July/August 2001

4.5.

 

Please see case of the month for September 1998

HISTOPATHOLOGY: Revealed numerous non-caseating granulomas in the superficial and mid-dermis consistent with sarcoidosis.

LABORATORY FINDINGS: Liver and renal function tests, CBC, serum Ca level, Chest X-ray and eye examination were unremarkable. A serum ACE-Level was 79 (normal range: 10-53).

DISCUSSION: Sarcoidosis is a systemic noncaseating granulomatous disorder of unknown etiology. It most commonly affects the lungs, lymph nodes, eyes and skin. 20-35% of patients with sarcoidosis has cutaneous involvement. In general, sarcoid-specific skin lesions have no prognostic significance, do not show any correlation with the extent of systemic involvement, and do not indicate a more serious form of sarcoidosis. This is with the exception of erythema nodosum which has been associated with sarcoidosis that spontaneously resolves. A report investigating cutaneous sarcoidosis in Caucasian patients found that lupus pernio, scar infiltrates and plaque lesions are the most common clinical lesions in this population. It also showed that these lesions were most likely to be associated with systemic disease.

Sarcoidosis in scars has been reported in scars following trauma, surgery, venipuncture, tattoos, and pseudofolliculitis barbae. Although scar sarcoidosis can be limited to the skin, most patients described have evidence of systemic involvement (often hilar lymphadenopathy). It may occur with erythema nodosum. Usually, scar sarcoidosis appears early in the disease before involvement of the lungs.

Indications for treatment are based on disabling symptoms, organ dysfunction and/or disfigurement. Local treatment options are reserved for limited cutaneous disease and includes the following: topical and intralesional corticosteroids, CO2 or PDL laser, hydrocolloid dressings or dermabrasion. Systemic therapy is used for extensive disease or systemic disease. Options include glucocorticoids (first-line therapy), antimalarials, methotrexate, allopurinol, thalidomide, isotretinoin, PUVA, and recently, minocycline.

COURSE: The patient was given a slowly tapering course of prednisone as chloroquine phosphate (Aralen) 250 mg qd was initiated. She has improved clinically with this regimen and has been able to taper the prednisone to 10 mg qd. As her disease has become significantly limited, we plan to start minocycline 100 mg bid to hopefully hasten her prednisone taper.

REFERENCES:

1. Caro I. Scar sarcoidosis. (1983) Cutis: 32, 591- 593.

2. Manz LA, Rodman OG. Reappearance of quiescent scars. Sarcoidosis. (1993) Arch Derm:129,105 108.

3. Veien NK, Stahl D, Brodthagen H. Cutaneous sarcoidosis in Caucasians. (1987) JAAD:16, 534-540.

4. English JC, Patel PJ, Greer KE. Sarcoidosis. (2001) JAAD:44,725-743.

My thanks to Drs. Timothy Storer and Pui-Yan Kwok for their assistance in the preparation of this case.