Abstract

Rosai-Dorfman disease (RDD) is a rare disorder of histiocytic origin with nodal, extranodal, and cutaneous classifications often presenting as eruptive rashes, most commonly affecting the face, trunk, and thigh. Due to its rarity, no consensus exists regarding standard of care for these lesions, as most knowledge regarding treatment is provided by case reports. To our knowledge, we report the first case of a patient with a recurrent lower extremity mass consistent with cutaneous RDD, despite surgical excision and topical steroids treatment. A 51-year-old female presented with recurrent RDD after failed treatment with clobetasol ointment and surgical excision at an outside practice. Examination revealed violaceous plaques with overlying scale, without lymphadenopathy. With the failure of the previous excision, the patient was prescribed 5 mg/ml intralesional Kenalog (ILK) injections to suppress inflammation of the lesion. She experienced improved clinical response to treatment and remission of active disease with residual post-inflammatory hyperpigmentation at 10 months after two rounds of injections, although the patient reported vast improvement at 5 months as well. Treatment of RDD is difficult due to its rarity, hindering clinical trial development. Therapeutics reported to be efficacious include topical, intralesional, and systemic steroids, retinoids, methotrexate, chemotherapy, cryotherapy, phototherapy, and radiation. Clinical management is variable and often requires multiple forms of treatment on a case-to-case basis, highlighting the need for larger clinical studies to establish a standard of care. Our case poses interest in the use of ILK for RDD and should be considered in recurrent cases after prior surgical excision.

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