Nam A, Han S-M, Go D-M, Kim D-Y, Seo K-W, Youn H-Y
Department of Veterinary Internal Medicine, College of Veterinary Medicine, Seoul National University, Seoul, Korea.
Department of Veterinary Pathology, College of Veterinary Medicine, Seoul National University, Seoul, Korea.
J Vet Intern Med. 2017 Sep;31(5):1514-1519. doi: 10.1111/jvim.14798. Epub 2017 Aug 7.
Hepatocutaneous syndrome (HS) is an uncommon skin disorder that occurs in conjunction with liver disease and is diagnosed based on decreased plasma concentrations of amino acids and the histopathology of skin lesions. The survival period generally is <6 months. A 10-year-old castrated male Maltese dog was presented for evaluation of lethargy, polyuria, polydipsia, and skin lesions including alopecia, erythema, and crusts. Based on increased liver enzyme activity, low plasma amino acid concentrations, and findings from liver cytology and skin biopsy, the dog was diagnosed with HS. In addition to administration of antioxidants, hepatoprotective agents, and amino acids IV, allogenic adipose tissue-derived mesenchymal stem cells were infused 46 times over a 30-month period: 8 times directly into the liver parenchyma guided by ultrasonography and the remainder of the times into peripheral veins. After commencing stem cell therapy, the dog's hair re-grew and the skin lesions disappeared or became smaller. During ongoing management, the patient suddenly presented with anorexia and uncontrolled vomiting, and severe azotemia was observed. The dog died despite intensive care. On necropsy, severe liver fibrosis and superficial necrolytic dermatitis were observed. The dog survived for 32 months after diagnosis. A combination of amino acid and stem cell therapy may be beneficial for patients with HS.
肝皮肤综合征(HS)是一种与肝脏疾病相关的罕见皮肤疾病,其诊断基于血浆氨基酸浓度降低和皮肤病变的组织病理学检查。生存期一般小于6个月。一只10岁去势雄性马尔济斯犬因嗜睡、多尿、多饮以及包括脱毛、红斑和结痂在内的皮肤病变前来就诊。基于肝酶活性升高、血浆氨基酸浓度降低以及肝脏细胞学和皮肤活检结果,该犬被诊断为HS。除静脉注射抗氧化剂、保肝药物和氨基酸外,在30个月内共46次输注同种异体脂肪组织来源的间充质干细胞:8次在超声引导下直接注入肝实质,其余经外周静脉注入。开始干细胞治疗后,犬的毛发重新生长,皮肤病变消失或变小。在后续治疗过程中,该犬突然出现厌食和无法控制的呕吐,且观察到严重氮质血症。尽管进行了重症护理,该犬仍死亡。尸检时,观察到严重肝纤维化和浅表坏死性皮肤炎。该犬在诊断后存活了32个月。氨基酸和干细胞联合治疗可能对HS患者有益。