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A Journal on Pediatrics, Neonatology, Adolescent Medicine,
Child and Adolescent Psychiatry

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Minerva Pediatrica 2011 August;63(4):279-91

language: English

Management of pemphigus in pediatric patients

Gürcan H. M., Mabrouk D., Razzaque Ahmed A.

Center for Blistering Diseases, Boston, MA, USA


The management of pemphigus in pediatric patients is divided into childhood (patient ≤12 years) pemphigus and juvenile (patients 13-18 years) pemphigus. In both groups the majority of patients have mucocutaneous disease. The mucose involved are oral, nasal, ocular, and anal. In both groups there is a high prevalence of genital involvement. Autoantibody titers can be detected in the sera of the majority of patients. The mainstay of therapy is oral corticosteroids. About half to two thirds of the patients develop systemic side effects. The most concerning is growth retardation present in 50% of the patients. Others include infection, obesity, psychological, and social distress. Immunosuppressive agents are used in many patients for their steroid-sparing effects. Surprisingly, Dapsone or sulphapyridine has not been used in more patients. The treatment lasts between two to three years. The prognosis in most cases reported was good. Intravenous immunoglobulin (IVIg) shows promise in early studies. Rituximab was effective in recalcitrant cases. There is no evidence in the literature suggesting that disease can continue into adult life or recur during adult life after a prolonged remission after childhood or juvenile disease. Even though it is quite rare, pemphigus should be considered in a pediatric patient presenting with blisters or erosions and excluded by a routine biopsy and direct immunofluorescence studies.

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