title: Juvenile generalized pustular psoriasis with IL36RN mutation treated with short-term infliximab.
abstract: A 8-year-old Chinese boy with generalized pustular psoriasis (GPP) refractory to cyclosporine and methylprednisolone was treated successfully with two infusions of infliximab 3.3 mg/kg. He remained in remission for 21 months. Direct sequencing of IL36RN gene showed a homozygous mutation, c.115 + 6T>C. Juvenile GPP is a rare severe form of psoriasis occasionally associated with life-threatening complications. Like acitretin, cyclosporine and methotrexate, infliximab has been reported to be effective for juvenile GPP in case reports. However, there is a lack of data in the optimal treatment course of infliximab for juvenile GPP. Prolonged administration of these medications may cause toxic or fatal complications. We suggest that short-term infliximab regimen should be recommended as a choice for acute juvenile GPP refractory to traditional systemic therapies. WBC count and CRP are sensitive parameters to reflect the disease activity and evaluate the effectiveness of treatment. Monitoring CD4 T lymphocyte count, preventing and correcting CD4 lymphocytopenia are important in the treatment course of juvenile GPP.
authors.affiliation: Department of Dermatology, No. 1 Hospital, China Medical University, 155 North Nanjing Street, Shenyang, 110001, China.
authors.forename: Junwei
authors.initials: J
authors.lastname: Pan
authors.affiliation: Department of Dermatology, No. 1 Hospital, China Medical University, 155 North Nanjing Street, Shenyang, 110001, China.
authors.forename: Li
authors.initials: L
authors.lastname: Qiu
authors.affiliation: Department of Dermatology, No. 1 Hospital, China Medical University, 155 North Nanjing Street, Shenyang, 110001, China.
authors.forename: Ting
authors.initials: T
authors.lastname: Xiao
authors.affiliation: Department of Dermatology, No. 1 Hospital, China Medical University, 155 North Nanjing Street, Shenyang, 110001, China.
authors.forename: Hong-Duo
authors.initials: HD
authors.lastname: Chen
mesh_terms: D002648:Child; D004252:DNA Mutational Analysis; D003879:Dermatologic Agents / Q000008:administration & dosage*; D004334:Drug Administration Schedule; D020022:Genetic Predisposition to Disease; D006720:Homozygote; D006801:Humans; D000069285:Infliximab / Q000008:administration & dosage*; D007262:Infusions, Intravenous; D007378:Interleukins / Q000235:genetics*; D008297:Male; D009154:Mutation; D010641:Phenotype; D011565:Psoriasis / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D012074:Remission Induction; D012867:Skin / Q000187:drug effects* / Q000473:pathology; D012872:Skin Diseases, Vesiculobullous / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D003879:Dermatologic Agents; C122187:IL36RN protein, human; D007378:Interleukins; D000069285:Infliximab
keywords: IL36RN; generalized pustular psoriasis; infliximab; juvenile; mutation