Appearance on the skin
Skin manifestations of sarcoidosis are also relatively common, affecting up to one third of patients.1,7 Cutaneous sarcoidosis is divided into nonspecific and specific lesions. Nonspecific skin lesions do not contain granulomas on biopsy.
Erythema nodosum, the most common nonspecific skin lesion of sarcoidosis, is commonly seen in European, Puerto Rican, and Mexican patients.8 It presents as tender, subcutaneous erythematous nodules on the lower legs.
Lofgren’s syndrome is the combination of erythema nodosum, bilateral hilar lympha-denopathy, fever, and polyarthritis.
Specific sarcoid lesions may present as cutaneous papules, plaques, or enlarging scars.
Lupus pernio is another specific skin lesion characteristic of sarcoidosis. It presents as chronic, indurated, violaceous plaques on the nose, cheeks, lips, ears, or nasal mucosa. Lupus pernio is more common in African American women.8
Other manifestations
Ocular manifestations of sarcoidosis include uveitis or chorioretinitis and are present in 27% of patients with sarcoidosis.1
Sarcoidosis in peripheral lymph nodes, spleen, and liver is seen in 10% to 30% of patients with sarcoidosis; however, these tend to be incidental findings.1 Occasionally, hepatomegaly and splenomegaly are present.
Liver enzymes are typically normal, however a mildly elevated alkaline phosphatase is sometimes seen.1 Neurological, cardiac, and renal involvement are uncommon, affecting less than 5% of patients with sarcoidosis.1,13 Hypercalciuria with or without hypercalcemia is commonly seen secondary to overproduction of 1,25-vitamin D.8
Differential diagnosis
The differential diagnoses of solid splenic lesions can be divided into 3 categories: granulomatous disease, metastasis, and primary masses. Granulomatous diseases include tuberculosis, histoplasmosis, and sarcoid.
Metastasis is most commonly from melanoma, lymphoma, breast, or lung cancer, although prostate, colon, stomach, ovarian, and pancreatic metastasis have occurred. Primary masses include hemangioma, hemangiosarcoma, lymphangioma, and splenic infarction. Occasionally, regenerating nodules of hematopoeisis are seen in the spleen.
Testing: A Diagnosis Of Exclusion
The diagnosis of sarcoidosis is ultimately a diagnosis of exclusion. No laboratory tests can confirm the diagnosis of sarcoid. However, serum levels of angiotensin-converting enzyme are elevated in 50% to 80% of patients with sarcoidosis. Also, cutaneous anergy to common antigens (mumps, candida) is seen in 50% of patients with sarcoidosis.1,9
Once the diagnosis of sarcoidosis is made, additional clinical information is needed. In addition to a detailed history and physical, a chest x-ray, pulmonary function tests (PFT), electrocardiogram, comprehensive metabolic profile, and slit lamp evaluation should be obtained.
Some clinicians follow angiotensin-converting enzyme levels to monitor disease activity; however, treatment is ultimately based on the patient’s symptoms.
Treatment: Corticosteroids Treat The Symptoms
Corticosteroids are the cornerstone of treatment for sarcoidosis. Asymptomatic, early disease can be observed with routine follow-up. Oral corticosteroids should be prescribed for symptomatic cardiac, neurologic, and ocular sarcoidosis. Oral corticosteroids should also be prescribed for any symptomatic pulmonary disease, especially in the setting of worsening PFTs, severe hypercalcemia, and painful or disfiguring skin lesions (LOE: 2a).7,8,10
For symptomatic or worsening pulmonary sarcoidosis, patients are prescribed prednisone 20 to 40 mg daily for 3 months. If the patient responses to prednisone, it should slowly be tapered to 5 to 10 mg and continued for a period of 12 months.7,11,12
Topical steroids can be used for uveitis and skin lesions;1,8 the latter also respond well to intralesional steroids.7 Inhaled corticosteroids are now being used to treat pulmonary disease, but their efficacy is uncertain.1,8,10
Of patients being treated with corticosteroids, 25% to 40% will relapse and require further treatment.1,8 The addition of methotrexate, azathioprine, or hydroychloroquine is sometimes required in recalcitrant cases of sarcoidosis (LOE: 2b).1,8,11