Narbutt Joanna, Lesiak Aleksandra, Kwiecień Aneta, Sysa-Jedrzejowska Anna, Kuydowicz Jan
Katedra i Klinika Dermatologii i Wenerologii, Uniwersytetu Medycznego w Lodzi.
Przegl Lek. 2005;62(5):314-6.
We present a case of a 31-year old man with a 17 year history of Raynaud's symptom and contractures of the both hands' interdigitalis joints, progressive skin sclerosis of the arms, forearms and hands, telangiectases on the face skin. Because of the painful leg ulcers on the outer surface of both shins and on the dorsal part of the right foot (they appeared almost 10 years ago) the patient finally was admitted to our Department. Physical examination revealed hepatosplenomegaly, in laboratory tests leukopenia, mild thrombocytopenia, elevated ESR, elevated serum levels of IgG, IgM, IgA, creatine kinase level and hypergammaglobulinaemia were found. Neither anti-HBV or anti-HCV or antiphospholipid antibodies were present. Immunofluorescence test (HEP-2 cells) revealed the presence of antinuclear antibodies in a titer of 1:2560 (homogenous and partially speckled pattern) and by immuno-diffusion-anti-Ro antibodies. Hepatosplenomegaly was diagnosed by USG examination. Endocrinological, neurological, surgical and pulmonological examinations gave no abnormalities. Liver biopsy showed features of chronic inflammation and autoimmune hepatitis was diagnosed.
the presence of scleroedematous lesions of the skin accompanying chronic hepatic inflammation enabled us to establish the diagnosis of scleroderma-like lesions in the course of long lasting mild autoimmune hepatitis.
我们报告一例31岁男性病例，该患者有17年雷诺氏症状病史，双手指间关节挛缩，双臂、前臂和手部皮肤进行性硬化，面部皮肤有毛细血管扩张。由于双小腿外表面和右脚背出现疼痛性腿部溃疡（几乎10年前出现），患者最终入住我院。体格检查发现肝脾肿大，实验室检查显示白细胞减少、轻度血小板减少、血沉升高、血清IgG、IgM、IgA水平升高、肌酸激酶水平升高以及高球蛋白血症。未发现抗乙肝病毒、抗丙肝病毒或抗磷脂抗体。免疫荧光试验（HEP - 2细胞）显示抗核抗体滴度为1:2560（均质和部分斑点状模式），免疫扩散法检测到抗Ro抗体。通过超声检查诊断为肝脾肿大。内分泌、神经、外科和肺部检查均未发现异常。肝活检显示慢性炎症特征，诊断为自身免疫性肝炎。
皮肤硬化性水肿病变伴有慢性肝脏炎症，使我们能够诊断出长期轻度自身免疫性肝炎过程中的硬皮病样病变。
