Kavehmanesh Zohreh, Beiraghdar Fatemeh, Saburi Amin, Hajihashemi Ali, Amirsalari Susan, Movahed Morteza
Research Center for Gastroenterology and Liver Diseases, Baqiyatallah University of Medical Sciences, Tehran, IR Iran.
Hepat Mon. 2012 Jan;12(1):42-5. Epub 2012 Jan 20.
Autoimmune hepatitis (AIH) is a form of chronic hepatitis with unclear causative factors and is characterized by immunological and auto-immunological manifestations. Several extrahepatic manifestations, such as other autoimmune disorders, are associated with AIH. AIH with dermatological conditions as the initial manifestation is rare. We report the case of AIH in which erythema nodosum (EN) was the first manifestation.
An 8-year-old girl with several persistent dermatological lesions was referred to our hospital several months ago. Her skin had nodular, painful, dry, and erythematous lesions, predominantly on the extensor areas of both the legs, with some erythematous patches on her face. Physical examination revealed that she had hepatosplenomegaly as well. Skin biopsy indicated EN. The results of the laboratory tests showed increased levels of several liver enzymes. The patient's International Autoimmune Hepatitis Group (IAIHG) score was a definite indicator of AIH. The results of liver biopsy indicated AIH. Other causes of EN and abnormal liver function were ruled out. The only obvious cause of skin lesions was chronic inflammation due to an autoimmune response. The patient was treated for AIH, and her skin lesions along with other signs and symptoms resolved.
AIH can present with protean clinical manifestations, and is thus associated with the risk of delayed diagnosis. Dermatological manifestations, including EN, could indicate a serious disease, and further investigation might be required. AIH should be considered as the possible diagnosis in such cases.
自身免疫性肝炎(AIH)是一种病因不明的慢性肝炎,其特征为免疫和自身免疫表现。几种肝外表现,如其他自身免疫性疾病,与AIH相关。以皮肤病学状况为初始表现的AIH较为罕见。我们报告一例以结节性红斑(EN)为首发表现的AIH病例。
一名8岁女童数月前因多处持续性皮肤病变转诊至我院。她的皮肤有结节状、疼痛、干燥和红斑性病变,主要分布在双腿伸侧,面部有一些红斑。体格检查发现她也有肝脾肿大。皮肤活检显示为EN。实验室检查结果显示几种肝酶水平升高。患者的国际自身免疫性肝炎小组(IAIHG)评分是AIH的明确指标。肝活检结果显示为AIH。排除了EN和肝功能异常的其他原因。皮肤病变的唯一明显原因是自身免疫反应引起的慢性炎症。该患者接受了AIH治疗,其皮肤病变以及其他体征和症状均得到缓解。
AIH可表现出多种临床表现,因此存在诊断延迟的风险。包括EN在内的皮肤病学表现可能提示严重疾病,可能需要进一步检查。在此类病例中应考虑AIH作为可能的诊断。