Juvenile generalized pustular psoriasis with IL36RN mutation treated with short‐term infliximab. Issue 3 (2nd December 2015)

• Record Type: Journal Article
• Authors: Pan, Junwei; Qiu, Li; Xiao, Ting; Chen, Hong‐Duo
• Is Part Of: Dermatologic therapy. Volume 29:Issue 3(2016)
• Page Start: 164
• Page End: 167
• Publication Date: 2015-12-02
• Subjects: generalized pustular psoriasis -- IL36RN -- infliximab -- juvenile -- mutation; Skin -- Diseases -- Periodicals; Dermatology -- Periodicals; 616.5
• DOI: 10.1111/dth.12325
• Languages: English
• ISSNs: 1396-0296

Abstract

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.