Le bolle che non ti aspetti: un raro caso di pemfigo volgare in età pediatrica

Abstract

A healthy 15-year-old boy in full well-being presents with multiple ulcerative lesions with an erosive appearance at the level of the oral cavity and the nasal mucosa. Two months after the onset, vesicular lesions also appear on the skin. As pemphigus vulgaris is suspected, a biopsy of the oral mucosa is performed which shows suprabasal bubbles, which confirms the diagnosis. Direct immuno-fluorescence tests show the presence of IgG against the epidermal substance and immunoserological tests detect anti-desmoglein I and III autoantibodies. Oral steroid therapy is prescribed, showing initial good symptom control. During the decalage, the lesions reappear, associated with significant difficulty in feeding and weight loss. After excluding paraneoplastic forms and other autoimmune pathologies, intravenous steroid therapy and azathioprine are started. At the follow-up after 3 months, a marked improvement of the lesions was observed, with the persistence of only a few skin vesicles. The patient has currently been on therapy for 10 months, with complete remission of the clinical picture. Pemphigus vulgaris is an autoimmune disease, rare in childhood, involving skin and mucous membranes, characterized by intraepidermal blisters with a flaccid roof. Treatment should be prompt and appropriate, using full dose corticosteroids. It is also recommended the concomitant initiation of immunosuppressant therapies to allow the steroid to be phased out. There are rare secondary forms, which must therefore be excluded at the time of diagnosis.