Kidney Res Clin Pract.  2014 Dec;33(4):222-225. 10.1016/j.krcp.2014.08.002.

Toxic epidermal necrolysis associated with deflazacort therapy with nephrotic syndrome

• Lee EC
• Kim GA
• Koo JW

• KMID: 2224265
• DOI: http://doi.org/10.1016/j.krcp.2014.08.002

Abstract

Toxic epidermal necrolysis (TEN) is a drug-related fatal disease. Extensive necrosis of the epidermis can lead to serious complications. This report describes two cases of TEN, associated with deflazacort (DFZ), in two boys, aged 4 years and 14 years, with nephrotic syndrome (NS). The 14-year-old male teenager received DFZ following NS relapse. After 17 days, pruritic papules appeared on the lower extremities. Another case involved a 4-year-old boy receiving DFZ and enalapril. After a 41-day DFZ treatment period, erythematous papules appeared on the palms and soles. Within 3 days, both boys developed widespread skin lesions (>50%) and were admitted to the intensive care unit for resuscitative and supportive treatment. The patients showed improvement after intravenous immunoglobulin-G therapy. Owing to the rapid, fatal course of TEN, clinicians need to be aware of the adverse effects of this drug when treating cases of NS.

Keyword

Deflazacort; Intravenous immunoglobulin-G; Nephrotic syndrome; Toxic epidermal necrolysis

MeSH Terms

Adolescent
Child, Preschool
Enalapril
Epidermis
Humans
Intensive Care Units
Lower Extremity
Male
Necrosis
Nephrotic Syndrome*
Recurrence
Skin
Stevens-Johnson Syndrome*
Enalapril