Diagnosis: Sarcoidosis

Sarcoidosis is a multisystem disease of unknown etiology with no race predilection. There is a bimodal age distribution, with peaks at 25-35 years and 45-65 years. Cutaneous lesions occur in 20%-35% of patients, and are often an early manifestation of disease. Lesions are often red-brown and include papules, nodules, and plaques.

Sarcoidosis is an immune-mediated disease. CD4+ Th1 helper T cells are upregulated, producing cytokines such as interleuken-2 and interferon-gamma, which lead to B-cell stimulation. Epithelioid granulomas are formed in multiple organ systems. The initiating antigen is unknown.

Pulmonary fibrosis occurs in 20%-25% of patients with sarcoidosis. The most common ophthalmologic manifestation, chronic anterior uveitis, may result in glaucoma and vision loss. The patient had a skin biopsy performed prior to initial visit that revealed the common histological finding: noncaseating granulomas. Sarcoid granulomas produce angiotensin-converting enzyme inhibitors in 60% of patients.

Lupus pernio is the most characteristic skin manifestation of sarcoidosis. Increased vascularity results in violaceous, indurated small papules to large plaques on the nose, lips, cheeks, and earlobes. Concurrent upper respiratory disease is typical, with approximately 75% of patients having chronic sarcoidosis of the lungs. Cystic bone lesions on radiographic imaging may also be seen.

Medical therapy is often difficult. Treatment options include oral prednisone, hydroxychloroquine (Plaquenil), and infliximab. The patient was treated with hydroxychloroquine, which resulted in improvement.

This case was first presented at Maryland Derm, at the University of Maryland School of Medicine in Baltimore, by Dr. Donna Bilu Martin, Dr. Vera David, and Dr. Anthony Gaspari.