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.
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