Recalcitrant cutaneous sarcoidosis: an evidence-based sequential approach.

Background: Cutaneous sarcoidosis in black-skinned people is more severe and, in a subset, recalcitrant to therapy. Management of these patients is a challenge.

Aim: To document the clinical features of recalcitrant cutaneous sarcoidosis (RCS) and its response to sequential therapy. A treatment algorithm is suggested.

Methods: A cross-sectional retrospective analysis was made of patients with RCS. Demographic data, clinical features, histology, blood parameters, radiology and management and response to therapy were recorded.

Results: A total of 30 patients with cutaneous sarcoidosis were seen, of which six had recalcitrant lesions. All had black skin, with a male to female ratio of 1:5. The average age was 48.5 years (41-67) and the average duration of lesions was 11.3 years (2-29). Skin lesions were papules (three), plaques (four), annular (three), nodules (four), ulcers (one), alopecia (one) and lupus pernio (one). Extracutaneous involvement was noted in four of six patients as follows: pulmonary (three of six), dactylitis (two of six) and hepatosplenomegaly (one of six). Histopathology was undertaken in all confirmed non-caseating granulomas. None of the cases responded to systemic prednisone alone. Alternative therapies were: chloroquine (six of six), methotrexate (four of six), doxycycline (two of six), allopurinol (two of six) and isotretinoin (one of six), and azathioprine (one of six). All patients responded well to a stepwise approach to therapy using second-line agents with no relapses during the follow-up period.

Conclusion: Sequential therapy avoids the side effects of toxic drugs whilst controlling aggressive cutaneous lesions.