Dermatoneuro Syndrome (DNS) in Scleromyxedema

Features of the dermatoneuro syndrome:

(1) scleromyxedema with monoclonal gammopathy

(2) flu-like prodrome

(3) fever

(4) skin lesions

(5) neurologic deterioration (seizures, confusion, coma)

(6) absence of other systemic findings

The mechanism is uncertain. Charles et al believed that the monoclonal protein resulted in neutrophilic aggregates that impaired CNS function. Bhoyrul et al hypothesized a viral infection. Others have suggested a cytokine-related disorder.