Scleromyxedema

RecommendationsIG may be considered in severe scleromyxedema when other therapies are ineffective or contraindicated.

Qualifying Criteria
Diagnosis should be made by an appropriate specialist such as a dermatologist.
Dose/Frequency of Administration2 g/kg adjusted body weight divided over 2 to 5 days.

In the case of severe organ involvement, particularly kidney or heart, the treatment should be administered slowly (i.e., over 5 days).

IVIG should be administered every 4 weeks initially. If clinical response is good, based on objective measures of effectiveness established at the outset of treatment, the interval between infusions can be gradually increased.

IVIG should be administered for 6 months to assess efficacy