Evidence-based therapy for cutaneous sarcoidosis
Introduction
The treatment of cutaneous sarcoidosis has been usually derived from agents used for pulmonary sarcoidosis. Table 1 lists the agents described as useful for sarcoidosis in general. The purpose of this report is to discuss the individual agents for sarcoidosis in general and for cutaneous disease in particular. The information regarding therapy for sarcoidosis will be as evidence based as possible. Recent evidence-based guidelines for therapy for sarcoidosis have been made.1 The recommendations pointed out, however, provided limited information regarding treatment of cutaneous disease. The limited information about the treatment of extra pulmonary disease has been noted.2
This article will focus on the conventional agents used for sarcoidosis. There has been a recent interest in the use of biologic agents for sarcoidosis, including cutaneous disease.
In another section, Dr Sweiss will discuss some of these newer agents proposed for cutaneous sarcoidosis.
Section snippets
Corticosteroids
Since the original reports of the utility of corticosteroids for sarcoidosis,3 corticosteroids have been the mainstay of therapy for systemic sarcoidosis. Glucocorticoids have been used for cutaneous sarcoidosis. Topical therapy with high-potency fluorinated corticosteroids is commonly used in patients with localized disease. In some cases, the addition of an occlusive dressing to enhance local absorption has proved effective in refractory skin lesions.4 High-dose topical glucocorticoids have
Antimalarials
The antimalarial agents chloroquine and hydroxychloroquine have been used for many years in rheumatologic diseases such as lupus erythematosus and rheumatoid arthritis.24 These drugs have been used in sarcoidosis for over 40 years.25, 26, 27 In a recent evidence-based review of noncorticosteroid treatment of pulmonary sarcoidosis, antimalarials were felt to be possibly useful for treatment.28 The rate of response appears to be higher for cutaneous compared with pulmonary sarcoidosis.26, 29
Methotrexate
Methotrexate has been the most widely studied nonsteroidal therapy for sarcoidosis. It has been shown to steroid sparing in acute pulmonary sarcoidosis.36 It has been reported as useful by several groups for chronic pulmonary,37, 38 ocular,39, 40 and neurologic41 sarcoidosis. It has been recommended as an alternative to corticosteroids for pulmonary disease.28
Several authors have reported on the use of methotrexate for cutaneous disease.37, 42, 43, 44, 45, 46 Fig. 2 summarizes the studies with
Thalidomide
Thalidomide was first used as a hypnotic in the 1950s. It was withdrawn from the market because of its teratogenic effects. It was then approved by the United States Food and Drug Administration for erythema nodosum leprosum. It has been subsequently found useful in the treatment of a wide variety of skin lesions.59, 60 The agent has multiple effects, including the blockade of tumor necrosis factor (TNF). This led to its study in patients with sarcoidosis.61
The response of thalidomide in
Minocycline and other agents
Minocycline has been reported as useful in treating cutaneous sarcoidosis. In 1 report of 12 patients with chronic cutaneous sarcoidosis treated with 200 mg a day of minocycline, 8 had complete response and 2 had partial response.69 Two of the patients were switched to doxycycline because of toxicity and had continued response. The mechanism of action is unclear. One possibility is the antibacterial effect, including the activity against Propionibacteria acnes, a putative agent for sarcoidosis.
Conclusions
Several agents have been used successfully for cutaneous sarcoidosis. Most of the evidence for these agents, however, is based on case reports and case series. Many of the recommendations for treatment have been extrapolated from studies of pulmonary sarcoidosis. Despite these limitations, there is sufficient evidence to give guidelines for the treatment of cutaneous sarcoidosis with various agents. These include new agents that are specifically directed against tumor necrosis factor.
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