RECURRENT ERYTHEMA MULTIFORME, RESOLVED AFTER IMMULOGLOBULIN REPLACEMENT THERAPY IN A PATIENT WITH NORMAL IMMUNOGLOBULIN LEVELS

Publication date: November 2018

Source: Annals of Allergy, Asthma & Immunology, Volume 121, Issue 5, Supplement

Author(s): A. McInerney, S. Siegel

Introduction

While immunoglobulin has been used for Stevens-Johnson syndrome (SJS), its use in erythema multiforme (EM) is limited. Our case describes recurrent EM after SJS with antibiotic exposure and Mycoplasma pneumoniae infection, with normal immunoglobulin levels, which failed standard treatment but resolved following immunoglobulin replacement therapy (IRT).

Case Description

A 13-year-old boy developed fever, cough, and rash progressing to generalized sloughing bullae, with dyspnea that deteriorated to emphysematous lung changes. The lesions were targetoid with erythematous base, dusky center, including mucosa. Symptoms developed after treatment for presumed pneumonia with amoxicillin-clavulanate. He was admitted with SJS and Mycoplasma pneumoniae. Steroids, azithromycin, and acyclovir were given. Biopsy was consistent with EM. He was discharged on azithromycin, doxycycline, and valacyclovir for persistent lesions, although HSV titers and cultures were negative. Immune work-up was unremarkable. He lacked immunity to multiple pathogens, despite vaccination, but responded to re-immunization. Immunoglobulins remained normal. Lesions recurred frequently, with minimal response to steroids, and flares with weaning. IRT was administered during re-admission with near resolution. He was then started on IRT therapy. Steroids were weaned without recurrence.

Discussion

Traditionally described secondary to HSV, Mycoplasma and medications are implicated frequently in pediatric EM. Standard treatment includes prolonged antivirals, antibiotics, and steroids. With treatment failure, immunosuppressants, anti-malarials, and IRT are used. However, controlled trials and consistent data are lacking. As our patient demonstrates, IVIG's immunomodulatory effect can facilitate resolution of recurrent EM. Furthermore, prophylactic use for infections may prevent recurrence. Finally, IVIG represents an alternative to immunosuppressants in pediatric patients with recalcitrant EM despite normal immunoglobulins.

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