title: Granulocyte and monocyte apheresis can control juvenile generalized pustular psoriasis with mutation of IL36RN.
abstract: Patients with deficiency of interleukin-36 receptor antagonist (DITRA), due to mutation of IL36RN, exhibit psoriatic phenotypes, typically generalized pustular psoriasis (GPP). We report a paediatric patient with DITRA, whose cutaneous lesions varied from psoriasis vulgaris in infancy to annular pustular psoriasis with acute exacerbation to GPP at 13 years of age. Conventional systemic treatments for GPP, which include oral retinoids, ciclosporin and methotrexate, are controversial in paediatric cases, because of their adverse effects and uncertain long-term consequences. Granulocyte monocyte apheresis, a process associated with few adverse events, promptly controlled the GPP of our paediatric patient, and has potential as a suitable alternative treatment for paediatric patients with DITRA.
authors.affiliation: Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
authors.forename: Y
authors.identifier: 0000-0002-7921-8840
authors.initials: Y
authors.lastname: Koike
authors.affiliation: Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Okubo
authors.affiliation: Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Kiyohara
authors.affiliation: Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
authors.forename: R
authors.initials: R
authors.lastname: Fukuchi
authors.affiliation: Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Sato
authors.affiliation: Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Kuwatsuka
authors.affiliation: Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: T
authors.identifier: 0000-0001-5958-2875
authors.initials: T
authors.lastname: Takeichi
authors.affiliation: Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Akiyama
authors.affiliation: Department of Dermatology, Fujita Health University School of Medicine, Toyoake, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Sugiura
authors.affiliation: Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Utani
mesh_terms: D000293:Adolescent; D016238:Cytapheresis / Q000379:methods*; D006098:Granulocytes; D006801:Humans; D007378:Interleukins / Q000235:genetics*; D008297:Male; D009000:Monocytes; D009154:Mutation / Q000235:genetics; D011565:Psoriasis / Q000235:genetics / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: C122187:IL36RN protein, human; D007378:Interleukins