EPS is characterized by transepidermal elimination of dermal substances (abnormal elastic fibers) with thickened papillary dermis, which is caused by systemic fibrous tissue diseases like Down syndrome, Ehlers-Danlos syndrome, Marfan syndrome, osteogenesis imperfecta, scleroderma, and pseudoxanthoma elasticum. Also, it can be caused by D-penicillamine therapy for Wilson’s disease, cystinuria, and rheumatoid arthritis.