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Cutaneous Manifestation of a Systemic Disease



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Am Fam Physician. 2004 Jan 1;69(1):145-146.

A 40-year-old man presented with erythematous and lavender nodules, as well as ulcerations on the nasal and malar areas of his face (see accompanying figure). These lesions had slowly enlarged during the previous eight years. His initial presentation was remarkable only for an erythematous papule to the right of the nose that was resistant to steroid treatment. He denied other symptoms, including itching or pain from the lesion, fatigue, shortness of breath, fever, chills, weight loss, or arthralgias. The patient had no ill contacts and no known immunocompromised state. Physical examination was remarkable only for the facial lesions. Chest radiograph was within normal limits without infiltrates or hilar fullness. Biopsy of a facial lesion showed epithelioid, noncaseating granulomas.

Question

Based on the patient's history and physical examination, which one of the following is the correct diagnosis?

A. Secondary syphilis.

B. Lepromatous leprosy.

C. Cutaneous tuberculosis.

D. Cutaneous sarcoidosis.

E. Discoid lupus erythematosus.

Discussion

The answer is D: cutaneous sarcoidosis. Cutaneous sarcoidosis occurs in 20 to 35 percent of patients with sarcoidosis. Cutaneous lesions fall into two categories: specific and nonspecific. Specific lesions include plaques, papules, nodules, scarring, or ulcerative lesions. Typically, they are brown, red, or purple lesions.1 Erythema nodosum is the most common nonspecific manifestation. The diagnosis of cutaneous sarcoidosis is based on its histologic appearance in the setting of typical systemic involvement. Symptoms include peripheral lymphadenopathy, fatigue, weight loss, dyspnea on exertion, shortness of breath, or cough. Chest radiograph often reveals hilar lymphadenopathy in sarcoidosis. Biopsy of the skin reveals the classic noncaseating sarcoid granulomas.1 Treatment may include systemic steroids, intralesional steroid injection, antimalarials, or methotrexate.

The differential diagnosis in this case includes secondary syphilis, lepromatous leprosy, cutaneous tuberculosis, and discoid lupus erythematosus. Secondary syphilis is characterized by scaling red-brown papules and plaques, typically smaller and flatter than those seen in this patient. The rash of secondary syphilis may appear anywhere on the body, including the palms and soles. Skin lesions appear six to 12 weeks following the primary chancre and last four to six weeks.2 Other common symptoms of syphilis at this stage include lymphadenopathy, fever, and arthralgias. Dark-field microscopic examination of a swabbed sample from the chancre may reveal the presence of vibrating spirochetes, but this is technically difficult and the chancre often has resolved by this stage.2 Serologic testing is the usual means by which secondary syphilis is confirmed.

Lepromatous leprosy, caused by the organism Mycobacterium leprae, is characterized by nondescript brown or red papules or plaques that commonly affect the ears and nose. Nodules also may develop. These lesions are usually bilateral, symmetric, and painful.2 Frequently, biopsy of the skin is diagnostic. Granulomas and acid-fast bacilli are noted.

Next on the differential is cutaneous tuberculosis, of which lupus vulgaris is the most common type. It is characterized by groups of reddish-brown nodules that have an “apple-jelly” color when blanched.3 Lupus vulgaris often is found on the face. Ulceration and scarring may occur. On biopsy, the physician may see caseating necrosis, epithelioid cells, Langerhans' giant cells, and lymphocytes. Acid-fast bacilli are rarely seen. Culture of lesions or polymerase chain reaction can be used to identify mycobacteria.3 The systemic manifestations of tuberculosis assist in differentiating this disease from sarcoidosis.

Discoid lupus erythematosus is a chronic disease characterized by inflammatory, scarring lesions. It has been reported that 20 percent of patients with systemic lupus erythematosus will develop discoid skin lesions; however, most patients who have only cutaneous lupus at presentation will not progress to systemic disease. The classic presentation is an inflammatory plaque with scaling, follicular plugging, telangiectasias, atrophic scarring, and central hypopigmentation.4 Common sites of involvement include the face, scalp, and ears. Skin biopsy typically reveals hyperkeratosis and follicular plugging, with superficial and deep lymphocytic infiltrates. Direct immunofluorescence of lesions characteristically shows immunoglobulin and complement deposition at the dermal-epidermal junction.4

JACQUELINE REARDON JONES, LT, MC, USNR

CHRISTOPHER L. PARMAN, LCDR, MC, USNR

Naval Hospital Jacksonville

2080 Child St.

Jacksonville, FL 32214

The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the U.S. Navy Medical Department or the U.S. Navy Service at large.

REFERENCES

1. Albertini JG, Tyler W, Miller OF 3rd. Ulcerative sarcoidosis. Case report and review of the literature. Arch Dermatol. 1997;133: 215–9.

2. Tomecki KJ, Dijkstra JW. Treponemes, rickettsia, and mycobacteria. In: Sams WM Jr, Lynch PJ, eds. Principles and practice of dermatology. New York: Churchill Livingstone, 1996:171–8.

3. Fitzpatrick TB. Color atlas and synopsis of clinical dermatology: common andserious diseases. 4th ed. New York: McGraw-Hill, 2001:662,664.

4. Lee LA. Lupus erythematosus. In: Sams WM Jr, Lynch PJ, eds. Principles and practice of dermatology. New York: Churchill Livingstone, 1996:581–98.

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