Antimalarials such as chloroquine and hydroxychloroquine are 4-aminoquinolones and have been shown to be effective in treating connective tissue diseases. While no randomised placebo-controlled studies exist to evaluate their effectiveness in cutaneous sarcoidosis, a number of open non-randomised non-controlled prospective studies and one comprehensive literature review have been conducted. In 1991, Zic et al.29 published a literature review of studies evaluating the use of antimalarials for treatment of sarcoidosis. They concluded that while corticosteroids should remain first-line treatment for patients with extracutaneous sarcoidosis, chloroquine should be strongly considered in patients for whom the main indication for treatment is disfiguring cutaneous lesions. Zic et al. recommended an initial 14-day course of chloroquine, 500 mg/day, followed by long-term therapy with 250 mg/day. These conclusions were based on the following studies.
Morse et al.31 conducted the first open prospective trial of chloroquine for the treatment of cutaneous sarcoidosis in 1961. The trial included seven patients with chronic cutaneous sarcoidosis (i.e. 2 or more years duration) treated with chloroquine, 500 mg/day for 6 months. All had improvement of their skin lesions. Four out of seven relapsed after treatment was withdrawn, but responded again when therapy was reinstated.
Hirsch et al.32 presented a case series of eight patients with chronic sarcoidosis (i.e. 5-15 years duration) treated with chloroquine, 500 mg/day, for 6 months. Seven of the eight patients had cutaneous lesions, which all improved with therapy. Six of the eight patients also had pulmonary disease - only two of the six pulmonary lesions improved.
Stilzbach et al.33 conducted an open prospective study of 43 patients with pulmonary sarcoidosis treated with chloroquine, 500 mg/day. Fourteen of the 43 patients also had skin lesions, all of whom saw improvement of their skin lesions with therapy. In contrast, only 31 of the 43 patients had improvement of their pulmonary lesions.
Brodthagen et al.34 published a study of 15 patients with cutaneous sarcoidosis treated with hydroxychloroquine, 500-1000 mg/day. Only two patients had improvement of their lesions after 6 months of therapy.
Johns et al.35 report on an open prospective trial of 25 black patients on maintenance corticosteroid therapy who had regression of their skin lesions with the addition of chloroquine, 500 mg/day.
Finally, Jones et al.36 report the results of a non-randomised open study of 17 patients with steroid-resistant cutaneous sarcoidosis treated with hydroxychloroquine, 2-3 mg/kg/day, for at least 3 months. Twelve patients improved and were able to discontinue other therapies. Five patients did not improve and three had worsening of their cutaneous disease. Of the twelve patients who were successfully treated, six discontinued the hydroxychloroquine and had re-emergence of their lesions, which again regressed when hydroxychloroquine was reinstated.
In addition to studies mentioned in the literature review by Zic et al, Johns et al.26 reported the results of an unpublished retrospective review by Hackett and Hambrick describing treatment of cutaneous sarcoidosis at the Johns Hopkins Sarcoid Clinic. In this study, 15 of 18 patients treated with chloroquine had improvement of their lesions. Chloroquine was typically administered for 6 months, followed by 6 months off treatment to avoid ocular toxicity. Multiple courses of therapy were often required.
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