IVIg responders have included where patients were treated with Ig as first line therapy or after failure to respond to steroids and other immunosuppressant agents. Indications Scleromyxedema (skin involvement only) unresponsive to steroids Two indications have been developed to and other immunosuppressant agents or where contra-indicated support differing of access to Ig therapy. Patients with skin involvement only, must be Scleromyxedema - systemic involvement as first line therapy non-responsive to alternative medications while first-line treatment is allowed for patients with systemic disease. Scleromyxedema is a chronic, idiopathic disorder characterized by Intravenous The description and diagnostic criteria section Description excessive deposition of mucin in the skin in association with immunoglobulin (IVIg) and has been updated to reflect the findings of increased dermal collagen and absence of thyroid disease, and may be indicated in Diagnostic more recently published evidence regarding usually associated with a monoclonal gammopathy. Extracutaneous select cases not Criteria disease presentation, progression and systemic manifestations also occur with neurologic, rheumatologic, responding to management. pulmonary, renal, muscular and/or cardiac involvement. steroids, or when steroids and other This is a rare condition with proven difficulty in establishing specific alternative treatments or definitive treatment. Successful treatment of skin disease has (e.g. thalidomide) are been reported with melphalan, dexamethasone, thalidomide, contraindicated. lenalidomide and IVIg. In the three largest case series, CNS was predominantly involved in 10-15% of cases associated with poor clinical outcome, often with global CNS dysfunction with encephalopathy, seizures and coma. The pathophysiology of this fatal complication of Scleromyxedema remains unknown. Evidence supporting effective treatment may suggest that targeting the paraprotein may be of benefit. Most of the reports of successful treatment of CNS manifestations are with IVIg.
IVIg responders have included where patients were treated with Ig as first line therapy or after failure to respond to steroids and other immunosuppressant agents. Indications Scleromyxedema (skin involvement only) unresponsive to steroids and other immunosuppressant agents or where contra-indicated Scleromyxedema - systemic involvement as first line therapy Two indications have been developed to support differing of access to Ig therapy. Patients with skin involvement only, must be non-responsive to alternative medications while first-line treatment is allowed for patients with systemic disease. Description and Diagnostic Criteria Intravenous immunoglobulin (IVIg) may be indicated in select cases not responding to steroids, or when steroids and other alternative treatments (e.g. thalidomide) are contraindicated. Scleromyxedema is a chronic, idiopathic disorder characterized by excessive deposition of mucin in the skin in association with increased dermal collagen and absence of thyroid disease, and usually associated with a monoclonal gammopathy. Extracutaneous systemic manifestations also occur with neurologic, rheumatologic, pulmonary, renal, muscular and/or cardiac involvement. This is a rare condition with proven difficulty in establishing specific or definitive treatment. Successful treatment of skin disease has been reported with melphalan, dexamethasone, thalidomide, lenalidomide and IVIg. In the three largest case series, CNS was predominantly involved in 10-15% of cases associated with poor clinical outcome, often with global CNS dysfunction with encephalopathy, seizures and coma. The pathophysiology of this fatal complication of Scleromyxedema remains unknown. Evidence supporting effective treatment may suggest that targeting the paraprotein may be of benefit. Most of the reports of successful treatment of CNS manifestations are with IVIg. The description and diagnostic criteria section has been updated to reflect the findings of more recently published evidence regarding disease presentation, progression and management.