No abstract

We report the case of a 62-year-old woman with Gottron's acrogeria and peliosis hepatis. Gottron's acrogeria is a rare congenital disease characterized by atrophic acral skin with mottled hyperpigmentation and an aged appearance. It is mostly caused by a mutation in COL3A1 gene that leads to defective production of type III collagen. These abnormalities in collagen produce fragility in different organs such as blood vessels, causing multiple vascular disorders, which would include peliosis hepatis. Peliosis hepatis is a pathological condition with multiple blood-fi lled lacunar lesions within the hepatic parenchyma. It is induced by several mechanisms, from chronic debilitating conditions to infections or immunocompromised status, standing out long-term oral contraceptive therapies, which our patient had taken for fi fteen years. Peliosis hepatis lesions tend to regress after cessation of estrogen treatment, but our patient developed the vascular disorder eighteen years after ending the therapy, with no evidence of peliosis in previous radiological tests. We suggest that peliosis is related to collagen defects caused by Gottron's acrogeria. No previously known associations between these entities were found despite exhaustive investigations.