INTRODUCTION: Decompression sickness (DCS) is a medical condition caused by outgassing of dissolved nitrogen following rapid ascent by divers and aviators. Cutaneous DCS, historically termed cutis marmorata (CM), presents as a predominantly truncal reticular violaceous-to-dusky eruption. The prevailing theories for its pathogenesis include: localized cutaneous outgassing, paradoxical embolism across a right-to-left shunt (RLS), and brainstem emboli disrupting autonomic control of cutaneous microcirculation.METHODS: We conducted a systematic review of reports of cutaneous DCS to investigate relationships among CM, RLS, and neurological sequelae to better elucidate the mechanism of CM. A literature search examining reports of cutaneous DCS yielded 31 eligible studies, comprising a pooled total of 128 patients.RESULTS: Of the patients with documented workup, 84% showed evidence of RLS with CM. Subsequently 18 patients underwent percutaneous closure of intracardiac RLS with no recurrence of DCS. Of the patients with documented neurological evaluations, 57% experienced both CM and neurological DCS manifestations. The coexistence of RLS and neurological symptoms with CM was noted in numerous cases; exact percentages of overlap cannot be stated due to data unavailability.DISCUSSION: Our results indicating the striking coexistence of RLS and neurological sequelae in CM patients is supportive of the paradoxical embolism theory of pathogenesis. The frequent coincidence of CM with RLS and neurological symptoms raises concern that CM may signify vulnerability to devastating systemic gas emboli. CM has historically been considered trivial and self-limiting; however, our results support reappraisal of its clinical significance and potential reclassification to the more severe subtype.Breen ID, Stepanek J, Marks L, Yale K, Mesinkovska N, Swanson D. Clinical significance of mottling rashes in diving decompression sickness. Aerosp Med Hum Perform. 2024; 95(9):695-702.