Bullous pemphigoid after liver transplantation for liver failure.
Coomb's positive autoimmune hemolytic anemia with giant cell hepatitis (GCH) is a rare cause of liver failure and is usually associated with poor prognosis. A child with liver kidney microsomal (LKM) antibody positivity underwent successful liver transplantation for liver failure secondary to GCH with Coomb's positive hemolytic anemia. Autoimmune neutropenia developed ten months after transplant. Four months later, pemphigoid skin lesions developed. The diagnosis of bullous pemphigoid (BP) was made on the basis of skin biopsy, direct and indirect immunofluorescence test results. Treatment was with immunosuppressants - prednisone and azathioprine/rapamycin, with addition of dapsone when lesions persisted. This child is unique in that his liver function and hemolytic anemia appeared to normalize after liver transplant, but neutropenia and BP both thought to be autoimmune in etiology, developed more than a year post-transplant.
Kerkar
Nanda
N
Division of Pediatric Hepatology, Department of Pediatrics, Mount Sinai School of Medicine, New York, NY, USA. Nanda.Kerkar@msnyuhealth.org
Cohen
Steven
S
Dugan
Christina
C
Morotti
Raffaella A
RA
Phelps
Robert G
RG
Herold
Betsy
B
Shneider
Benjamin
B
Emre
Sukru
S
D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications / Q000503:physiopathology; D015726:Giant Cells / Q000473:pathology; D006505:Hepatitis / Q000150:complications / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D008099:Liver / Q000503:physiopathology; D017093:Liver Failure / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D016031:Liver Transplantation / Q000009:adverse effects*; D008297:Male; D010391:Pemphigoid, Bullous / Q000188:drug therapy / Q000209:etiology*; D011184:Postoperative Period; D020127:Recovery of Function
D007166:Immunosuppressive Agents