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Lupus erythematosus tumidus

Disease definition

A rare form of chronic cutaneous lupus erythematosus characterized by extreme photosensitivity with intermittent formation of erythematous, edematous, urticarial-like, smooth plaques on sun-exposed skin areas. The lesions heal without scarring. The course of the disease is benign, and development of systemic lupus erythematosus is infrequent. Most patients do not have lupus-related autoantibodies. Skin biopsy shows a perivascular and periadnexal lymphocytic infiltrate and increased dermal mucin deposition without involvement of the dermoepidermal junction.

ORPHA:90283

Classification level: Disorder
  • Synonym(s):
    • Intermittent cutaneous lupus
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: -
  • ICD-10: L93.2
  • OMIM: -
  • UMLS: C0406636
  • MeSH: -
  • GARD: 13003
  • MedDRA: -
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