Scleromyxedema and dermato–neuro syndrome in a patient with multiple myeloma effectively treated with dexamethasone and bortezomib

Fett, Nicole; Toporcer, Mary; Dalmau, Josep; Shinohara, Michi M.; Vogl, Dan T.

doi:10.1002/ajh.22131

Cited by 30 publications

(16 citation statements)

References 17 publications

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“…Our case has certain common features with previous reports. Elevated CSF protein has previously been reported, raising the possibility of a disruption of the blood–brain barrier . During his admission, our patient developed a high fever and severe hypertension.…”

Section: Reportmentioning

confidence: 58%

Does dermatoneuro syndrome have a viral aetiology?

et al. 2015

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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.

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Section: Reportmentioning

confidence: 58%

Does dermatoneuro syndrome have a viral aetiology?

et al. 2015

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“…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). Spontaneous improvement also has been reported (6).…”

Section: Discussionmentioning

confidence: 99%

Dermato-neuro syndrome in a case of scleromyxedema

Savran

Akarsu

2015

Eur J Rheumatol

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Scleromyxedema is an uncommon connective tissue disease characterized by mucin deposits, fibrosis, and proliferation of fibroblasts in the dermis. Although it shares similar sclerodermoid features, it is a different clinical entity than scleroderma. A monoclonal gammopathy is almost always present; however, progression to multiple myeloma is rare. It may have many systemic manifestations, of which the most notable being the dermato-neuro syndrome because of its rarity and potential fatal outcome. We present a case of a 50-year-old woman with scleromyxedema in whom the dermato-neuro syndrome developed.

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“…In addition to our case, review of the literature in the English language identified 18 cases of dermato‐neuro syndrome showing the classic presentation of acute onset seizure and coma, usually accompanied by fever and preceded by a flu‐like illness 5,8,10,14,25–28,30–40 (Table 1). There were seven additional cases in which patients with scleromyxedema had acute onset of severe neurologic symptoms, however the combination of both seizure and coma did not occur 8,9,13,26,29,41,42 (Table 2).…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2012

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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.

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Scleromyxedema and dermato–neuro syndrome in a patient with multiple myeloma effectively treated with dexamethasone and bortezomib

Cited by 30 publications

References 17 publications

Does dermatoneuro syndrome have a viral aetiology?

Does dermatoneuro syndrome have a viral aetiology?

Dermato-neuro syndrome in a case of scleromyxedema

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

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