Sarcoidosis: thalidomide treatment in ten patients

Background. Acute cutaneous sarcoidosis is generally spontaneously regressi ve but persistent chronic cutaneous lesions are esthetically prejudicial. T here have been several case reports on thalidomide efficacy but long-term o utcome is unknown. We report results in 10 cases oi cutaneous sarcoidosis t reated with thalidomide. Patients and methods. Data from ten patients with sarcoidosis tretaed with thalidomide between January 1998 and March 1999 were collected from deliver y authorizations and analyzed. All ten patients had chronic cutaneous sarco idosis resistant to conventional therapy. Six patients had an associated vi sceral localization and disease duration of 2 to 18 years (median 6 years). We considered that regression was complete when erythema and infiltration had totally disappeared, that regression was incomplete when cutaneous sign s remained, and that treatment had failed when no effect was observed or wh en the disease worsened. Results. Disease regression was noted in 7 patients for a daily dose of 1.8 4 mg/kg for 2.8 months. Skin lesions totally regressed in 3 patients, an in completely in 4. Treatment failed in 3 patients. Patients were treated for to months (2 to 21 months). The daily dose of thalidomide was gradually red uced in 5 of 7 patients for whom treatment was effective. Th ree of these 5 patients relapsed and thalidomide was again given and was effective again at the same dose and after the same delay. We observed improved kidney func tion in one patient, improvement in nasal infiltration in one other and com plete regression in 3 patients who achieved long lasting reduction in angio tensin convertase level. When treatment failed, the daily dose was 1.15 mg/ kg and the treatment had to be stopped for 2 patients. Side effects were mi nor. excepting 2 cases of neuropathy. Discussion. This open study of 10 patients treated with thalidomide showed the efficacy of a 1.84 mg/kg daily dose in 7 out of 10, but complete regres sion of the lesions was obtained in only 3 patients. Thalidomiide appears t o suspend the disease, with relapse when the drug is discontinued and effic acy at re-introduction. This would argue against a placebo effect. The mode of action could involve immunomodulating and antiinflammatory mechanisms.