Slideshow
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CA0811Sarcoidosis1
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CA0811Sarcoidosis2
by Kristy Fleming, MD
A 48-year-old black man presented for evaluation of multiple skin plaques. He complained of several papules on his nose and nasal rim as well as larger plaques on his forehead, which had been present and slowly growing for many years. The lesions were asymptomatic, but cosmetically bothersome for the patient. He complained of difficulty breathing, but was otherwise healthy and not taking any medications. What’s your diagnosis?Submit your answer and read the full explanation below. If you like this activity or have a suggestion, tell us about it in the comment box at the bottom of the page.
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Sarcoidosis is a chronic systemic granulomatous disease of unknown origin affecting a variety of tissues including the lungs, skin, eyes, liver, brain, heart and kidneys. The skin is involved in approximately 25% of cases.1
Most patients present with symptoms between the ages of 20 and 40 years, though rarely children can also be affected.1 The course may be steadily progressive or interrupted by remissions and relapses. Sarcoidosis is more common in blacks and is often more recalcitrant to treatment in this population.
Cutaneous sarcoidosis manifestations are quite varied. Most sarcoid lesions are asymptomatic, but approximately 10% to 15% of patients complain of pruritus. Morphologic variants include: papules, nodules, plaques, subcutaneous nodules, lesions confined to scars or tattoos, erythroderma and ulcerations.1 Lesions may be verrucous, psoriasiform, ichthyosiform, hypopigmented, hyperpigmented or alopecic. This striking variability has led some to refer to sarcoidosis as one of “the great imitators.”
Small papules on the nares and nasal rim are a characteristic sarcoidosis finding. Lupus pernio, a more fulminant sarcoidosis form affecting the nose and central face, may lead to significant deformity and functional impairment with nasal perforation and collapse of the nasal bridge.1
Diagnosis
Sarcoidosis can often be suspected based on clinical findings, but demonstrated involvement of two separate organ systems is necessary to confirm the diagnosis. Almost all patients present with lung or chest symptoms.
Skin lesions are the least invasive organ to biopsy, and the histopathology of sarcoid presents as noncaseating “naked granulomas” in the dermis. Tissue culture is also performed to rule out an infectious cause of granulomas, such as deep fungal or atypical mycobacterial.
Ophthalmologic evaluation is also recommended and patients may require a conjunctival biopsy. Biopsying minor salivary glands may also demonstrate sarcoidal granulomas.
Imaging studies include chest radiograph to evaluate for bilateral hilar lymphadenopathy, pulmonary infiltrates and pulmonary fibrosis.
Laboratory workup consists of a complete blood count and differential, serum calcium and 24-hour urine calcium, serum angiotensin-converting enzyme level, liver function tests and renal evaluation.
Treatment
There are numerous treatment options available for cutaneous sarcoidosis. Most patients are primarily concerned with the lesions’ cosmetic appearance, particularly those that appear on the face.
Systemic corticosteroids are virtually universally effective, but long-term use is associated with significant side effects, and it is typical for lesions to promptly recur upon discontinuation.
Topical corticosteroids are safer, but much less effective. Limited skin disease lends itself well to intralesional injection of triamcinolone. Other current therapies include methotrexate, chloroquine, hydroxychoroquine, oral retinoids and occasionally cyclosporine when other modalities fail.2
Some patients require combination treatment, and others do not respond to conventional therapy at all. Recently, antibiotics have been introduced into the treatment regimen. Minocycline and doxycycline have been shown to be effective in small case series, sometimes resulting in complete remission with much safer adverse event profile than other systemic treatments.3
Kristy Fleming, MD, is a third year dermatology resident at Baylor College of Medicine.
References:
1. James WD, Berger TG, Elston DM. Andrew’s Diseases of the Skin Clinical Dermatology. 10th ed. Pennsylvania: Saunders Elsevier; 2006: 708-713.
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