Orphanet: Malignant atrophic papulosis

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Malignant atrophic papulosis

Disease definition

Malignant atrophic papulosis (MAP) is a rare, chronic, thrombo-obliterative vasculopathy characterized by papular skin lesions with central porcelain-white atrophy and a surrounding teleangiectatic rim. Systemic lesions may affect the gastrointestinal tract and the central nervous system (CNS) and are potentially lethal.


Classification level: Disorder
  • Synonym(s):
    • Degos disease
    • Köhlmeier-Degos disease
    • Köhlmeier-Degos-Delort-Tricort syndrome
    • Papulosis atrophican maligna
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Adult
  • ICD-10: I77.8
  • ICD-11: EF5Y
  • OMIM: 602248
  • UMLS: C0221011
  • MeSH: D054853
  • GARD: 6249
  • MedDRA: 10064281

Detailed information

Disease review articles

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