Pathology showed noncaseating granulomas consistent with cutaneous sarcoidosis. Based on these biopsy findings, a chest x-ray was ordered, and it confirmed a pulmonary sarcoid. A multidisciplinary work-up (including cardiac evaluation, continued rheumatologic care, and evaluation by Hematology) addressed this new finding.
Sarcoidosis is a multisystem inflammatory disorder characterized by the development of granulomas that can arise in any organ, but frequently involve the skin and lungs. Patients with cutaneous disease develop smooth skin lesions, including flesh-colored to pink or brown papules on the face. Genetic and environmental factors are both thought to contribute to the disease.
Race is a significant factor in the development of disease. Hispanic and Asian patients are significantly less likely to develop the disease compared to White or Black patients. In the Black Women’s Health Study, incidence in Black women was 71 per 100,000.1 Women are more likely to be affected than men.1
Many patients with sarcoidosis have a mild course, but for others the disease may progress on the skin or include pulmonary, renal, neurologic, or cardiac disease. Sometimes sarcoidosis is fatal. Recurrence can occur at any point later in life. Race influences disease severity as well as incidence, with hospitalization being 9 times as likely in Black patients compared with White patients.2 One recent study puts sarcoidosis mortality rates for Black women at 10 per million compared with 3 per million in Black men, and 1 per million in White women or men.3
Patients with disease limited to the skin may be treated with topical steroids such as clobetasol 0.05% cream or ointment or intralesional triamcinolone 10 mg/mL injected into affected lesions every 2 to 4 weeks. With pulmonary or other systemic disease, treatment may include various disease-modifying agents including prednisone, methotrexate, hydroxychloroquine, and TNF-alpha inhibitors. Because of the long-term adverse effects of systemic steroids, these agents are reserved for instances when pulmonary function is significantly impacted.
This patient had a reassuring cardiac and hematology work-up. Her pulmonary function was impacted sufficiently enough that her pulmonologist added a course of prednisone 10 mg daily tapered over 6 weeks. She had been on hydroxychloroquine 200 mg twice daily prior to the diagnosis of sarcoidosis for presumed mixed connective tissue disease and was continued on it for sarcoidosis after completing the prednisone taper. With these treatments, her facial lesions cleared and her breathing symptoms and fatigue improved. She remains under surveillance with a multidisciplinary team.
Photos and text for Photo Rounds Friday courtesy of Jonathan Karnes, MD (copyright retained). Dr. Karnes is the medical director of MDFMR Dermatology Services, Augusta, ME.