A Case of Old Age-Onset Generalized Pustular Psoriasis with a Deficiency of IL-36RN (DITRA) Treated by Granulocyte and Monocyte Apheresis.
A Case of Old Age-Onset Generalized Pustular Psoriasis with a Deficiency of IL-36RN (DITRA) Treated by Granulocyte and Monocyte Apheresis.
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DOI：
10.1159/000380876
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发表时间：
2015-01
影响因子：
0.9
通讯作者：
Yamanishi K
中科院分区：
文献类型：
--
作者：
Tominaga C;Yamamoto M;Imai Y;Yamanishi K
一位78岁的妇女，患有寻常型银屑病31年，因红皮病入院治疗。疑似中毒性皮疹，每日给予泼尼松龙30 mg治疗。然而，它是无效的，怀疑银屑病红皮病，环孢素150毫克，每天与泼尼松龙减量。环孢素剂量降至100 mg/天后2周，出现红皮病伴广泛全身性脓疱和发热。活检的组织学检查显示皮肤中的炎性浸润伴Kogoj海绵状脓疱，这与泛发性脓疱性银屑病（GPP）一致。她的脓疱改善与额外的etretinate 20毫克/天，但红皮病持续存在，她咨询了我们。每周一次的三次粒细胞和单核细胞单采术对她的病情有效，并降低了她的血清IL-6和IL-8水平。她有c基因的纯合突变。[28C>T]在IL 36 RN中，其导致p。[Arg10Ter].她是最古老的GPP报告的情况下，缺乏白细胞介素-36受体拮抗剂（DITRA），虽然GPP在DITRA已被建议通常发生在年轻的情况下，没有预先存在的寻常型银屑病。
A 78-year-old woman who had been suffering from psoriasis vulgaris for 31 years was admitted to hospital because of her erythroderma. A toxic eruption was suspected and she was treated with prednisolone 30 mg daily. However, it was ineffective and, suspecting psoriatic erythroderma, cyclosporine 150 mg daily was administered with tapering of the prednisolone. Two weeks after a dose reduction of cyclosporine to 100 mg/day, erythroderma with widespread generalized pustules and fever developed. Histology of a biopsy revealed inflammatory infiltrates in the skin with a spongiform pustule of Kogoj, which was consistent with generalized pustular psoriasis (GPP). Her pustules improved with additional etretinate 20 mg/day, but the erythroderma persisted and she consulted us. Three sessions of granulocyte and monocyte apheresis once weekly were effective for her condition and decreased her serum levels of IL-6 and IL-8. She had homozygous mutations of c.[28C>T] in IL36RN which cause p.[Arg10Ter]. She is the oldest reported case of GPP with a deficiency of interleukin-36 receptor antagonist (DITRA), although GPP in DITRA has been suggested to usually occur in younger cases with no pre-existing psoriasis vulgaris.
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