Chilblain lupus is a rare cutaneous form of systemic lupus erythematosus (SLE; 152700) characterized by tender, bluish-red swellings and nodules on the hands, feet, ears, and nose, with histologic changes of lupus. The phenotype is induced by cold, ... Chilblain lupus is a rare cutaneous form of systemic lupus erythematosus (SLE; 152700) characterized by tender, bluish-red swellings and nodules on the hands, feet, ears, and nose, with histologic changes of lupus. The phenotype is induced by cold, such that patients frequently report a worsening of lesions in the winter months (summary by Ravenscroft et al., 2011). For a general description and a discussion of genetic heterogeneity of chilblain lupus, see CHBL1 (610448).
Ravenscroft et al. (2011) reported a mother and son with chilblain lupus. From the age of 4 years, the 46-year-old white mother experienced recurrent lesions, particularly prominent over the winter months, affecting her hands, feet, buttocks, and thighs. ... Ravenscroft et al. (2011) reported a mother and son with chilblain lupus. From the age of 4 years, the 46-year-old white mother experienced recurrent lesions, particularly prominent over the winter months, affecting her hands, feet, buttocks, and thighs. She also had sun sensitivity with a tendency to develop a sunburn-like reaction with minimal sun exposure. She developed angiomatous lesions on the fingers, which became persistent. Biopsy of chilblain skin demonstrated a florid lymphocytic vasculitis, with papillary dermal edema, interface dermatitis, and keratinocyte necrosis, consistent with lupus. She was managed with nifedipine over the winter months and hydroxychloroquine plus sun block in the summer. At age 3 months, her 15-year-old son developed chilblains on the feet, fingers, and ears. Later, he also experienced photosensitivity with a sunburn-like reaction to sunlight, and developed fixed angiomatous lesions on the fingers. Both patients were in otherwise good health.