Cutaneous Sarcoidosis

Background

• Sarcoidosis is a systemic disease of unknown cause characterized by formation of immune granulomas in any organ, most commonly lungs and intrathoracic lymph nodes.
• Cutaneous involvement affects 15%-30% of patients with systemic sarcoidosis, while isolated cutaneous sarcoidosis affects 4%-5%.
• Controversy exists as to whether isolated cutaneous sarcoidosis is a type of sarcoidosis or should be considered a separate clinical entity, that is, sarcoid-like granulomatous disease of unknown significance.
• Cutaneous sarcoidosis lesions are either specific (containing noncaseating granulomas) or nonspecific (inflammation without noncaseating granulomas).
• Most common lesions include erythema nodosum, macules and papules, and plaques. Lupus pernio is the most characteristic cutaneous manifestation of sarcoidosis.

Evaluation

• Suspect cutaneous sarcoidosis based on clinical appearance of cutaneous lesions.
• Perform punch biopsy of cutaneous lesions other than erythema nodosum, which do not contain granulomas. Key histologic finding is presence of noncaseating epithelioid granulomas.
• Lofgren syndrome may be diagnosed clinically without the need for biopsy in patients with acute presentation of erythema nodosum, arthritis, and bilateral hilar adenopathy.
• All patients with suspected sarcoidosis (including those with only cutaneous manifestations) should have a complete evaluation to assess for multisystem disease, which should include pulmonary, cardiac, neurological, and ocular testing.

Management

• There is no cure for sarcoidosis and treatment only impacts the granulomatous process and its clinical consequences.
• Treatment of sarcoidosis should focus on therapy aimed at the most severely affected organ. Treatment of systemic organ involvement often improves cutaneous symptoms.
• There are several therapeutic options for cutaneous sarcoidosis, although most efficacy data is limited to small case series or case reports.
  • Local therapy with high-potency topical steroid, intralesional corticosteroid, or topical tacrolimus may be sufficient in patients with limited cutaneous sarcoidosis such as a few papules or plaques.
  • Consider systemic immunosuppressive therapy with low-risk agents such as antimalarial drugs, tetracycline antibiotics, or phosphodiesterase type 4 inhibitors for patients who do not respond to local therapy or for those with more extensive cutaneous disease.
  • Consider more potent immunosuppressive therapy such as corticosteroids, methotrexate, tumor necrosis factor antagonists, or thalidomide for patients with severe, disfiguring, or ulcerative cutaneous disease or systemic disease.
  • Combinations of systemic therapies may be considered with or without adjunct local therapy.
• Treatment may be deferred for patients with limited, stable, or asymptomatic cutaneous disease or Lofgren syndrome, which often resolves spontaneously.
• Interventions aimed at slowing the granulomatous process are notoriously slow, with objective response often taking up to 3 months.