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Scleromyxedema

Key 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).
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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
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2.Medical management

General principles
Weight the risk-benefit ratios of treatment carefully before initiating therapy.
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Admit patients with neurologic and flu-like symptoms (possible dermato-neuro syndrome) to the hospital for close observation and evaluation.
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  • First-line therapy

  • Second-line therapy

  • Third-line therapy

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.
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