Sarcoidosis is a systemic disorder characterized by noncaseating granulomatous inflammation at sites of disease.1 Although any organ can be involved, the disease most commonly affects the lungs and intrathoracic lymph nodes. A diagnosis of sarcoidosis is most securely established by a compatible clinical presentation and laboratory and radiographic abnormalities, and is confirmed by a biopsy showing noncaseating epithelioid granulomas in more than one organ plus the exclusion of granulomatous disorders of known cause.2 The disease results from the combined effects of genetic susceptibility and environmental exposures.3 Disease-specific immune responses contribute to the nonnecrotizing granulomatous inflammation identified as sarcoidosis. The clinical course is highly variable according to clinical presentation and demographic features.4 Corticosteroids remain the mainstay of treatment for patients with threatened organ failure or progressive disease. Long-term management must include a careful review of systems to identify symptoms that are not due to direct damage to tissues and organs and require alternative treatment strategies.
The first descriptions of sarcoidosis originated in the late 1800s (Table 53-1). A series of letters and reports from Drs. Jonathan Hutchinson and Carl W. Boeck described patients who had characteristic skin lesions on their face and extremities, often referred to as Mortimer’s malady, named after one of Hutchinson’s patients. Several years later, Caesar Boeck (nephew of C.W. Boeck) recognized that these patients often had evidence of disease in multiple organs, such as regional lymph nodes in the neck and axilla, nasal and sinus passages, lung, and bones. The routine practice of biopsies and histopathology in the late 1800s confirmed the heterogeneity of sarcoidosis, causing granulomatous inflammation in different organs in different patients. The advent of radiography established that sarcoidosis most commonly affects the lungs.
TABLE 53-1Historic Sarcoidosis Discoveries and Eponyms ||Download (.pdf) TABLE 53-1 Historic Sarcoidosis Discoveries and Eponyms
|Descriptions of sarcoidosis by Jonathan Hutcherson (England) and Carl W. Beck (Norway) (late 1800s) ||A series of letters described patients with a disease that caused characteristic skin lesions, often referred to as Mortimer’s malady |
|Cutaneous sarcoidosis frequently presents with characteristic skin lesions on the face and scalp (1888) ||Ernest Besnier (France) popularized “lupus pernio” as violaceous skin lesions of the nose, ear lobes, face, and scalp (initially hypothesized to be a variant of lupus erythematosus) |
|Sarcoidosis is a systemic disease—Caesar Boeck (Norway) and Jorgen Schaumann (Sweden) (early 1900s) ||Advances in medical science usher the routine practice of obtaining biopsies and histopathology, characterize sarcoidosis as a systemic disease: granulomatous inflammation in the skin, lungs, eyes, bone, lymph nodes and spleen (hypothesized to be a variant of tuberculosis) |
|A causative agent for sarcoidosis is identified in diseased tissues ||An extract prepared from sarcoidosis tissues results in granulomatous inflammation when injected into the skin of patients with sarcoidosis—Drs. Williams and Nickerson (United States, 1935) and Ansgar Kveim (Norway, 1941). |
|The Kveim reaction (positive ...|