Mariko Seishima

poster at ISFA 2019 pag 57

Generalized pustular psoriasis (GPP) is a rare inflammatory skin disorder characterized by a fever, edema, and generalized erythema with neutrophilic pustules. It sometimes occurs in the course of psoriasis vulgaris, or develops suddenly without any history of psoriasis. Mutations of the IL36RN (deficiency of interleukin thirty-six receptor antagonist: DITRA), CARD14 and AP1S3 genes underlie monogenic auto-inflammatory disorders causing GPP. GPP patients are usually treated with oral administration of etretinate, cyclosporine, and metrexate, biologics including TNF α inhibitors, antibodies to IL-17, IL-17 receptor, and IL-23 p19, and granulocyte and monocyte adsorption apheresis (GMA). Cyclosporine, TNF α inhibitors, and GMA are used for GPP in pediatric, pregnant, or lactating patients. GMA is an extracorporeal apheresis that removes activated granulocytes and monocytes using a column packed with cellulose acetate beads. Multicenter study was performed to access efficacy of selectively depleting the myeloid lineage leukocytes in GPP patients. Fifteen patients with persistent moderate to severe GPP despite conventional therapy were included. Based on the GPP severity scores relative to entry, the overall scores improved, and the area of erythroderma, pustules, and edema decreased. Likewise, Dermatology Life Quality Index (DLQI) improved, reflecting better daily function and quality of life. Twelve out of 14 patients were judged as responders (85.7%), and 10 out of 12 patients maintained the clinical response for10 weeks after the last GMA session without any change in medication. Thus, GMA is estimated to be safe and effective, suggesting a major role of granulocytes/ monocytes in the immunepathogenesis of GPP. Recent study showed that GMA was effective for 100% of DITRA patients and for 64.7% of the patients with IL36RN mutation-negative GPP. Thus, GMA is effective therapy for both DITRA and non-DITRA GPP patients. GMA may be a useful therapy for all GPP patients

http://www.atalacia.com/isfa/data/abstract.pdf

Hideki Fujita ¹, Tadashi Terui ¹, Koremasa Hayama ¹, Masashi Akiyama ², Shigaku Ikeda ³, Tomotaka Mabuchi ⁴, Akira Ozawa ⁴, Takuro Kanekura ⁵, Michiko Kurosawa ⁶, Mayumi Komine ⁷, Kimiko Nakajima ⁸, Shigetoshi Sano ⁸, Osamu Nemoto ⁹, Masahiko Muto ¹⁰, Yasutomo Imai ¹¹, Kiyofumi Yamanishi ¹¹, Yumi Aoyama ¹², Keiji Iwatsuki ¹³, J Dermatol . 2018 Nov;45(11):1235-1270. 

 The aim of the guidelines was to provide current information to aid in the treatment of patients with GPP in Japan. Its contents include the diagnostic and severity classification criteria for GPP, its pathogenesis, and recommendations for the treatment of GPP.

https://pubmed.ncbi.nlm.nih.gov/30230572/

Ryoko SHUKUYA,Toshio HASEGAWA,Yusuke NIWA,Keiko OKUMA,Shigaku IKEDA, Journal of dermatology First published: 18 October 2011

In both patients, GCAP resulted in an immediate improvement in skin lesions and fever reduction, without any adverse effects. We suggest that GCAP is an effective therapy for refractory GPP.

https://onlinelibrary.wiley.com/doi/10.1111/j.1346-8138.2011.01279.x