Juvenile Generalized Pustular Psoriasis: Management in Children—A Case Series

Juvenile Generalized Pustular Psoriasis (GPP) is an extremely rare and potentially life-threatening inflammatory disease. We report three cases of Juvenile GPP that presented with recurrent episodes of widespread painful erythema with sterile pustules accompanied by fever and malaise. None of the patients had a prior history of plaque-type psoriasis. There are no established guidelines for the management of pustular psoriasis in the pediatric age group. During our treatment, one patient responded well to a low dose of Cyclosporine alone without any recurrence following treatment. However, in the other two patients, an escalation of Cyclosporine dose (≥ 5 mg/kg) was required due to frequent relapses. Additionally, Acitretin 0.2-1 mg/kg was considered for maintenance therapy. However, the commercially available dose of Acitretin in our institution was soft capsules of 25 mg only. Hence, we administered Acitretin 25 mg to our patients on alternate days for maintenance therapy. Patients showed significant improvement in cutaneous lesions, and the frequency of relapses gradually declined. Despite the increasing recommendation of biologic agents, the high cost and unavailability of biologic agents restrict their use in clinical practice. Cyclosporine and Acitretin have remained the main therapeutic strategies in resource-limited settings.