Lupus pernio

A detailed overview of Lupus Pernio

Classification
External resources

Lupus Pernio

Lupus pernio affecting the nose and cheeks

Lupus pernio is a chronic, disfiguring skin condition associated with sarcoidosis. It is characterized by violaceous, raised lesions that typically appear on the face, particularly the nose, cheeks, lips, and ears. The condition is named for its resemblance to the lesions seen in lupus vulgaris, although it is not related to lupus erythematosus.

Pathophysiology

Lupus pernio is a manifestation of sarcoidosis, a systemic inflammatory disease characterized by the formation of non-caseating granulomas in various organs. The exact cause of sarcoidosis and lupus pernio is unknown, but it is believed to involve an abnormal immune response to an unknown antigen. The skin lesions in lupus pernio are thought to result from granulomatous inflammation in the dermis.

Clinical Presentation

Patients with lupus pernio typically present with chronic, indurated, violaceous plaques on the face. These lesions are often asymptomatic but can be disfiguring and may lead to cosmetic concerns. The lesions are usually persistent and may slowly enlarge over time. In some cases, lupus pernio can be associated with other systemic manifestations of sarcoidosis, such as pulmonary sarcoidosis, uveitis, or cardiac sarcoidosis.

Diagnosis

The diagnosis of lupus pernio is primarily clinical, based on the characteristic appearance of the skin lesions. A skin biopsy can be performed to confirm the presence of non-caseating granulomas, which are indicative of sarcoidosis. Additional tests, such as chest X-ray or CT scan, may be conducted to assess for systemic involvement of sarcoidosis.

Treatment

Treatment of lupus pernio can be challenging, as the lesions are often resistant to therapy. The mainstay of treatment is systemic corticosteroids, which can help reduce inflammation and improve the appearance of the lesions. Other treatments that may be used include methotrexate, hydroxychloroquine, and tumor necrosis factor (TNF) inhibitors. In some cases, laser therapy or surgical excision may be considered for cosmetic improvement.

Prognosis

The prognosis of lupus pernio varies depending on the extent of the disease and the response to treatment. While the condition is chronic and can be disfiguring, it is not life-threatening. With appropriate management, many patients can achieve significant improvement in their symptoms and quality of life.

Related pages

• Sarcoidosis
• Granuloma
• Lupus vulgaris
• Lupus erythematosus