Journal
DERMATOLOGIC THERAPY
Volume 29, Issue 3, Pages 164-167Publisher
WILEY
DOI: 10.1111/dth.12325
Keywords
generalized pustular psoriasis; IL36RN; infliximab; juvenile; mutation
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Funding
- National Natural Science Fund, China [81470142]
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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.
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