeSH
  • D034701
MedDRA -
SNOMED CT
  • 397012002

Summary

Cutaneous mastocytosis is a term referring to a group of diseases characterized by abnormal accumulation and proliferation of skin mastocytes. In some cases (most commonly in adults), cutaneous mastocytosis may occur in association with mast cell infiltration of various extracutaneous organs, in which case the disorder is referred to as systemic mastocytosis (see this term).

The prevalence of cutaneous mastocytosis in the general population is unknown but the disorder is believed to represent between 0.1 and 0.8% of all dermatological consultations and is more common in the Caucasian population.

Three main forms of cutaneous mastocytosis have been described: diffuse cutaneous mastocytosis (with two subforms: bullous diffuse cutaneous mastocytosis and pseudoxanthomatous diffuse cutaneous mastocytosis), cutaneous mastocytoma and, the most common form, maculopapular cutaneous mastocytosis (see these terms).


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Detailed information

Summary information
Emergency guidelines
  • FR (2013,pdf)
Review article
  • FR (2006,pdf)
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