Cohen Philip R, Kurzrock Razelle
University of California San Dieog.
Dermatol Online J. 2014 Jan 15;20(1):21241.
Anakinra is a recombinant form of interleukin-1 receptor antagonist. It is the drug of choice for Schnitzler syndrome and cryopyrin-associated periodic syndromes. It has also recently been demonstrated to have activity in the treatment of the non-Langerhans cell histiocytosis known as Erdheim-Chester disease.
To describe the activity of anakinra in a patient with co-existing lichen planus and Erdheim-Chester disease.
A 43-year-old woman with progressive Erdheim-Chester disease presented for management of her night sweats and chills, systemic skeletal bone pain, and neurologic (diabetes insipidus) manifestations. She also had widespread cutaneous lichen planus. Anakinra, 100 mg subcutaneously daily, was initiated for the treatment of her Erdheim-Chester disease.
Within 2 days of starting anakinra, there was prompt resolution of her Erdheim-Chester disease-related symptoms. Subsequently, her bone pain resolved and her diabetes insipidus improved. Also, the lichen planus-associated pruritus rapidly ceased and most of the skin lesions improved.
Our experience confirms the efficacy of anakinra for the treatment of Erdheim-Chester disease. The concomitant improvement of her lichen planus on anakinra suggests that this agent warrants additional study in this disorder.
阿那白滞素是白细胞介素-1受体拮抗剂的重组形式。它是施尼茨勒综合征和冷吡啉相关周期性综合征的首选药物。最近还证明它在治疗一种名为 Erdheim-Chester 病的非朗格汉斯细胞组织细胞增生症方面具有活性。
描述阿那白滞素在一名同时患有扁平苔藓和 Erdheim-Chester 病患者中的活性。
一名患有进行性 Erdheim-Chester 病的 43 岁女性前来治疗盗汗、寒战、全身性骨骼疼痛和神经学（尿崩症）表现。她还患有广泛的皮肤扁平苔藓。开始皮下注射阿那白滞素，每日 100mg，用于治疗她的 Erdheim-Chester 病。
开始使用阿那白滞素后 2 天内，她的 Erdheim-Chester 病相关症状迅速缓解。随后，她的骨痛缓解，尿崩症有所改善。此外，扁平苔藓相关的瘙痒迅速停止，大多数皮肤病变有所改善。
我们的经验证实了阿那白滞素治疗 Erdheim-Chester 病的疗效。她的扁平苔藓在使用阿那白滞素后同时得到改善，这表明该药物在这种疾病中值得进一步研究。
