CHILBLAIN LUPUS ERYTHEMATOSUS
By Gary M. White, MD
Chilblain lupus erythematosus is an autosomal dominant form of lupus that tends to present in early childhood.
- Pernio-like lesions precipitated by the cold are characteristic.
- Many patients have facial DLE and a smaller percentage have SLE.
- A heterozygous mutation the TREX1 gene and a mutation in the SAMHD1 gene have been found.
- Some patients have been found to be Ro positive.
- A damp, cold environment is the main precipitating factor.
Lesions on the nose and dorsa of the fingers and toes are most common. The lips and ears may be involved as well. A very typical finding is tumid, violaceous plaques on the dorsa of the fingers, with discoloration most prominent over the DIP joints. The lesions may be keratotic, atrophic and sometimes ulcerative.
Criteria Used to Diagnose
- Skin lesions in acral locations induced by exposure to cold or a drop in temperature.
- Coexistence of SLE or other skin lesions of DLE.
- Evidence of lupus on HE or immunofluorescence.
- Response to anti-lupus therapy
- Negative results of cryoglobulin and cold agglutinin studies.
- Warm weather is the treatment of choice. Cold weather with low humidity does not seem to precipitate this condition and may explain why there are few cases in the US where central heating is common.
- Topical steroids may be tried.
- Antimalarials are usually of little value, but may be tried.
- Vasodilators such as nifedipine may be helpful.
- Other options that have been recommended include pentoxifylline, dapsone, and low-dose systemic steroids.
- Patients who smoke should quit.
Chilblain lupus erythematosus in an adolescent girl. Indian Dermatol Online J 2014;5, Suppl S1:30-2
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