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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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