Does dermatoneuro syndrome have a viral aetiology?

Abstract

Scleromyxoedema is a rare disease characterized by a generalized papular and sclerodermoid cutaneous eruption. It is associated with fibroblast proliferation and mucin deposition in the dermis. Most patients have a monoclonal gammopathy, defined by the presence of IgG. Treatment of scleromyxoedema is challenging, but there is mounting evidence to support the use of intravenous immunoglobulin (IVIg). Individual reports of systemic complications have been described. Dermatoneuro syndrome (DNS) is a rare but sometimes fatal manifestation, which consists of a triad of fever, coma and seizures preceded by a flu-like illness. We describe a patient with scleromyxoedema who developed DNS. Our case highlights interesting findings suggesting that DNS may have a viral aetiology. In addition, this case demonstrates a favourable response of the cutaneous features of scleromyxoedema to IVIg.

Two patients with dermatoneuro syndrome after influenza A infection

“…One previous case report has reported a possible link between influenza A and DNS development in patients with scleromyxedema [1], and two more patients are reported here. Particularly in our first patient this is unlikely to be an influenza encephalopathy case, considering the normal magnetic resonance imaging findings, negative CSF influenza polymerase chain reaction, and clinical improvement after IVIG.…”

“…DNS aetiology is unclear [1]. Here two patients are described who developed DNS and tested positive for influenza A virus.…”

“…Lichen myxedematosus is a rare cutaneous disorder [1]. Additional systemic involvement defines the more severe form scleromyxedema, which may develop into dermatoneuro syndrome (DNS), defined by fever, coma and seizures with a flu-like prodrome [1].…”

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

“…The pathogenesis of DNS remains uncertain; however, reports speculate that elevated levels of vasoactive cytokines (eg, interleukin 6) during viral infection may contribute by compromising the blood-brain-barrier. 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. 6,10 Here, we describe a patient with SMX complicated by 2 episodes of DNS, each associated with different RNA virus infections: Influenza A or SARS-CoV-2.…”