Hepatoportal sclerosis (HPS) is characterized by presinusoidal intrahepatic portal hypertension associated with splenomegaly and anemia in patients with non-cirrhotic liver. Liver biopsy is essential, especially to rule out other processes. Being a disease of unknown etiology, the majority of cases have been described in eastern countries. However, it may be an underdiagnosed disease in the West. Symptoms are related to portal hypertension and the clinical spectrum is wide, ranging from anemia with normal liver function tests to bleeding due to esophageal varices. Treatment is directed to the complications and the prognosis is better than in patients with cirrhosis. We report three cases of HPS presenting at different clinical stages and the findings of liver biopsies, the clinical outcomes and a review of scientific literature.
Scleroderma is a disease characterized by multiorgan fibrosis due to connective tissue proliferation and vasculitis of small vessels. Sclerodermia can be localized or systemic, and the latter can compromise skin in a limited or diffuse type. Digestive tract involvement is common in the systemic form (82%) and is often characterized by dysphagia and gastroesophageal reflux disease.
CASE REPORTA 50 year old male, with no relevant family history, who was diagnosed of subclinical hypothyroidism and early scleroderma has been follow-up by Rheumatology and Dermatology. Due to the finding of necrosis in his finger pads since the previous year examinations were initiated. Capillaroscopy was consistent, ANA were positive (1/640) and anticentromere antibody, RNP and Scl-70 were negative. Therefore, treatment was started with bosentan and aspirin.The patient was referred to the Emergency ward of our hospital because of melena and epistaxis. At admission, hemoglobin level was 8.5 mg/dL, with normal platelet count, normal coagulation and an ESR of 48 mm/h. Examination revealed multiple telangiectasias on his fingertips (Fig. 1), in oral mucosa and perioral region. Gastroscopy was performed which showed multiple small vascular appearance lesions (less than 5 mm) in esophageal, gastric and duodenal mucosa, with no signs of recent bleeding or clots (Fig. 2). In colonoscopy, multiple telangiectases were visualized from the anus to the cecum (Fig. 3). Neither melena nor decrease in hematocrit was presented during admission and he was discharged with supplemental oral iron and folic acid.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.