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Interstitial granulomatous dermatitis with arthritis

Disease definition

Interstitial granulomatous dermatitis with arthritis is a rare rheumatologic disease characterized by the occurrence of inflammatory arthritis in association with large, erythematous, symmetrical cutaneous lesions (ranging from typical, but infrequent, cord-like lesions on the flanks to more common violaceous plaques on the trunk and limbs) featuring a typical histologic infiltrate mainly constituted of histiocytes.

ORPHA:79099

Classification level: Disorder
  • Synonym(s):
    • Ackerman dermatitis syndrome
    • Ackerman syndrome
    • IGDA
  • Prevalence: <1 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: L30.8
  • ICD-11: LD27.0Y
  • OMIM: -
  • UMLS: C4751206
  • MeSH: -
  • GARD: -
  • MedDRA: -

  A summary on this disease is available in Español (2018) Français (2018) Italiano (2018) Nederlands (2018) Polski ()

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.