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Limited Stage IA mycosis fungoides (MF) is often treated with topical steroids, which can cause atrophy, or with nitrogen mustard, which imposes several limitations on the patient's lifestyle. Topical bexarotene is a novel synthetic rexinoid with few side-effects that has shown efficacy for treatment of mycosis fungoides skin lesions in recent Phase II–III clinical trials. The Phase I–II trial involving 67 stage IA–IIA MF patients demonstrated complete response (CR) in 21% and partial response (PR) in 42% of the patients. The median time to response was approximately 20 weeks. In the phase III trial of refractory stage IA, IB and IIA MF, the patients demonstrated a 44% response rate (8% CR). Patients with no prior therapy for mycosis fungoides responded at a higher rate (75%) than those with prior topical therapies.Case report of a patient with MF limited to the hands treated with topical bexarotene 0.1% gel in a open label phase II clinical trial.Partial response occurred after 2 weeks of topical bexarotene therapy and the lesions were well controlled for 5 years using bexarotene monotherapy, with only occasional mild local irritation.Topical bexarotene is effective as long-term treatment monotherapy for limited MF lesions. To our knowledge this is the longest use of the drug by any individual.