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A case is presented of a 17-year-old boy with disseminated skin lesions of elastosis perforans serpiginosa of five years' duration, who died of a ruptured thyrocervical artery. At autopsy examination, them was evidence of systemic involvement of the connective tissue. Histologic study showed, in addition to the skin findings of dermal elastosis, fragmentation of the elastic fibers within the wall of large blood vessels, with foci of deposition of acid mucopolysaccharide suggesting cystic medial necrosis, and elastic tissue breakdown of perivisceral fat, mesentery, fascia, and perialveolar areas of the lungs. This case may represent Ehlers-Danlos syndrome, type 4, or “ecchymotic” type, or “arterial” type, but probably represents a case of Sack-Barabas disease.