Sarcoidosis of the skin

Sarcoidosis is a multi-system disease of unknown cause. It most often affects young and middle-aged people, more women. It is often manifested by bilateral hilar adenopathy, pulmonary infiltrates, eye and skin lesions. The liver, spleen, lymph nodes, salivary glands, heart, nervous system, muscles, bones and other organs can also be affected.

Forms of skin involvement are divided into non-specific and specific according to the presence or absence of non-caseating granulomas during biopsy.

Epidemiology
The disease occurs worldwide. About 25% of patients are affected. Specific disability occurs in 10,5% of cases in our country. Lupus pernio appears more often in blacks and Indians, and affects especially older women.

Symptoms
The most common form of non-specific skin lesion is erythema nodosum (28,2% in our country). It is a symptom of acute sarcoidosis and often occurs in Europeans. It usually disappears within 6-8 weeks. Erythema nodosum in association with infections, neoplasia, vasculitides, and drug reactions is not pathognomonic for sarcoidosis. Erythema nodosum as part of Löfgren’s syndrome together with fever and bilateral hilar lymphadenopathy (BHL) is so specific for sarcoidosis that it does not require histological evidence of non-caseating granulomas in the case of spontaneous and rapid resolution symptoms.

The most common specific lesion is lupus pernio. It represents chronic sarcoidosis. It manifests as indurated surfaces, painless, non-itchy, without ulceration, associated with discoloration of the nose, lips, eyelids and scars. It is often associated with bone cysts, involvement of the nasal mucosa and lung fibrosis. Spontaneous remissions are rare.

Another specific skin manifestation can be maculopapular eruptions, subcutaneous nodules, infiltration of old scars and skin plaques.

Therapy
For erythema nodosum non-steroidal anti-rheumatic drugs are usually sufficient. For small sarcoid papules and plaques, monthly injection intrapapular administration of triamcinolone, or weekly topical treatment with corticoids and hydrocolloids. Large or disfiguring lesions require systemic treatment. New infliximab, allopurinol, thalidomide.
 * 1) alternative regimen 30 mg Prednisone
 * 2) weekly pulse regimen with Methotrexate
 * 3) hydrochloroquine

Histology
Sharply circumscribes circular non-caseating granulomas. Composed of epithelioid macrophages (histiocytes), surrounded by a negligible lymphocytic infiltrate. They are therefore referred to as "naked".

Related articles

 * Sarcoidosis (pathology)
 * Sarcoidosis (internal)

Source

 * ANTON, Jan. Materials for the lecture "Sarcoidosis".