Scleromyxedema is a primary cutaneous mucinosis characterized by a diffuse and generalized papular skin eruption of mucinous deposits throughout the upper dermis. In addition to dermatologic manifestations, scleromyxedema may involve the cardiopulmonary, gastrointestinal, renal and nervous systems. Dermato-neuro syndrome (DNS) is a rare, severe neurologic complication of scleromyxedema.1,2 The pathogenesis of DNS is unknown, but…