title: Management of pemphigus in pediatric patients.
abstract: 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.
authors: Center for Blistering Diseases, Boston, MA, USA.
authors: H
authors: H
authors: Gürcan
authors: D
authors: D
authors: Mabrouk
authors: A
authors: A
authors: Razzaque Ahmed
mesh_terms: D000305:Adrenal Cortex Hormones / Q000009:adverse effects / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D002648:Child; D004359:Drug Therapy, Combination; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010392:Pemphigus / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology / Q000276:immunology; D017410:Practice Guidelines as Topic; D011241:Prednisone / Q000009:adverse effects / Q000627:therapeutic use*; D011379:Prognosis; D000069283:Rituximab; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology
chemical_list: D000305:Adrenal Cortex Hormones; D058846:Antibodies, Monoclonal, Murine-Derived; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D011241:Prednisone