Red-brown papules and plaques on the head and body

Sarcoidosis is a disease that involves an abnormal infiltration of granulomas that can form in any organ system.1 
Histologically, sarcoidosis presents itself as various nodules of specifically noncaseating "non-necrotizing" granulomas, which are covered by fibrotic scar tissue, instead of containing...

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Sarcoidosis is a disease that involves an abnormal infiltration of granulomas that can form in any organ system.¹ 


Histologically, sarcoidosis presents itself as various nodules of specifically noncaseating “non-necrotizing” granulomas, which are covered by fibrotic scar tissue, instead of containing necrosis.² Typically, the inflammatory cells of granulomas are an aggregation of macrophages, monocytes, and various T-lymphocytes.² The most common site of accumulation is within the lungs or lymph nodes of the chest cavity, but the spleen, liver, eyes, and skin are all common sites of infection. 


There is evidence to suggest increased rates of sarcoidosis within Northern European and African American populations, specifically among individuals aged 20 years to 29 years, but the disease can be found in all individuals, regardless of race, age, or gender.³ Although the cause for sarcoidosis is still unknown, possible explanations include both genetic predisposition and exposure to environmental toxins that trigger an autoimmune response. 


Cutaneous sarcoidosis is seen in approximately 25% to 30% of patients who have systemic sarcoidosis.⁴ When cutaneous lesions are present, they serve as significant clinical markers for the possible presence of systemic sarcoidosis. 


However, cutaneous sarcoidosis has a variety of physical representations, making it difficult to associate one manifestation as a clear-cut sign of the disease. The majority of authors separate the lesions associated with cutaneous sarcoidosis into nonspecific and specific subsets. Nonspecific lesions, while still linked to sarcoidosis, do not present granulomas under the microscope; the most common of the nonspecific lesions is erythema nodosum.⁴ Specific sarcoid lesions, which do show histologic granulomas, are seen on a gross scale as papules, plaques, lupus pernio, and in rare instances, alopecia, ulcers, and icthyosis.⁴ 


There is no single test that can be used for the diagnosis of sarcoidosis. The Kveim-Siltzbach test is the most specific test available, but the test is not typically used, because it is not readily available and there is a chance for transmission of infection.⁵ Thus, when cutaneous lesions are present, they serve as an important landmark for the diagnosis of sarcoidosis and as a source for biopsy for histologic examination. 


Otherwise, a diagnosis of sarcoidosis is typically made through the exclusion of other ailments. Chest x-rays and biopsies of lymph nodes are taken to find evidence that is consistent with sarcoidosis; these are especially helpful if there are related respiratory complications.² Blood tests examine for levels of several serum markers including for interleukin-2, glycoprotein KL-6, and serum amyloid A.⁶ Cultures are also sometimes drawn from lesions to exclude possible infections.⁴ 


Treatment for the majority of cases of sarcoidosis focuses on the symptoms of the disease and uses nonsteroidal anti-inflammatory drugs such as ibuprofen or aspirin.³ Corticosteroids (oral prednisone), administered in doses of 40 mg to 80 mg daily for 4 to 6 weeks, have also been historically used to treat systemic sarcoidosis.⁷ If this route is taken, however, patients should be monitored for the consequences of chronic steroid use. 


For localized treatment of skin lesions and other cutaneous manifestations, topical or intralesional corticosteroids may be prescribed.⁷ A common intralesional steroid is triamcinolone acetonide given in doses of 3 mg/mL to 10 mg/mL at a 2- to 3-week interval.⁷ If intralesional corticosteroids prove ineffective, other standard therapies include systemic corticosteroids, low-dose subcutaneous or oral methotrexate, and antimalarial agents (chloroquine and hydroxychloroquine).⁷ 


The long-term prognosis of sarcoidosis is quite favorable. Almost half of the cases that present will resolve without treatment, and with treatment, the majority of cases resolve within five years.² Cutaneous manifestations of sarcoidosis may come about even after successful intervention, but these are usually treated with localized drugs as mentioned above. 


For the patient in our case, biopsy of the lesion revealed cutaneous sarcoidosis, which is a manifestation that occurs in up to one-third of patients with a systemic presentation of sarcoidosis. He was treated with injections of intralesional steroid, and improvement of the skin lesions was noted.

Treatment options for cutaneous sarcoidosis

Nonsteroidal anti-inflammatory drugs

Oral corticosteroids

Intralesional corticosteroids

Topical corticosteroids

Methotrexate

Chloroquine

Hydroxychloroquine

George V. Polson, BS, is a medical student and Maura Holcomb, MD, is a dermatology resident at Baylor College of Medicine in Houston. Nicole R. Bender, MD, is an internal medicine intern in the dermatology residency program at the Medical College of Wisconsin in Milwaukee. 


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This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read White papules and 
follicular abnormalities on a toddler’s face and Enlarging brown spots. Then take the post-test here.

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This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read White papules and 
follicular abnormalities on a toddler’s face and Enlarging brown spots. Then take the post-test here.

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