Sarcoidosis and the skin: manifestations, symptoms and treatment

Skin manifestations of sarcoidosis
Skin changes in sarcoidosis are varied and may be the first or only sign of the disease. They differ in appearance, location, colour and progression.

Erythema nodosum
Erythema nodosum is one of the most common skin manifestations of sarcoidosis. It causes painful red nodules, most often on the front of the lower legs, although the thighs and forearms may also be affected. It usually indicates an acute form of sarcoidosis and often disappears spontaneously within several weeks to a few months without specific treatment. It is commonly accompanied by fever, fatigue and joint pain.

Lupus pernio
Lupus pernio is a chronic skin manifestation that most often affects the face, especially the nose, cheeks, ears and lips. It causes a red-purple discoloration, swelling and thickening of the skin. These lesions rarely resolve spontaneously and often require prolonged treatment with corticosteroids or immune-modifying medicines. Lupus pernio may be associated with more severe sarcoidosis affecting the lungs and upper airways.

Maculopapular rash
A maculopapular rash consists of small red or purple papules that may appear on the face, trunk or limbs. It is usually painless and does not leave scars, although it may cause distress because of its appearance. In many cases it is a sign of systemic sarcoidosis.

Scar sarcoidosis
Scar sarcoidosis occurs when granulomas form within existing scars, tattoos or areas of previous injury. This can change the colour of the skin and thicken the lesions. It may persist for years and requires specialist assessment.

Plaques and nodules
Plaques are well-defined, infiltrated lesions that often occur on the limbs and face. Nodular sarcoidosis can resemble other dermatological diseases, making diagnosis more difficult. Lesions may last for years if untreated.

Symptoms and diagnosis
Skin sarcoidosis may be asymptomatic or may cause itching, pain or a feeling of tightness. In some cases, lesions can ulcerate and lead to secondary infection.

Diagnostic assessment may include:
• Dermoscopy, for detailed examination of skin lesions.
• Skin biopsy, which can help confirm the diagnosis by showing characteristic non-caseating epithelioid granulomas.
• Blood tests, including angiotensin-converting enzyme (ACE), calcium and other inflammatory markers.
• Chest X-ray and CT, to assess possible lung involvement.
• Pulmonary-function tests, when systemic disease or respiratory involvement is suspected.

Factors that may worsen symptoms
Sun exposure and ultraviolet radiation may aggravate lesions in photosensitive patients. Bacterial or viral infections can trigger inflammatory reactions. Chronic stress may worsen overall symptoms. Cold and dry air can crack the skin and aggravate symptoms in people with lupus pernio. Smoking may adversely affect the course of systemic sarcoidosis. Some medicines can cause or worsen sarcoidosis-like reactions; medication changes should always be discussed with a doctor.

Treatment
Treatment depends on symptom severity, the extent of the lesions and whether other organs are involved.

Topical treatment
Mild disease may be treated with topical corticosteroid creams or gels. Calcineurin inhibitors such as tacrolimus or pimecrolimus may be useful on sensitive areas such as the face. Corticosteroid injections directly into individual persistent lesions may also be considered.

Systemic treatment
More severe disease may require oral corticosteroids such as prednisone. Methotrexate, hydroxychloroquine or azathioprine may be used as steroid-sparing immune-modifying treatments. Anti-TNF medicines such as infliximab or adalimumab may be considered in resistant cases. Phototherapy, including UVB or PUVA, may help reduce lesions in selected patients who cannot take systemic medicines.

Sarcoidosis and your skin: diagnosis and treatment
When sarcoidosis develops in the skin, it is often an early sign of the disease, so a dermatologist may be the first doctor to notice it. Signs and symptoms can appear and disappear; keeping a record of changes can help doctors recognise the pattern.

How dermatologists diagnose sarcoidosis
There is no single blood test or X-ray that can prove sarcoidosis. If you are already seeing a dermatologist, you may need assessment by other specialists because sarcoidosis can affect the lungs, eyes, liver and other organs.

During a skin examination, the dermatologist looks for lumps, bumps and other changes, sarcoidosis developing in scars or tattoos, and lymph nodes near the lesions. The aim is to identify granulomas, which are masses of inflamed tissue. If a suspicious lesion is found, a skin biopsy can usually be performed safely during an outpatient visit. You remain awake while a small sample of affected skin is removed and examined under a microscope. A biopsy cannot by itself prove sarcoidosis, but it can exclude other conditions such as infection. Sarcoidosis is often diagnosed after other diseases with similar signs and symptoms have been excluded and the results of appropriate tests have been compared.

How dermatologists treat skin sarcoidosis
Mild disease may sometimes clear without treatment, over a period ranging from several weeks to several years. Dermatologists are more likely to recommend treatment when sarcoidosis may cause thickening or scarring, when smooth bumps or growths are present, when a large area of skin is involved, or when pain or itching occurs.

Treatment is tailored to the appearance and extent of the lesions, overall health, age and other factors. A corticosteroid applied to the skin may be sufficient when only a few lesions are present. It is often applied twice daily; sometimes a corticosteroid is injected into the lesions. More extensive disease may require an antimalarial medicine, methotrexate, an antibiotic or prednisone. Laser or light therapy may be another option, but several sessions may be needed and side effects can include colour changes, bleeding and crusting. Skin sarcoidosis can be persistent, and the first treatment may not work; the dermatologist may add another treatment or change the plan.

Outlook
There is no universal cure for sarcoidosis, but in many people the skin clears on its own. Sarcoidosis can also develop in organs such as the lungs, eyes or liver, so ongoing medical follow-up is important while the disease is active. Early recognition and treatment of organ involvement may help prevent more serious problems.

Conclusion
Skin sarcoidosis can range from a mild rash to severe chronic lesions. Early recognition and appropriate treatment are important for disease control. Because internal organs may also be involved, care may require collaboration between dermatologists, pulmonologists, rheumatologists and other specialists.

References
Haimovic A, Sanchez M, et al. “Sarcoidosis: A comprehensive review and update for the dermatologist. Part I. Cutaneous disease.” Journal of the American Academy of Dermatology. 2012;66:699.e1–18.
Haimovic A, Sanchez M, et al. “Sarcoidosis: A comprehensive review and update for the dermatologist. Part II. Extracutaneous disease.” Journal of the American Academy of Dermatology. 2012;66:719.e1–10.
Source: https://www.aad.org/public/diseases/a-z/sarcoidosis-treatment

This page is educational and does not replace an examination or individual medical advice.