Sarcoidosis is a multisystem disease characterized by granulomatous inflammation of unknown etiology commonly occurring in the lung and the skin, but any organ system can be affected.
The type of cutaneous lesion may suggest prognosis. Lupus pernio lesions are associated with chronic disease course and scarring. Erythema nodosum lesions portend an acute self-resolving course.
Specific lesions of sarcoidosis have a varied clinical morphology, including macules, papules, plaques, nodules, and ulcerations.
Topical, systemic, and intralesional corticosteroids are the mainstay of therapy.
Patients initially diagnosed with cutaneous sarcoidosis should get baseline testing to look for deleterious systemic disease, including a physical examination; neurologic review of systems; a chest radiograph; an electrocardiogram; pulmonary function tests; urine analysis; complete blood count; comprehensive metabolic panel; serum calcium level; tuberculosis screening; and an ophthalmologic examination.
Sarcoidosis is a multisystem disease characterized by granulomatous inflammation. It is theorized to be a disease of hyperactivation of the immune system, but its etiology is unknown.
Although the lungs are the organs most commonly afflicted, sarcoidosis was actually first described in the late 1800s because of its skin manifestations. Boeck reported a case of “multiple benign sarkoid of the skin” as he believed the skin lesions resembled sarcomas but were benign.1
Sarcoidosis occurs worldwide and may affect all ages and races. Disease onset is most common in the third decade of life and is slightly higher in women.2 Sarcoidosis has a higher incidence with greater distance from the equator. The highest prevalence of sarcoidosis is found in whites in Denmark and Sweden, and in persons of African descent in the United States.3 In the United States, the lifetime risk of sarcoidosis is 2.4% in African Americans and 0.85% in whites.3 The incidence rate of sarcoidosis in the United States is 17.8 per 100,000 for African Americans and 8.1 per 100,000 for whites; the prevalence rate is 141.8 per 100,000 for African Americans and 49.8 per 100,000 for white individuals.4 The highest prevalence is in African American women (178.5 per 100,000).4 African Americans tend to have more severe symptomatic disease. African Americans are more likely to have extrapulmonary disease,5 require treatment,6 and have a lower rate of clinical recovery7,8 than whites.
Table 35-1 summarizes the variable presentations of sarcoidosis.
Table 35-1Variable Presentations of Sarcoidosis ||Download (.pdf) Table 35-1 Variable Presentations of Sarcoidosis
| ||CLINICAL MANIFESTATIONS |
|Lupus pernio ||Symmetric, violaceous, indurated plaques and nodules on nose, earlobes, cheeks, and digits |
|Mikulicz syndrome ||Sarcoidal granulomas on mucosal surface and tongue, with bilateral enlargement of the lacrimal, parotid, sublingual, and submandibular glands |
|Darier-Roussy sarcoid ||Presents as persistent subcutaneous nodules that may be tender or painless; occurs on extremities |