Multiple autoimmune syndrome in a patient with pemphigus vulgaris: a new combination

The occurrence in the same patient of three or more autoimmune diseases defines multiple autoimmune syndrome (MAS). Various autoimmune dermatoses, especially pemphigus, have been described in association with systemic autoimmune diseases as component of MAS.

The aim of our study is to describe MAS with a new combination in a 55-year-old woman having a pemphigus vulgaris, associated with three other autoimmune diseases.

A 55-year-old woman presented with multiple oral erosions of one year and blistering over the trunk and limbs of 15 days. Diagnosis of pemphigus vulgaris was confirmed by histology and direct immunofluorescence on cutaneous biopsy. The patient was started on oral prednisone of 1.25 mg/Kg/day with which the lesions subsided gradually. On day 32 of prednisone, she developed jaundice and purpura. Biology showed an exacerbation of a previous anaemia with thrombocytopenia and elevation of serum aminotransferase, consisting in the diagnosis of autoimmune haemolytic anaemia, autoimmune thrombocytopenia and autoimmune hepatitis. After 40 days of oral steroids (1.25 mg/Kg/day), cutaneous lesions, purpura and jaundice had completely resolved. Oral steroids were progressively diminished. The patient was seen after a two-year follow-up period without any skin lesions. Anaemia had improved and there was normal serum aminotransferase.

Our patient presented a combination of four autoimmune diseases, i.e. pemphigus, autoimmune haemolytic anaemia, autoimmune thrombocytopenia and autoimmune hepatitis consisting in the diagnosis of type 11 multiple autoimmune syndrome.