Functional and Cutaneous Treatment Outcomes With Intravenous Immunoglobulin for Eosinophilic Fasciitis: A Retrospective Study

April 2024 | Volume 23 | Issue 4 | e107 | Copyright © April 2024


Published online March 7, 2024

doi:10.36849/JDD.8017e

Bianca Obiakor BAa, Winnie Fan BSa, Rebecca Jacobson BMb, Jocelyn Gandelman MDc, Anna Haemel MDd

aUniversity of California, San Francisco, School of Medicine, San Francisco, CA 
bUniversity of California, Irvine, School of Medicine, Irvine, CA 
cSchweiger Dermatology Group, New York, NY 
dDepartment of Dermatology, University of California, San Francisco, San Francisco, CA
 

Abstract
Background: Eosinophilic fasciitis (EF) is a rare subtype of deep morphea with an elevated risk of functional impairment. No treatment algorithm has been established for adults with EF refractory to traditional corticosteroid or immunomodulatory treatments. Research on cutaneous and functional outcomes of alternative therapies, such as intravenous immunoglobulin (IVIG), remains scarce. 
Objective: To describe the functional and cutaneous outcomes associated with IVIG in adults with treatment-refractory EF at a tertiary referral center.
Methods: We performed a retrospective chart review of 18 consecutive patients with EF identified through a billing code search seen within the UCSF Department of Dermatology between 2015 and 2022. 
Results: Seven patients (41.2%) underwent at least one course of intravenous immunoglobulins (IVIG) during the study period. Of 6 patients with available follow-up data, 5 patients (83.3%) achieved both sustained cutaneous and functional improvement. In the IVIG cohort, 1 patient (16.7%) achieved complete response with relapse, 4 (66.7%) were partial responders, and 1 (16.7%) was a non-responder who required treatment with mepolizumab.
Conclusion: Adverse effects of IVIG included headaches in 1 patient (14.3%) and rash in 2 patients (28.6%). There were no reported veno-occlusive or thromboembolic events associated with IVIG. 

J Drugs Dermatol. 2024;23(4):e107-e109.    doi:10.36849/JDD.8017e

INTRODUCTION

Eosinophilic fasciitis (EF) is a rare subtype of deep morphea characterized by progressive symmetric sclerosis of the fascia of the distal extremities with or without truncal involvement.1 Joint contractures, myalgias, and reduced mobility are potential complications.2,3 While oral corticosteroids remain first-line, almost half of EF patients require other immunosuppressants due to incomplete response or treatment intolerance.3,4 The use of intravenous immunoglobulin (IVIG) in addition to standard immunosuppressive treatments has been described in a small retrospective cohort study and a few case reports, which reported IVIG treatment responses in adult patients with recalcitrant EF.4-6 This retrospective observational study describes patient- and physician-reported cutaneous and functional outcomes for adults with EF treated with IVIG at a tertiary referral center. 
 
 

MATERIALS AND METHODS

This IRB-approved study is a retrospective chart review of all adult patients with EF seen within the University of California San Francisco School of Medicine (UCSF) Department of Dermatology from 1/1/2015 to 6/13/2022. Patients with morphea were identified via a search of ICD-10 billing codes: morphea (L94.0) and eosinophilic fasciitis (M35.4). Authors B.O., W.F., and J.G. reviewed electronic health records to assess for clinical diagnosis of morphea. Author A.H. validated cases of diagnostic uncertainty. Data on demographics, morphea subtype, disease characteristics, treatment course, and response were reviewed. Patients were classified as complete responders, partial responders, or non-responders based on erythema and/or induration (resolved, decreased, or increased, respectively), new or expanding lesions (absent, absent, and present, respectively), functional impairment (significantly