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

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.

Rituximab therapy in pediatric pemphigus patients: A retrospective analysis of five Turkish patients and review of the literature

“…There is no epidemiologic research on the incidence of pediatric pemphigus; however, it is suggested that 1.4%‐2.9% of all PV cases are pediatric cases . It affects both sexes equally, and the mean age of onset is 12 years . Pediatric pemphigus affected approximately 3% in two case series of pemphigus in Turkey .…”

Mycophenolate mofetil in the treatment of childhood pemphigus vulgaris

“…Only 46 cases of childhood PV (onset age range, 1.5-12 years), including the present case, have been reported in the literature. Mycophenolate mofetil has been used successfully by Baratta et al 6 , obtaining durable remission. It should be started at a low dosage (500 mg/day), and then increased gradually.…”

“…In conclusion, based on our and Terashita's observations, 1 and other recent studies. 6,8 Mycophenolate mofetil can be considered as an appropriate treatment option for pediatric patients with BD, PV, and a range of autoimmune diseases.…”

Pénfigo vulgar de la infancia, a propósito de un caso

“…Los más utilizados en pediatría son la azatioprina (2-4 mg/kg/día, hasta 100-300 mg al día) 7 y el mofetilmicofenolato (2-3 g/día). 5,8 Otras drogas descritas son la ciclofosfamida por vía oral (1-3 mg/kg/día), la ciclosporina (5 mg/kg/día), la dapsona (1,5 mg/kg/día) y el metotrexate (10-20 mg/semana). 4,5,7 Para los casos de pénfigo grave que no responden a la combinación de corticoides e inmunosupresores, está descrito el uso de plasmaféresis o IgG endovenosa.…”