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Bullous pemphigoid associated with renal transplant rejection

Bullous pemphigoid (BP) is a rare disease in childhood, characterized by subepidermal bullae and antibodies against the 180-kDa BP antigen. We report a male patient on renal dialysis who presented with BP after rejection of his donor kidney. The patient was a 13-year-old boy, the fourth child of consanguineous parents, who had multiple congenital problems including cleft lip and palate, hypopituitarism and small genitalia. At the age of 9 years, a diagnosis of chronic renal failure due to congenital renal dysplasia was made. Almost immediately, the patient required haemodialysis treatment. He was transplanted with an unrelated donor kidney, and initially given immunosuppression with tacrolimus, mycophenolate mofetil and prednisolone. After approximately 26 months, because of severe rejection, tacrolimus was switched to sirolimus. After recurrent acute rejection and severe allograft nephropathy, the transplanted organ failed approximately 6 months later. The patient was started on peritoneal dialysis, and all renal immunosuppression was stopped after 3 months. 2 months following cessation of immunosuppression, the patient presented with multiple tense bullae over the trunk and limbs (Fig. 1). In addition, there were signs of skin fragility where dressings had been removed. Histological examination of a skin biopsy confirmed a subepidermal blister containing fibrin and scanty eosinophils. Using direct immunofluorescence, linear C3 was seen at the dermoepidermal junction. Indirect immunofluorescence on salt-split skin found IgG antibasement membrane zone antibodies binding to the epidermal surface (Fig. 2). IgG antibodies to the NC16A domain of BP180 were detected by ELISA at a titre of 133.62 (positive > 15). ELISA for BP230 was negative. Immunoblotting using epidermal extracts did not reveal any additional antigens. CP D