Pediatric Pemphigus Vulgaris: Durable Treatment Responses Achieved with Prednisone and Mycophenolate Mofetil (MMF)

Authors

  • Andrea Baratta D.O.,

    1. Department of Dermatology, St. John’s Episcopal Hospital, Far Rockaway, New York
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  • Diana Camarillo M.D.,

    1. Department of Dermatology, University of California at San Francisco, San Francisco, California
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  • Christine Papa D.O.,

    1. Private Practice, Voorhees, New Jersey
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  • James R. Treat M.D.,

    1. Section of Dermatology, Division of General Pediatrics, Department of Pediatrics, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
    2. Department of Dermatology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania
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  • Aimee S. Payne M.D., Ph.D.,

    1. Department of Dermatology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania
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  • Suzanne S. Rozenber D.O.,

    1. Department of Dermatology, St. John’s Episcopal Hospital, Far Rockaway, New York
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  • Albert C. Yan M.D.

    1. Section of Dermatology, Division of General Pediatrics, Department of Pediatrics, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
    2. Department of Dermatology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania
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Address correspondence to Albert C. Yan, M.D., e-mail: mdyana@email.chop.edu.

Abstract

Abstract:  Pemphigus vulgaris (PV) is a chronic autoimmune blistering disease of the skin and mucous membranes. Most cases occur in adults; cases in children are rare. This report describes the clinical presentations and treatment responses of three children with PV, as confirmed according to histology and indirect immunofluorescence studies. In all three cases, oral prednisone used in conjunction with mycophenolate mofetil (MMF) resulted in complete clinical remission, during which all pharmacotherapy was successfully discontinued. Resolution of the skin and mucosal blistering tended to occur quickly with prednisone, and after initiation of treatment with MMF, discontinuation of all pharmacotherapy was achieved within a range of 10 to 30 months in the three patients. One patient experienced a recurrence of genital lesions 19 months after discontinuation of therapy, but the condition remitted within 2 weeks with topical corticosteroid therapy. At the time of this report, the duration of complete remission ranged from 6 to 19 months. In summary, combination therapy with prednisone and MMF for pediatric PV appears to be a safe and effective approach that is associated with durable remission.

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