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口服环孢素 A 成功治疗罗厄尔综合征。

Successful treatment of Rowell syndrome using oral cyclosporine A.

机构信息

Department of Dermatology, Saarland University Hospital, 66421 Homburg/Saar, Germany.

出版信息

Int J Dermatol. 2011 Aug;50(8):1020-2. doi: 10.1111/j.1365-4632.2010.04848.x.

DOI:10.1111/j.1365-4632.2010.04848.x
PMID:21781081
Abstract

BACKGROUND

To present and discuss successful treatment of Rowell syndrome with low dose orally administered cyclosporine in a male patient with refractory course of disease.

METHODS

A 63-year-old male patient presented with a five-week history of widespread, severely pruritic erythematous skin changes that were first seen on the back. Within a few weeks, skin rashes extended to the whole body recessing the face. Because treatment with topical and orally administered corticosteroids was ineffective, we began an immunosuppressive therapy using cyclosporine. Treatment was initiated with an orally administered dosage of 100mg/day and subsequent dose reduction to 50mg/day after 14 days.

RESULT

After four weeks of treatment, all clinical signs were resolved; only postinflammatory hyperpigmentation persisted for several weeks. Treatment-related side effects included arterial hypertension that could be controlled with antihypertensive drugs.

CONCLUSION

Primarily described in 1963, Rowell syndrome represents the combination of cutaneous lupus erythematosus and erythema multiforme in a single individual. Controversial discussion concerning the genuine character of the disease is still going on, and even the existence of this entity remains unclear. Several therapeutic regimes are known, including corticosteroids, methotrexate, or dapsone. To the best of our knowledge, this is the first report of successful treatment of Rowell syndrome with low doses of cyclosporine. Orally administered low-dose cyclosporine seems to be a potent alternative treatment option for patients with Rowell syndrome refractory to standard therapies.

摘要

背景

介绍并讨论一例难治性罗厄尔综合征(Rowell 综合征)患者,采用低剂量环孢素口服治疗获得成功。

方法

一名 63 岁男性患者,全身广泛出现严重瘙痒性红斑性皮损,病史 5 周。最初见于背部,数周内皮疹蔓延至全身,面部未受累。因外用和口服皮质类固醇治疗无效,我们开始采用环孢素进行免疫抑制治疗。初始剂量为 100mg/d,口服 14 天后减至 50mg/d。

结果

治疗 4 周后,所有临床体征均消退;仅遗留数周的炎症后色素沉着。治疗相关的副作用包括高血压,可通过降压药物控制。

结论

罗厄尔综合征于 1963 年首次描述,为单个患者同时出现红斑狼疮样皮疹和多形红斑样皮疹。关于该病的真实性质仍存在争议,甚至该疾病的存在也不明确。有多种治疗方案,包括皮质类固醇、甲氨蝶呤或氨苯砜。据我们所知,这是首例采用低剂量环孢素成功治疗罗厄尔综合征的报道。口服低剂量环孢素似乎是对标准治疗方案无效的罗厄尔综合征患者的有效替代治疗选择。

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