| Sumario: | Pseudoxanthoma elasticum is a multi-systemic, genetic disorder of the connective tissue characterized by progressive calcification and fragmentation of elastic fibers in skin, retina, gastrointestinal and cardiovascular systems. Skin changes generally manifest in adulthood and rarely in childhood. Upper gastrointestinal hemorrhage occurs in some cases and is frequently resistant to nonsurgical methods of treatment. As the prognosis of pseudoxanthoma elasticum is related to the involvement of extracutaneous organs, early recognition of the disease is important to take preventive measures and to decrease the morbidity. Herein, we presented a case of pseudoxanthoma elasticum with Mallory-Weiss syndrome, who had repetitive and massive upper gastrointestinal bleeding. To our knowledge, there is no reported case of pseudoxanthoma elasticum with Mallory-Weiss syndrome in the literature.
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