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In 1983, a female patient born in 1963 presented with symptoms of ulcerative colitis and typical clinical and histological signs of primary sclerosing cholangitis (PSC). At this time only pANCA were positive while other marker antibodies for autoimmune liver disorders could not be detected. In summer 1987 the clinical picture changed and was replaced by laboratory and histological signs typical of autoimmune hepatitis (AIH). Thus, IgG levels increased considerably and cholestatic enzymes became normal. For the first time, anti-liver-pancreas antibodies (LP), a diagnostic marker for AIH type III could be detected. In the following years several relapses occurred also induced by repeated discontinuation of immunosuppressive therapy. Symptoms of colitis persisted but signs of cholestasis remained absent for the following ten years. In 1997, colitis exacerbated again and colectomy had to be performed together with liver transplantation. Surprisingly, histology of the explanted liver now showed the typical features of PSC stage III/IV while the significant criteria for AIH were now lacking. Thus, progression to cirrhosis was, probably, mainly induced by the biliary destructive and fibrotic process although biochemical and serological data were clearly indicative of an autoimmune, i.e. AIH-related manifestation. [ABSTRACT FROM AUTHOR]

Seven cases of tricholemmal carcinoma (TLC), a rarely recognized cutaneous adnexal neoplasm of external hair, sheath origin, are described. Most occurred on sun-exposed skin; five involved the head and neck, one the right leg and one the right thigh. TLC had a generally short history and all were treated by local excision. The lesions had an exophytic (3 cases) or polypoid (4 cases) gross appearance. Histologically, TLC exhibited a sharply circumscribed, lobular epithelial proliferation in continuity with the epidermis. A cytologic hallmark of these tumors was the presence of large cells with PAS-reactive, diastase-sensitive, clear or pale eosinophilic cytoplasm. High mitotic rate was a constant feature. Four tumors were infiltrative, with pushing borders, whereas three were intraepithelial. One case showed acantholysis. Immunocytochemistry revealed positivity for prekeratin and negativity for CEA and EMA, supporting the trichogenic origin of these tumors. Ultrastructural examination gave clear indication of epithelial origin for the cells but did not verify hair follicular differentiation. Despite locally aggressive growth, the clinical course of TLC appeared indolent. Moreover, there are no cases with metastases reported in the literature. [ABSTRACT FROM AUTHOR]