Some patients with scleromyxedema develop the dermatoneuro syndrome (DNS). This can be a life-threatening condition.

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.

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