Orphanet: Diffuse cutaneous mastocytosis

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Diffuse cutaneous mastocytosis

Disease definition

Diffuse cutaneous mastocytosis (DCM) is a rare form of cutaneous mastocytosis (CM; see this term) characterized by generalized erythroderma, various degrees of blistering, skin with a ''peau d'orange'' appearance and the accumulation of mast cells in the skin. At least two DCM variants are recognized, one with extreme blistering (Bullous DCM; see this term) and one with infiltrations (Pseudoxanthomatous DCM; see this term).


Classification level: Disorder
  • Synonym(s):
    • DCM
    • Diffuse cutaneous maculopapulous mastocytosis
  • Prevalence: <1 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Infancy, Neonatal
  • ICD-10: Q82.2
  • OMIM: -
  • UMLS: C0024901
  • MeSH: -
  • GARD: 12686
  • MedDRA: 10012812

Detailed information

General public


ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
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