ABSTRACT

Elastosis perforans serpiginosa is a rare elastic tissue disorder characterized by keratotic papules, which are usually arranged in serpiginous, annular or arcuate patterns. It is usually observed in young adults. Associations with systemic disorders, such as pseudoxanthoma elasticum, Ehlers- Danlos syndrome, Marfan syndrome and osteogenesis imperfecta have been reported. Herein a case of generalized reactive elastosis perforans serpiginosa simultaneously diagnosed to have Ehlers-Danlos syndrome type IV with translucent skin, common atrophic scars, postinflammatory hyperpigmentation is presented because of the rare coexistence of these two entities.

Keywords: Ehlers-Danlos syndrome, elastosis perforans serpiginosa