Sarcoidosis

Sarcoidosis is a disease characterized by noncaseating granulomatous infiltration of 1 or more organs. In North America, after the lungs and thoracic lymph nodes, the skin is the next most commonly involved organ. Data from multiple studies indicate a coaction between genetic and environmental factors in immunologically susceptible hosts. The disease’s many clinical manifestations and course vary greatly and are influenced by race, ethnicity, and gender. In the skin, the lesions of sarcoidosis are classified as specific when noncaseating granulomas are present, and nonspecific when there is an inflammatory reaction pattern devoid of granulomas.

Key points

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Sarcoidosis is a multisystemic granulomatous disease of unknown cause that affects the skin in 20% to 30% of cases.
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Sarcoidosis skin lesions may be specific, which tend to be chronic, or nonspecific, which tend to be acute.
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Lupus pernio is associated with chronic disease, whereas erythema nodosum is associated with acute disease and spontaneous resolution.
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Systemic treatment of the skin is reserved for symptomatic, widespread, disfiguring, and/or quality of life–altering disease and includes oral corticosteroids, methotrexate, antimalarials, minocycline, and some tumor necrosis factor inhibitors.
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The diagnosis of sarcoidosis requires a multimodal approach that comprises clinical findings, histologic presence of noncaseating granulomas, demonstration of organ involvement radiologically or through other tests, and exclusion of other diseases.

Introduction

Sarcoidosis is a chronic but frequently self-resolving disease of unknown cause characterized histologically by the formation of noncaseating epithelioid cell granulomas in one or more organs. The protean skin manifestations can confound even experienced dermatologists, and, in some cases, effective treatment is challenging. A reasonable diagnosis can be made in most cases from the appearance of skin lesions, confirmatory histology, involvement of other organ systems, and exclusion of other noncaseating granulomatous diseases. It must always be remembered that sarcoidosis is a potentially lethal and disabling disease with a broad spectrum of heterogeneous anatomic involvement leading to a remarkable range of possible symptoms that can mimic those of many other diseases. Therefore, sarcoidosis should be included in many clinical differential diagnoses (see Table 2 ). Evaluation of extracutaneous involvement should be performed at the initial presentation of sarcoidosis on any organ ( Box 1 ), and a review of systems should be part of follow-up visits. Collaborative communication between specialists and primary care physicians engenders better patient care.

Box 1

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