Chilblain lupus erythematosus (lupus pernio): clinical review of the Mayo Clinic experience and proposal of diagnostic criteria

Five cases of chilblain lupus erythematosus were retrospectively reviewed regarding their clinical, histopathologic, serologic, and immunofluorescence findings. Ages at onset of chilblain lupus erythematosus varied from 26 to 73 years, with a female-to-male ratio of 3:2. Since other entities can be confused with this disorder, we propose the following diagnostic criteria. The two major criteria are skin lesions in acral locations induced by exposure to cold or a drop in temperature, and evidence of lupus erythematosus in the skin lesions by results of histopathologic examination or direct immunofluorescence study. The three minor criteria are coexistence of systemic lupus erythematosus or other skin lesions of discoid lupus erythematosus, response to anti-lupus erythematosus therapy, and negative results of cryoglobulin and cold agglutinin studies. We conclude that chilblain lupus erythematosus can be diagnosed and treated. Discoid lupus erythematosus lesions respond more quickly to treatment than chilblain lupus erythematosus lesions. Treatment with antimalarial agents, prednisone, pentoxifylline, or dapsone was of benefit to our patients.