SINDROME DE EHLERS-DANLOS

Abstract

A 21-yr-old male patient is presented, in whom clinical and histopathological characteristics were typical of Ehlers-Danlos syndrome (cutaneous and articular hyperelasticity and cutaneous fragility with the presence of atrophic scars). Investigation into the family history indicated that other relatives had the same disease; they had died during their childhood due to internal hemorrhage. The patient presented did not have significant hemorrhages at any time. The elastic fibers in the dermal papilla are reduced, giving rise to the appearance of being increased in the middle and deep dermis.