Chilblain lupus erythematosus

A form of lupus erythematosus characterized by skin lesions in cold conditions

Chilblain lupus erythematosus is a rare form of lupus erythematosus, a chronic autoimmune disease that primarily affects the skin. This condition is characterized by the development of painful skin lesions, particularly in response to cold temperatures. It is considered a variant of cutaneous lupus erythematosus.

Pathophysiology

Chilblain lupus erythematosus is believed to be triggered by exposure to cold and damp environments, which can lead to the development of chilblains, or pernio. In individuals with a predisposition to autoimmune diseases, these chilblains can evolve into lupus lesions. The exact mechanism involves an abnormal immune response where the body's immune system attacks its own tissues, leading to inflammation and damage.

Clinical Presentation

Patients with chilblain lupus erythematosus typically present with red to purple papules or plaques on the skin. These lesions are most commonly found on the fingers, toes, ears, and nose—areas that are frequently exposed to cold. The lesions can be painful and may ulcerate in severe cases. Unlike other forms of lupus, systemic involvement is less common, although it can occur.

Diagnosis

The diagnosis of chilblain lupus erythematosus is primarily clinical, based on the characteristic appearance of the skin lesions and their association with cold exposure. A skin biopsy may be performed to confirm the diagnosis, revealing features typical of lupus erythematosus, such as interface dermatitis and perivascular lymphocytic infiltrate. Laboratory tests may show the presence of antinuclear antibodies (ANA) and other autoantibodies associated with lupus.

Treatment

Management of chilblain lupus erythematosus involves both lifestyle modifications and medical therapy. Patients are advised to avoid cold exposure and to keep affected areas warm and dry. Topical corticosteroids may be used to reduce inflammation and pain. In more severe cases, systemic treatments such as antimalarials (e.g., hydroxychloroquine) or immunosuppressive agents may be necessary.

Prognosis

The prognosis for individuals with chilblain lupus erythematosus varies. While some patients may experience only mild symptoms, others may have recurrent or persistent lesions. The condition can be chronic, with flares occurring during colder months. With appropriate management, many patients can achieve good control of their symptoms.

Related pages

• Lupus erythematosus
• Cutaneous lupus erythematosus
• Autoimmune disease
• Antinuclear antibody