Interleukin-36–Receptor Antagonist Deficiency and Generalized Pustular Psoriasis

2011 ◽  
Vol 365 (7) ◽  
pp. 620-628 ◽  
Author(s):  
Slaheddine Marrakchi ◽  
Philippe Guigue ◽  
Blair R. Renshaw ◽  
Anne Puel ◽  
Xue-Yuan Pei ◽  
...  
Keyword(s):  
Receptor Antagonist ◽  
Pustular Psoriasis ◽  
Generalized Pustular Psoriasis

scholarly journals A Case of Old Age-Onset Generalized Pustular Psoriasis with a Deficiency of IL-36RN (DITRA) Treated by Granulocyte and Monocyte Apheresis

2015 ◽  
Vol 7 (1) ◽  
pp. 29-35 ◽  
Author(s):  
Chiharu Tominaga ◽  
Masaaki Yamamoto ◽  
Yasutomo Imai ◽  
Kiyofumi Yamanishi
Keyword(s):  
Receptor Antagonist ◽  
Dose Reduction ◽  
Old Age ◽  
Psoriasis Vulgaris ◽  
Serum Levels ◽  
Pustular Psoriasis ◽  
Generalized Pustular Psoriasis ◽  
Homozygous Mutations ◽  
Inflammatory Infiltrates

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.

scholarly journals The Majority of Generalized Pustular Psoriasis without Psoriasis Vulgaris Is Caused by Deficiency of Interleukin-36 Receptor Antagonist

2013 ◽  
Vol 133 (11) ◽  
pp. 2514-2521 ◽  
Author(s):  
Kazumitsu Sugiura ◽  
Akemi Takemoto ◽  
Michiya Yamaguchi ◽  
Hidetoshi Takahashi ◽  
Yukiko Shoda ◽  
...  
Keyword(s):  
Receptor Antagonist ◽  
Psoriasis Vulgaris ◽  
Pustular Psoriasis ◽  
Generalized Pustular Psoriasis

scholarly journals Association between mutation of interleukin 36 receptor antagonist and generalized pustular psoriasis

Medicine ◽  
2020 ◽  
Vol 99 (45) ◽  
pp. e23068
Author(s):  
Zhi-Jie Liu ◽  
Yu-Tong Tian ◽  
Bo-Yi Shi ◽  
Yin Zhou ◽  
Xue-Song Jia
Keyword(s):  
Receptor Antagonist ◽  
Pustular Psoriasis ◽  
Generalized Pustular Psoriasis
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