Scleromyxedema with monoclonal gammopathy and neurological involvement: recovery from coma after plasmapheresis?

Prado, Aline Defaveri do; Schmoeller, Deonilson Ghizoni; Bisi, Melissa Cláudia; Piovesan, Deise Marcela; Dias, Fernando Suparregui; Staub, Henrique Luiz

doi:10.1111/j.1365-4632.2010.04691.x

Cited by 7 publications

(5 citation statements)

References 17 publications

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“…35,36 In cases of DNS, hospitalization in the intensive care unit in a reference center is necessary, with treatment consisting of a combination of HDIVig, high-dose steroids, and plasmapheresis. 14,15,37,38 Rare cases of the spontaneous regression of DNS have *Bortezomib-or lenalidomide-based regimen. † Response will be assessed clinically and hematologically according to International Myeloma Working Group criteria.…”

Section: Discussionmentioning

confidence: 99%

Plasma cell-directed therapies in monoclonal gammopathy-associated scleromyxedema

Mahévas¹,

Arnulf

Bouaziz

et al. 2020

Blood

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Scleromyxedema is a rare skin and systemic mucinosis that is usually associated with monoclonal gammopathy (MG). In this French, multicenter, retrospective study of 33 patients, we investigated the clinical and therapeutic features of MG-associated scleromyxedema. Skin molecular signatures were analyzed using a transcriptomic approach. Skin symptoms included papular eruptions (100%), sclerodermoid features (91%), and leonine facies (39%). MG involved an IgG isotype in all patients, with a predominant λ light chain (73%). Associated hematologic malignancies were diagnosed in 4/33 patients (12%) (smoldering myeloma, n=2; chronic lymphoid leukemia, n=1; and refractory cytopenia with multilineage dysplasia n=1). Carpal tunnel syndrome (33%), arthralgia (25%) and dermato-neuro syndrome (DNS) (18%) were the most common systemic complications. One patient with mucinous cardiopathy died of acute heart failure. Intravenous immunoglobulin (HDIVig) treatment alone or in combination with steroids appeared to be quite effective in nonsevere cases (clinical complete response achieved in 13/31 patients). Plasma cell-directed therapies using lenalidomide and/or bortezomib with dexamethasone and HDIVig led to a significant improvement in severe cases (HDIVig-refractory or cases with central nervous system or cardiac involvement). The emergency treatment of DNS with combined plasmapheresis, HDIVig, and high-dose corticosteroids induced the complete remission of neurological symptoms in 4/5 patients. Quantitative reverse transcriptase-PCR (RT-PCR) analysis of 6 scleromyxedema skin samples showed significantly higher profibrotic pathway levels (transforming growth factor β (TGFβ) and collagen-1) than in healthy skin. Prospective studies targeting plasma cell clones and/or fibrotic pathways are warranted for long-term scleromyxedema management.

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Section: Discussionmentioning

confidence: 99%

Plasma cell-directed therapies in monoclonal gammopathy-associated scleromyxedema

Mahévas¹,

Arnulf

Bouaziz

et al. 2020

Blood

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“…In another case, coma was precipitated after IFN‐α‐2b therapy 91 . ECP or photopheresis was also noted to produce short‐ and long‐term cutaneous and systemic symptom resolution in SMX, either as a single agent or in combination with other treatments like steroids, immunosuppression, and bath PUVA 91‐94 . PUVA is known to be effective in a number of inflammatory and fibrotic skin disorders by inducing T‐cell apoptosis, collagenase activity, and antiproliferative pathways.…”

Section: Discussionmentioning

confidence: 99%

Scleromyxedema treatment: a systematic review and update

2020

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Importance Scleromyxedema is a chronic disease with high morbidity and mortality and no definitive therapeutic guidelines. Objective To review all available data on the efficacy and the safety of the available treatments of scleromyxedema and suggest a possible therapeutic approach. Evidence Review We performed a systematic literature review in Pubmed/Medline, Embase, and Cochrane collaboration databases, searching for all articles since 1990 on the treatments of scleromyxedema, with no limits on participant age, gender, or nationality. Findings Ninety-seven studies were included in this systematic review, of which one prospective, two retrospective, 70 case reports/case series, and 24 letters/correspondence/ clinical image. Intravenous immunoglobulin (IVIG) was the most used first-line therapy based on its efficacy and its generally well-tolerated nature; most patients require continued treatment to remain in remission. Thalidomide and systemic glucocorticoids were mostly considered as second-line therapies and were given alone or in association with IVIG. Patients with severe or refractory disease were treated with autologous bone marrow transplantation, melphalan, or bortezomib with dexamethasone. Conclusions and Relevance Consideration of patient comorbidities, disease distribution, clinician experience, and treatment accessibility is mandatory in every therapeutic approach of scleromyxedema.

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“…2,3,6 In patients with dermato-neuro syndrome, although many patients appeared to spontaneously recover, a number who were given supportive care only died of their disease. 5,26,34 To date, plasmapheresis 10,27,32,39 and IVIG 33,37 appear to have resulted in the most dramatic recovery of neurological status. Our patient did not respond to plasmapheresis and steroids, but subsequently had a good response to melphalan and IVIG.…”

Section: Discussionmentioning

confidence: 99%

“…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 with monoclonal gammopathy and neurological involvement: recovery from coma after plasmapheresis?

Cited by 7 publications

References 17 publications

Plasma cell-directed therapies in monoclonal gammopathy-associated scleromyxedema

Plasma cell-directed therapies in monoclonal gammopathy-associated scleromyxedema

Scleromyxedema treatment: a systematic review and update

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

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