Disseminated lupus erythematosus

Disseminated Lupus Erythematosus

Disseminated lupus erythematosus (DLE) is a chronic autoimmune disease that primarily affects the skin, but can also have systemic manifestations. It is a form of lupus erythematosus, which is a broader term encompassing several related conditions, including systemic lupus erythematosus (SLE) and discoid lupus erythematosus.

Pathophysiology

DLE is characterized by the immune system attacking the body's own tissues, leading to inflammation and damage. The exact cause of DLE is not fully understood, but it is believed to involve a combination of genetic, environmental, and hormonal factors. The immune system's production of autoantibodies plays a crucial role in the pathogenesis of the disease.

Clinical Features

Patients with disseminated lupus erythematosus often present with skin lesions that are red, scaly, and may lead to scarring. These lesions are typically found on sun-exposed areas such as the face, neck, and scalp. Unlike discoid lupus erythematosus, which is limited to the skin, DLE can have systemic involvement, although it is less common than in SLE.

Skin Manifestations

- Erythematous plaques - Scaling - Scarring - Photosensitivity

Systemic Manifestations

While primarily a cutaneous condition, DLE can occasionally affect other organs, leading to symptoms such as: - Fatigue - Joint pain - Fever

Diagnosis

The diagnosis of disseminated lupus erythematosus is based on clinical examination, laboratory tests, and sometimes a skin biopsy. Laboratory tests may reveal the presence of antinuclear antibodies (ANA) and other specific autoantibodies. A skin biopsy can show characteristic histological features such as interface dermatitis and follicular plugging.

Treatment

Treatment of DLE focuses on managing symptoms and preventing flares. Common treatments include: - Topical corticosteroids - Antimalarial drugs such as hydroxychloroquine - Sun protection measures - Systemic immunosuppressants in severe cases

Prognosis

The prognosis for patients with disseminated lupus erythematosus varies. While the condition can be chronic and relapsing, many patients achieve good control of symptoms with appropriate treatment. However, scarring from skin lesions can be permanent.

Also see

- Systemic lupus erythematosus - Discoid lupus erythematosus - Autoimmune diseases - Antinuclear antibody