Scleromyxedema with dermato-neuro syndrome

Gonzalez, J.R. Flores; Palangio, Mark; Schwartz, Joseph; Klainer, Albert S.; Bisaccia, Emil

doi:10.1016/s0190-9622(00)90274-3

Cited by 39 publications

(28 citation statements)

References 8 publications

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“…13,43 Treatment with IV steroids alone resulted in normalized mental status in one patient 8 and no improvement in another patient who rapidly deteriorated and ultimately died. 12 Our patient's symptoms were most consistent with dermatoneuro syndrome and readily responded to a combination of IVIG and corticosteroid, despite an initial lack of response to IV corticosteroid alone. Although there are many reports of scleromyxedema-induced CNS dysfunction responding favorably to IVIG, 5,9,11,34 we believe this to be the first documented case of dermatoneuro syndrome successfully treated with IVIG.…”

Section: Discussionmentioning

confidence: 50%

“…13 It is a rare manifestation of scleromyxedema with only 10 documented cases. [6][7][8]12,13,[39][40][41][42][43] Typically, dermatoneuro syndrome has been treated with intensive care monitoring, [39][40][41] plasmapheresis, 42 IV steroids, 8,12 or combinations of these treatments with variable outcomes. 13,43 Improved mental status has been observed in patients treated with plasmapheresis alone 42 and patients given combined treatment with both plasmapheresis and steroids.…”

Section: Discussionmentioning

confidence: 99%

“…2,5 Lung involvement may present with either a restrictive or obstructive pattern and may be associated with pulmonary hypertension. 2,7 Involvement of the central nervous system (CNS) is less common (10% of patients), 5,[8][9][10][11][12] and can range from mild confusion to fatal encephalopathy. 7 A rare manifestation of CNS involvement is the so-called dermatoneuro syndrome, which consists of fever, coma, and seizures with a flu-like prodrome.…”

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confidence: 99%

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Treatment of scleromyxedema and the dermatoneuro syndrome with intravenous immunoglobulin

Rey

Luria

2009

Journal of the American Academy of Dermatology

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Section: Discussionmentioning

confidence: 50%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Treatment of scleromyxedema and the dermatoneuro syndrome with intravenous immunoglobulin

Rey

Luria

2009

Journal of the American Academy of Dermatology

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“…Neurologic abnormalities occur. Several cases of severe acute central nervous system syndrome and encephalopathy have been reported in patients with scleromyxedema [45][46][47]. Unlike the relatively benign course characteristic of scleredema of Buschke, scleromyxedema is rarely selflimited.…”

Section: Scleromyxedemamentioning

confidence: 99%

Scleroderma-like cutaneous syndromes

Mori¹,

Kahari²,

Varga

2002

Curr Rheumatol Rep

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Several distinct entities associated with dermal fibrosis can mimic scleroderma/systemic sclerosis. The list of scleroderma-like conditions or scleroderma variants includes eosinophilic fasciitis, localized forms of scleroderma, scleredema and scleromyxedema, keloids, and environmental exposure-associated conditions including eosinophilia-myalgia syndrome and pseudosclerodermas induced by various drugs. Although these conditions are relatively uncommon, their accurate recognition is essential to avoid misdiagnosis and inappropriate therapy. The pathogenesis of these scleroderma variants appears to share similarities with each other and with that of scleroderma. Better understanding of scleroderma-like disorders is emerging through epidemiologic investigations, and in vivo and in vitro experimental research. Activation of eosinophils and disordered regulation of fibroblast collagen synthesis, apoptosis, and proliferation are recurrent findings in these disorders. The etiologic role of infection with Borrelia species or other microorganisms remains controversial. Cytokines such as transforming growth factor-beta, interleukin-4, interleukin-13, and connective tissue growth factor contribute to fibrosis in these disorders by inducing an accentuated and persistent fibrogenic response to tissue injury. The role of genetic factors in susceptibility and clinical expression of scleroderma-like conditions remains to be systematically addressed. Because of the relative rarity of these conditions, few well-controlled clinical treatment trials have been performed. In addition, there is no consensus on optimal management. Much anecdotal information and small clinical series indicate that phototherapy may have a role in the treatment of scleroderma-like conditions.

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“…Encephalopathies with grave prognosis appear not uncommonly during scleromyxedema without their frequency being accurately known [2]: central nervous system impairment is found in 10% of patients with scleromyxedema by Rey et al, [3]; confusion, coma, psychiatric disorders and focal neurological deficit can be observed [3]. A syndrome, sometimes fatal, consisting in fever, seizures and coma with a flu-like prodrome can rarely occur and is named "dermato-neuro syndrome'' [4,5]: Our patient had this particular presentation. Several assumptions have been made regarding the pathophysiology of neurological impairment in scleromyxedema [2]: damage secondary to the monoclonal gammapathy with hyperviscosity, mucin deposition in the brain.…”

Section: Case Reportmentioning

confidence: 99%