Successful treatment of dermato-neuro syndrome with plasmapheresis

Abstract

Altered mental status can have many causes ranging from emergent intracranial pathologies to more insidious, systemic toxic aetiologies. We report a rare case of dermato-neuro syndrome in a 71-year-old man with a known history of scleromyxoedema. The patient initially presented with encephalopathy which quickly progressed to generalised tonic–clonic seizures and coma. While his presentation fits with other, although rare, cases of dermato-neuro syndrome, it is imperative to rule out lethal, more common causes of altered mentation. Due to the rarity and difficulty in diagnosis of dermato-neuro syndrome, there is a significant debate regarding the optimal management as there are no standardised treatment protocols. In our case, the patient was successfully treated with plasmapheresis resulting in improved neurologic function.

Dermato‐neuro syndrome after COVID‐19 infection in a patient with scleromyxoedema: Previously successful treatment with intravenous immunoglobulins

“…IVIG can be administered alone or in combination with glucocorticoids and thalidomide. Alternative treatment options for patients who fail to respond to IVIG include melphalan, bortezomib + dexamethasone, plasmapheresis, 13 and autologous stem cell transplantation 14 . Maintenance treatment with IVIG provides remission of skin lesions but does not prevent dermatologic progression, and relapse occurs when therapy is discontinued.…”

Dermato-neuro Syndrome after COVID-19 Vaccination in a Patient with Scleromyxoedema Previously Controlled with Bortezomib and Intravenous Immunoglobulins

“…Thalidomide and systemic glucocorticoids, alone or in association with IVIG, are appropriate second-line therapies. Severe refractory patients are candidates for stem cell transplantation, melphalan, or bortezomib with dexamethasone ( 2 , 3 ). Bortezomib is a proteasome inhibitor which induces cell-cycle arrest and leads to enhanced apoptosis of monoclonal plasma cells.…”

“…To our knowledge, this is the first reported case of DNS secondary to SARS-CoV-2 vaccination. Two patients with SMX who developed DNS following SARS-CoV-2 infection have been reported recently ( 3 , 14 ). Both of these patients responded to plasmaphereses, while IVIG were ineffective.…”

“…Apart from the skin, SMX can involve other organs, and may be associated with a variety of gastrointestinal, pulmonary, cardiac, renal and neurological complications. The latter include dermato-neuro syndrome (DNS), a rare, potentially fatal complication, presenting as fever, seizures and coma with flu-like prodromes ( 3 ). The pathogenesis of the disease remains obscure, although plasma cell-associated monoclonal gammopathy or dysregulated cytokine stimulation of glycosaminoglycans synthesis and fibroblast proliferation have been postulated ( 4 ).…”

SARS-CoV-2: A potential trigger of dermato-neuro syndrome in a patient with scleromyxedema

“…5,6 Enhanced blood-brain-barrier permeability in the setting of monoclonal gammopathy may elevate IgG levels in the brain microvasculature, causing hyperviscosity, sludging, and neurologic abnormalities. [7][8][9] Available literature indicates that reported cases of DNS are frequently preceded by a flu-like prodrome 3,4,[9][10][11][12][13][14][15][16][17][18] or other suspected upper respiratory tract infection. 8,19 Notably, 2 case studies identified influenza A infection in association with DNS.…”