Table of contents
Scleromyxedema
Guidelines
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).
1
Screening and diagnosis
Diagnosis
As per EADV 2017 guidelines:
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
Medical management
General principles
As per EADV 2017 guidelines:
Weigh 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
More topics in this section
First-line therapy
Second-line therapy
Third-line therapy
Patient education
Follow-up and surveillance
Follow-up: as per EADV 2017 guidelines, 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
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