Chilblain Lupus Erythematous: A Rare Encounter

Chilblain lupus erythematosus is a rare, chronic variant of cutaneous lupus erythematosus that occurs during cold or damp periods on the hands, fingers, or feet. It is often associated with other forms of cutaneous lupus. Like idiopathic chilblains such as perniosis, CHLE presents with tender, reddish-blue papules, nodules, or plaques on the toes, fingers, nose, or ears precipitated by cold exposure. The prevalence of the disease is about 3-20%; moreover, it occurs more frequently in women. Purpuric erythematous-violet plaques characterize it in the distal regions, such as the ears, nose, fingers and toes. It has a symmetrical distribution, usually edematous, itchy and sometimes painful lesions. They generally occur as a result of exposure to cold. Chilblain lupus is defined by the Mayo Clinic criteria, including two major and four minor. Histologic features are identical to those of discoid lupus erythematosus. The damaged skin gives a positive fluorescent band test. Various medications have been tried, including steroids, mycophenolate mofetil, calcium channel blockers, and hydroxychloroquine, but the symptoms do not remit completely. Managing chilblain lupus erythematosus is still unclear, and more research needs to be conducted. Treatment aims are to reduce disease activity and damage control. The first-line treatment for mild and localised cutaneous lupus erythematosus is topical corticosteroids, while the second-line systemic treatments consist mainly of immunomodulators and immunosuppressants. Clinicians should recognise the importance of early diagnosis and prompt treatment initiation to reduce associated morbidity and possible disfigurement of the patients.