Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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Two Cases of Toxic Epidermal Necrolysis Associated with Deflazacort Therapy in Nephrotic Syndrome: Successfully Treated with Cyclosporine A

  • Lim, Myung Hee (Department of Pediatrics, Yeungnam University Hospital, Yeungnam University College of Medicine) ;
  • Bae, Hee Jung (Department of Pediatrics, Yeungnam University Hospital, Yeungnam University College of Medicine) ;
  • Park, Sun Young (Department of Pediatrics, Yeungnam University Hospital, Yeungnam University College of Medicine) ;
  • Kim, Sae Yoon (Department of Pediatrics, Yeungnam University Hospital, Yeungnam University College of Medicine) ;
  • Park, Yong Hoon (Department of Pediatrics, Yeungnam University Hospital, Yeungnam University College of Medicine)
  • Received : 2016.09.13
  • Accepted : 2016.10.10
  • Published : 2016.10.30
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Abstract

Toxic epidermal necrolysis (TEN) is a rare, acute, serious, and potentially fatal skin disease, in which cell death causes the epidermis to separate from the dermis. It is thought to be a hypersensitivity complex that affects the skin and mucous membranes, and is caused by certain medications, infections, genetic factors, underlying immunologic disease, or more rarely, cancers. We report two cases of TEN associated with deflazacort (DFZ), a derivative of prednisolone, used in the first episode of nephrotic syndrome (NS). The skin eruption appeared on the $4^{th}$ and $5^{th}$ weeks after DFZ administration, while NS was in remission. The widespread lesions were managed by intensive supportive treatment, discontinuation of DFZ, and oral administration of cyclosporine. Both patients showed a rapid improvement in symptoms of TEN without any complications or relapse of NS.

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References

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