1/8. Pustular dermatosis of the scalp associated with autoimmune diseases.A 36-year-old woman visited our hospital with a five month history of persistent pustulation, crusting, and alopecia on the vertex of the scalp. No pathological organisms were isolated from the lesions. Histological examination revealed non-specific changes of chronic inflammation with destroyed follicles. Antibiotic therapy produced no response, but steroid therapy was effective. From these observations, a diagnosis of erosive pustular dermatosis of the scalp (EPDS), as described by Pye et al., was made. The patient also had Hashimoto's thyroiditis, autoimmune hepatitis, and Takayasu's aortitis. The laboratory studies revealed an increased erythrocyte sedimentation rate, c-reactive protein 3 , hypergammaglobulinemia, and various auto-antibodies, suggesting the possibility of a pathogenesis common to both this dermatosis and the autoimmune diseases.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
2/8. Fulminant Wilsonian hepatitis: difficulties in diagnosis and treatment.We present herein the case of a 16-yr-old girl with fulminant Wilsonian hepatitis. Complications included renal tubular damage, hemolysis, marked hypocomplementemia and hypergammaglobulinemia, spontaneous bacterial peritonitis, and candida sepsis. The difficulties in diagnosis and treatment are discussed.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
3/8. Acute liver biopsy lesions in early autoimmune ("lupoid") chronic active hepatitis.We studied two female patients with autoimmune ("lupoid") chronic active hepatitis whose liver biopsies at initial presentation showed the unusual features of an acute hepatitis. Centrilobular hepatocyte swelling and multinucleation, acidophilic degeneration, cholestasis, mild fatty change and bile duct damage in one case resembled lesions of non-A, non-B hepatitis. Lobular and portal infiltrates of plasma cells with piecemeal necrosis suggested transition to chronicity as well as an autoimmune component. This was additionally supported by the presence of hypergammaglobulinemia and auto-antibodies in both patients. We conclude that liver biopsy features in the acute presentation of lupoid hepatitis may be difficult to distinguish from those seen in acute hepatitis due to virus or drugs.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
4/8. Chronic liver disease manifesting as Waldenstrom's macroglobulinemia.A 59-year-old woman was initially seen with anemia, purpura, gastrointestinal tract and vaginal bleeding, pronounced hyperglobulinemia, and an increased serum viscosity, all suggestive of Waldenstrom's macroglobulinemia. Subsequent investigation, however, indicated that these abnormalities were more likely due to chronic active hepatitis with cirrhosis. The striking similarity of this patient's symptoms to those of Waldenstrom's macroglobulinemia and the pathophysiologic characteristics of polyclonal hypergammaglobulinemia in chronic liver disease are discussed.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
5/8. Autoimmune hepatitis following hepatitis a virus infection.A 7-year-old patient is reported who suffered from fatigue and jaundice due to chronic hepatitis. He had acquired hepatitis a virus infection in his community and communicated the disease to his German family 4 weeks later. While the other family members recovered from acute viral hepatitis A, the patient presented 10 weeks after the onset of hyperbilirubinemia (12 mg/dl) with the histology of chronic hepatitis, absence of markers for viral persistence, presence of autoantibodies against smooth muscle (1:320) and the asialoglycoprotein receptor (1:600), and marked hypergammaglobulinemia (3700 mg/dl), leading to the diagnosis of autoimmune hepatitis. The patient received immunosuppressive therapy, symptoms of liver disease disappeared, and autoantibodies cleared from circulation. The case is discussed in the context of a putative virus-induced autoimmune hepatitis in childhood. Autoimmune hepatitis may be induced by an external trigger. hepatitis a virus infection is one of probably several triggers that may induce autoimmune hepatitis in predisposed individuals.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
6/8. Hepatocellular carcinoma associated with lupoid hepatitis: a review of Japanese reports.A 66-year-old woman was diagnosed as having lupoid hepatitis due to the presence of hypergammaglobulinemia, lupus erythematosus cells, and positivity for antinuclear, anti-dna, and anti-smooth muscle antibodies. None of the serum hepatitis B markers were positive. Symptomatic relief was obtained by prednisolone administration. Five years after the diagnosis of lupoid hepatitis, hepatocellular carcinoma (HCC) was detected by ultrasonography and computed tomography, after which hepatectomy was performed. Although transcatheter arterial embolization was done on two occasions and repeat hepatectomy was performed twice for recurrent HCC, her liver function remained good with the prednisolone treatment. Antibody for hepatitis c virus has been negative since our first check in 1992. As of this writing, the patient has been alive and well 6 years and 2 months after the first hepatectomy. There have been no previous reports of 6-year survival after hepatectomy for HCC associated with lupoid hepatitis.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
7/8. hepatitis b virus carrier status linked to autoimmune hepatitis.We describe a hepatitis b virus carrier who satisfied the criteria of autoimmune hepatitis proposed by the International Autoimmune Hepatitis Group. A 43-year-old Japanese female showed human leukocyte antigen typing including DR4 in addition to hypergammaglobulinemia, presence of autoantibodies, and liver histology suggestive of autoimmune hepatitis. Moreover, the predominant presence of hepatitis B core antigen in nuclei rather than in cytoplasm of hepatocytes suggested less of a possibility of liver cell damage related to hepatitis b virus infection. She completely responded to immunosuppressive therapy and no clinical or biochemical relapse has been recognized to date.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
8/8. Simultaneously found transient hypothyroidism due to Hashimoto's thyroiditis, autoimmune hepatitis and isolated ACTH deficiency after cessation of glucocorticoid administration.We present a 42-year-old woman with concomitant transient hypothyroidism due to Hashimoto's thyroiditis, autoimmune hepatitis and isolated ACTH deficiency. Two months after ceasing prednisolone (5 mg/day) for uveitis, she was discovered incidentally to have liver dysfunction with hypergammaglobulinemia, later diagnosed as autoimmune hepatitis by histological examination of the biopsied liver. In addition, primary hypothyroidism due to Hashimoto's thyroiditis and secondary hypocortisolism due to isolated ACTH deficiency were revealed by endocrinological examination. Although not treated, her liver dysfunction and hypothyroid state recovered simultaneously, and the isolated ACTH deficiency was restored six months later. We concluded, after a needle-biopsy of the thyroid, that the transient hypothyroidism was due to Hashimoto's thyroiditis and the reversible ACTH deficiency was probably due to autoimmune hypophysitis. This case shows that cessation of steroid treatment may transiently exacerbate the polyglandular autoimmune syndrome.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |