Abstract
ABSTRACT
Background
Scleromyxedema (SMX) is a cutaneous mucinosis characterised by an abnormal accumulation of mucin in the skin and limited treatment options. Assessment of therapy response during treatment is challenging.
Objectives
Patients with SMX receiving high‐dose intravenous IVIg therapy were included to assess validity of the double modified Rodnan skin score (dmRSS) to assess treatment response.
Methods
Observational study assessing dmRSS in six patients (five female, one male) with SMX during high‐dose IVIg therapy for 1 year.
Results
While three patients showed a relatively stable course of their scores, dmRSS was found to increase at elongated IVIg intervals in three patients as an early indicator of disease relapse. After return to shorter treatment intervals, dmRSS declined to baseline levels. For patient 1 dmRSS increased from 6 to 17 at 4‐ instead of 3‐week intervals. For patient 2 it increased to 12 following 6‐week intervals and back to 3 at 5‐week intervals. In patient 4 after a 10‐week interval dmRSS went up to 12 and declined to 3 at 4‐week intervals. Thus, dmRSS proves sufficient to evaluate skin involvement in SMX and to predict disease relapse early.
Conclusions
DmRSS is a valuable tool to assess therapy response during IVIg treatment in SMX.