Severe Cutaneous Adverse Reactions during Tapering of High-Dose Systemic Steroid Therapy for Autoimmune Diseases: Implications for Non-HIV Immune Reconstitution Inflammatory Syndrome

Abstract

We present 2 cases of severe cutaneous adverse reactions (SCARs) during the tapering of corticosteroids, following several courses of high-dose pulse therapy for Vogt-Koyanagi-Harada disease. Their general symptoms and mucous membrane lesions, including those of the eye, were milder than those usually seen in Stevens-Johnson syndrome/toxic epidermal necrolysis. Based on their initial presentation, these cases were not initially identified as SCARs, but continued to progress over the course of a few days. The mechanism underlying the paradoxical response to steroid administration seen in these patients can be interpreted as immune reconstitution inflammatory syndrome in human immunodeficiency virus-negative patients.

Keywords

• Immune reconstitution inflammatory syndrome
• Stevens-Johnson syndrome/toxic epidermal necrolysis
• Steroid pulse therapy
• Vogt-Koyanagi-Harada disease