Pemphigus vulgaris in a 14 year old girl

Summary A 14 years old with severe pemphigus vulgaris is reported. She received treatment with oral prednisone but new lesions developed and the 90% of the body surface was affected. Infusion of fresh frozen plasma was started and high dosis of oral prednisone was given for five days without improvement. On the sixth day, an intravenous pulse dose with methylprednisolone for five days was administered and followed by oral prednisone and oral azathioprine.With this treatment modality, there was no formation of new blisters, however, the patient presented infectious complications with pneumonia and septicemia which resolved with intravenous antibiotics.The patient was discharge from hospital without active lesions. The dermatosis was difficult to control and failed to tolerate tapering of oral prednisone. She presented Cushing syndrome as a complication of steroid therapy and oral cyclosporine A was initiated with oral prednisone. Pemphigus vulgaris is rare in children and it needs to be considerate within the differential diagnosis of bullous disease of childhood. There are no reported randomized control trials in the literature to provide evidence for the best therapeutic approach. Pemphigus vulgaris in childhood has been reported as case series and there are no well established standard recommendations for treatment.