Scleromyxedema and the dermato‐neuro syndrome: case report and review of the literature

Abstract

Scleromyxedema is a generalized form of lichen myxedematosus which is characterized by a papular and sclerodermoid skin eruption resulting from dermal fibroblast proliferation and mucin deposition. The majority of patients with scleromyxedema have a monoclonal gammopathy, and other systemic manifestations are common. Herein we describe a case of the 'dermato-neuro syndrome', a rare and sometimes fatal neurologic manifestation of scleromyxedema which consists of fever, convulsions and coma, often preceded by a flu-like prodrome. In addition, we provide a comprehensive summary of previously published cases of the dermato-neuro syndrome and discuss the current etiopathogenic theories and treatment options for this rare disease.

Status epilepticus in scleromyxedema

“…[2,5] Extracutaneous manifestations are due to cardiovascular, rheumatologic, renal, hematologic and neurologic involvement. [1][2][3][4] Encephalopathy, transient focal neurological disturbances, progressive cognitive decline, seizures, peripheral neuropathy, carpal tunnel syndrome and myopathy are included in the neurological manifestations. [2,4] They could be partially explained by the paraproteinemia and the disruption of the blood-brain barrier with elevated CSF protein concentration in the central nervous system (CNS) disorders or by the deposition of mucopolysaccharides within the nerve and muscle fibers in peripheral nervous system conditions.…”

“…Neurological manifestations are rare but well-recognized in patients with scleromyxedema, among other extracutaneous complications. [1][2][3][4] We report a patient with scleromyxedema presenting with refractory status epilepticus, caused by brain lesions, probably related to the disease.…”

Dermato-neuro syndrome in a case of scleromyxedema

“…It has been proposed that an increased blood viscosity with impaired microcirculation due to paraproteinemia may result in encephalopathy. A pathogenic role for IgG crossing a damaged bloodbrain barrier, mediated by increased IL-6 production, also has been suggested (5,6). Despite many clinical investigations, the pathogenesis of the dermato-neuro syndrome remains to be an enigma because brain imaging results are normal.…”

“…The approach to patients with the dermato-neuro syndrome is not standardized, and various treatments have seemed to yield benefit in case reports, such as intravenous immunoglobin (IVIG) (7), systemic glucocorticoids plus plasmapheresis or IVIG (8), systemic glucocorticoids plus cyclophosphamide and plasmapheresis (9), melphalan plus IVIG, and bortezomib plus dexamethasone (10). Spontaneous improvement also has been reported (6). Our efforts to clarify the etiology of the patient's neurological deterioration revealed no vascular or infectious pathology, and electroencephalography findings directed us to the diagnosis of the dermato-neuro syndrome.…”

“…The diagnosis of the dermato-neuro syndrome typically depends on the exclusion of different etiologies, leading to a comatose state in patients with known scleromyxedema diagnosis. A paroxysmal triad of high fever, seizures, and coma rarely occurs (6). The approach to patients with the dermato-neuro syndrome is not standardized, and various treatments have seemed to yield benefit in case reports, such as intravenous immunoglobin (IVIG) (7), systemic glucocorticoids plus plasmapheresis or IVIG (8), systemic glucocorticoids plus cyclophosphamide and plasmapheresis (9), melphalan plus IVIG, and bortezomib plus dexamethasone (10).…”