A Case of Unexplained Cutaneous Lesions, Cholestatic Hepatitis, and Noncirrhotic Portal Hypertension in a Female Patient

• Autor(a) principal: Carvão, Joana
• Data de Publicação: 2020
• Outros Autores: Pereira, Vítor Magno, Jasmins, Luís
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: http://hdl.handle.net/10400.26/34288
• topic: cutaneous lesions; epatitis; non cirrhotic portal hypertension(GRAIL); Madeira Island; Portugal

Question: A 57-year old woman with a previous history of contrast-related anaphylaxis was referred to the gastroenterology department with worsening liver function tests. She developed recurrent abdominal pain, diarrhea, significant weight loss, pruriginous generalized skin rash, and increased abdominal volume over 1 year. Physical examination revealed generalized small reddish monomorphic macules on the trunk and thighs (Figure A), hepatosplenomegaly, peripheral lymphadenopathy, and ascites. Blood tests showed normocytic normochromic anemia (hemoglobin 9.4 g/dL), monocytosis (1.3 103/mL), thrombocytopenia (97 103/mL), slight international normalized ratio prolongation, and a marked elevation of cholestasis parameters without jaundice (aspartate aminotransferase, 60 U/L; alanine aminotransferase, 56 U/L; gamma-glutamyl transferase, 324 U/L; and alkaline phosphatase, 1277 U/L). Abdominal Doppler ultrasound examination and a computed tomography scan revealed homogeneous hepatosplenomegaly (without signs of chronic liver disease), signs of portal hypertension without thrombosis of the splenoportal axis, moderate ascites, and intra-abdominal, axillary, and inguinal lymphadenopathies. Upper endoscopy showedesophageal varices and portal hypertensive gastropathy.Laboratory studies for chronic hepatopathy, infectious agents, and angiotensin-converting enzyme were negative. Bone marrow biopsy revealed granulomatous myelitis, and both lymphadenopathy and skin biopsies showed increased mononuclear cells.