The Hepato-Pulmonary-Cutaneous Syndrome: Description of a Case and Suggestion of a Unifying Hypothesis

• Main Authors: Enrico Capodicasa, Federica De Bellis, Christopher Muscat
• Format: Article
• Language: English
• Published: Karger Publishers 2010-08-01
• Series: Case Reports in Gastroenterology
• Subjects: Hypoxemia; Intrapulmonary vascular dilation; Arterovenous shunt; Platypnea-orthodeoxia; Cutaneous spider nevi; Telangiectasia; Digital clubbing; Palmar erythema
• Online Access: http://www.karger.com/Article/FullText/319502
• ISSN: 1662-0631

We report a 54-year-old patient with the association of hepatic dysfunction with cyanosis, severe hypoxemia, platypnea-orthodeoxia, diffuse cutaneous spider nevi, telangiectasia, palmar erythema, digital clubbing and findings of marked intrapulmonary vascular dilation and arterovenous shunt. The diagnosis of hepato-pulmonary-cutaneous syndrome, a term we think more appropriate and inclusive than that of hepato-pulmonary syndrome for this clinicopathological picture, is proposed. The putative underlying mechanism for these connected pulmonary and extrapulmonary syndromic features is discussed.