Introduction: Pemphigus vulgaris is a serious autoimmune bullous disease, that may be difficult to control. Although corticosteroids have dramatically improved the outcome of the disease, this treatment may be complicated by unresponsiveness or serious side-effects. We report the case of a patient with pemphigus vulgaris refractory to corticosteroids who responded rapidly to the addition of high-dose intravenous immunoglobulins.
Case report: A 38-year-old man presented with a 1-month history of widespread bullous lesions of the skin and oral mucosa. The diagnosis of pemphigus vulgaris was made on the results of histology and direct immunofluorescence of perilesional skin. Systemic corticosteroids were initially started, but cutaneous and mucosal lesions poorly responded after 6 weeks. Mensual cycled of intravenous immunoglobulins were then begun and led to a complete disappearance of the lesions after three cycles. Four courses of high-dose intravenous were administered, that allowed to reduce doses of steroids. The patient was in complete remission without treatment after a two-year follow-up.
Discussion: Pulse therapy with high-dose intravenous immunoglobulins has been occasionally used for the treatment of pemphigus vulgaris, especially in an attempt to reduce side-effects of immunosuppressive agents or when these therapies are ineffective. We report an additional case, suggesting in addition of recent data of literature, that immunoglobulins may be useful as an alternative treatment in pemphigus vulgaris.