Intralesional Kenalog Injections for Recurrent Cutaneous Rosai-Dorfman Disease (RDD) in the Lower Extremity

Victoria Jiminez, Lawangeen Zeb, Lauren Graham, Anna Gray
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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.
治疗下肢复发性皮肤罗赛-多夫曼病(RDD)的 KENALOG 局内注射疗法
罗赛-多夫曼病(RDD)是一种罕见的组织细胞源性疾病,有结节型、结节外型和皮肤型三种类型,通常表现为爆发性皮疹,最常累及面部、躯干和大腿。由于其罕见性,目前还没有就这些病变的治疗标准达成共识,因为有关治疗的大多数知识都是由病例报告提供的。据我们所知,我们报告了第一例复发性下肢肿块患者,尽管患者接受了手术切除和外用类固醇治疗,但其症状与皮肤 RDD 一致。一名 51 岁的女性患者在一家外院接受氯倍他索软膏和手术切除治疗失败后,复发了 RDD。检查发现该患者身上出现粟粒大小的斑块,上覆鳞屑,无淋巴结肿大。由于之前的切除手术失败,患者被处方注射 5 毫克/毫升的凯纳洛(ILK)以抑制皮损的炎症。经过两轮注射后,她的临床治疗反应有所改善,10 个月时活动性疾病得到缓解,但仍有炎症后色素沉着残留,不过患者称 5 个月时情况也大有好转。RDD 的治疗因其罕见性而困难重重,阻碍了临床试验的发展。据报道,有效的治疗方法包括局部、鞘内和全身类固醇、维甲酸、甲氨蝶呤、化疗、冷冻疗法、光疗和放射治疗。临床治疗方法多种多样,通常需要根据具体病例采取多种治疗方法,因此需要进行更大规模的临床研究,以确定治疗标准。我们的病例引起了人们对使用 ILK 治疗 RDD 的兴趣,在手术切除后复发的病例中应考虑使用 ILK。
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