SARCOIDOSIS

By Gary M. White, MD

Sarcoidosis of the skin


Sarcoidosis is a multisystem disease of exclusion characterized by noncaseating granulomas. An infection or presence of foreign body must be excluded. It can affect many organ systems including the skin, lungs, eye and bone.

Clinical

Sarcoidosis is a systemic disease and virtually any organ of the body may be affected. Cutaneous sarcoidosis may manifest itself in the skin in many ways including papules, plaques, nodules, annular lesions, ulcers, ichthyosis, scar infiltration, erythroderma, lupus pernio, ungual lesions, and erythema nodosum. Ocular (uveitis, iris nodules, conjunctivitis), pulmonary, musculoskelatal (polyarthritis, bone cysts) and lymph node involvement are common. Granulomas may be found in the kidneys, heart and elsewhere. Patients with sarcoidosis are at increased risk for malignancy, e.g. lymphoma.

Different skin lesions can have different implications. Lupus pernio and plaques are associated with more severe disease, whereas erythema nodusum is classically associated with acute and often self-limited disease. Maculopapular lesions often confer a more benign course. Subcutaneous nodular lesions are often reported with more systemic forms.

Scarring alopecia from infiltration of the scalp may occur. It is most common in African-American females in the forth to fifth decades.

Ulcerative sarcoidosis occurs rarely [Cutis 2017;100;312-316]. It is most common in black women. Ulcers may develop de novo or in preexisting sarcoidal plaques. The legs are most commonly involved.

Darier-Roussy sarcoidosis, or subcutaneous sarcoidosis, presents with multiple, mobile, non-tender subcutaneous nodules with no epidermal change. They usually are found on the arms. The most common systemic association is pulmonary sarcoidosis.

Tattoos

Sarcoidosis may present as nodular skin lesions localized to either single or multiple colors within a tattoo. The tattoo may be affected soon after placement or up to 45 years later. Patients may have a negative workup only to later develop sarcoidosis or sarcoidal granulomas in a tattoo may reveal asymptomatic lung and nodal involvement. Treatment for these is similar to non-tattoo related sarcoidosis [Dermatology Online Journal 2014;20;8].

Workup

Workup up by a non-dermatologist is usually done.

Treatment

Antimicrobial Therapy

Antimicrobial treatment improved sarcoidosis in a RPC trial of 30 patients. Concomitant levofloxicin, ethambutol, azithromycin and rifampin therapy decreased lesion size and granuloma burden over 8 weeks treatment. The tetracyclines have been used with benefit. For example, minocycline 100 mg po BID was very beneficial in a case series [Arch Dermatol. Jan 2001;137(1):69-73].

Intralesional Triamcinolone

IL TMC 2.5-5.0 mg/cc q 3-4 weeks may be done.

Prednisone

Systemic steroids are consistently helpful for patients with sarcoidosis, but side effects limit its longterm use.

Leflunomide

Leflunomide (Arava) is an inhibitor of pyrimidine synthesis that has immunomodulating/immunosuppressive properties. It has been used off-label for patients with sarcoidosis and has shown benefit for both the respiratory tract and skin.

Adalimumab

Adalimuumab improved cutaneous sarcoidosis in a DBPC trial over 12 weeks. It did not improve pulmonary function, nor CXR findings.

Infliximab

Infliximab is relatively effective, but infections are a potential side effect [JAMA Derm 2017;153;681].

Hydroxychloroquine

e.g. 200-400 mg/day

Chloroquine

e.g 250-500 mg/day,

Methotrexate

e.g. MTX 10-15 mg once a week

Thalidomide

Thalidomide is ineffective for cutaneous sarcoidosis [Chest 2014 Oct 1; 146:1046].

Pediatrics

Oral steroids may be used for patients with systemic disease. Methotrexate has been reported as useful in reducing the amount of steroid needed. Allopurinol 200 mg/day cleared sarcoidosis after 4 months in a 15 year-old boy [BJD 1999;140;1184].

Additional Pictures

Sarcoidosis of the skin Sarcoidosis of the skin Sarcoidosis of the skin Sarcoidosis of the skin Sarcoidosis of the skin

Sarcoidosis may cause hypopigmentation.
Sarcoidosis of the skin causing hypopigmentation

References

An. Bras. Dermatol. vol.89 no.4 Rio de Janeiro July/Aug. 2014

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