Chronic active hepatitis associated with vitiligo, nail dystrophy, alopecia and a new variant of LKM antibodies.
In this report we describe the case of a 7-year-old boy, suffering from autoimmune-type chronic active hepatitis (AI-CAH) associated with vitiligo, nail dystrophy, alopecia areata and a variant of liver kidney microsomal (LKM) autoantibodies. This patient's antibodies are different from LKM-1 which are directed against cytochrome P450 db1. They react predominantly with perivenous hepatocytes in contrast to LKM-1 antibodies which homogeneously stain the whole liver lobule in immunofluorescence. In Western blot analysis this LKM variant reacts with a liver microsomal protein of approx. 50 kDa, but not with recombinant LKM-1 (cytochrome P450 db1) antigen. Immunosuppressive treatment led to a normalization of liver histology after 1 year and a significant improvement of vitiligo and alopecia areata. In summary, a case of autoimmune-type chronic active hepatitis is presented which is associated with a new variant of LKM antibodies reacting with a 50 kDa microsomal protein different from cytochrome P450 db1. Furthermore, this patient suffers from extrahepatic syndromes (alopecia, nail dystrophy) that have not been described previously in LKM antibody-positive chronic active hepatitis.
G. v. Peyer'sches Kinderspital der Stadt Wien, Federal Republic of Germany.
M
M
Sacher
P
P
Blümel
H
H
Thaler
M
M
Manns
D000505:Alopecia / Q000150:complications* / Q000473:pathology; D001323:Autoantibodies / Q000235:genetics*; D001327:Autoimmune Diseases / Q000150:complications* / Q000276:immunology / Q000473:pathology; D015153:Blotting, Western; D002648:Child; D005455:Fluorescent Antibody Technique; D014644:Genetic Variation; D006521:Hepatitis, Chronic / Q000150:complications* / Q000276:immunology / Q000473:pathology; D006801:Humans; D008297:Male; D009260:Nail Diseases / Q000150:complications* / Q000473:pathology; D014820:Vitiligo / Q000150:complications* / Q000473:pathology
D001323:Autoantibodies; C060729:anti-liver kidney microsome antibody