What are the guideline sources, guidelines and references for Scleromyxedema ?
Guideline sources The following summarized guidelines for the evaluation and management of scleromyxedema are prepared by our editorial team based on guidelines from the European Academy of Dermatology and Venereology (EADV 2017). 1 Guidelines 1. Screening and diagnosis Diagnosis: Diagnose scleromyxedema based on consistent clinical, pathologic and laboratory findings (paraproteins). E Recognize that the following features are supportive of the diagnosis of scleromyxedema: generalized/diffuse papular and sclerodermoid eruption microscopic triad, including mucin deposition, fibrosis and fibroblast proliferation monoclonal gammopathy absence of thyroid disorder. E 2. Medical management General principles: https://web.pathway.md/diseases/recFLGw4XsdJNvxmP 1/2 6/24/23, 3:05 PM Scleromyxedema Pathway General principles: Weight the risk-benefit ratios of treatment carefully before initiating therapy. E Admit patients with neurologic and flu-like symptoms (possible dermato-neuro syndrome) to the hospital for close observation and evaluation. E First-line therapy: Initiate high-dose IVIG as initial therapy in patients with scleromyxedema. B Recognize that long-term maintenance treatment with IVIG is usually required. E Second-line therapy: Offer thalidomide (or lenalidomide) and systemic corticosteroids as alternative treatment options alone or in conjunction with IVIG in patients with scleromyxedema. E Insufficient evidence to support the use of TNF blockers for the treatment of patients with scleromyxedema. I Third-line therapy: consider offering autologous HSCT, melphalan or bortezomib plus dexamethasone in patients not responding to IVIGs, thalidomide or systemic corticosteroids. E 3. Patient education Patient education: inform patients to seek medical care in case of developing neurologic symptoms (such as dysarthria) and flu-like illness, which may be the initial signs of dermato-neuro syndrome. E 4. Follow-up and surveillance Follow-up: obtain close clinical follow-up in patients on long-term maintenance therapy with IVIG. Reassess patients monthly with a full skin examination, review of systems and reevaluation of the therapeutic regimen. Consider increasing assessment intervals to > 4 weeks when the disease has stabilized. E Show 2 more References 1. R Knobler, P Moinzadeh, N Hunzelmann et al. European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2 Scleromyxedema, scleredema and nephrogenic systemic fibrosis. J Eur Acad Dermatol Venereol. 2017 Oct;31 10 1581 1594. Open https://web.pathway.md/diseases/recFLGw4XsdJNvxmP 2/2