RESUMEN
The treatment of epidermal necrolysis in pediatric patients remains a major challenge. Cyclosporine A has emerged as a promising therapy for epidermal necrolysis in adults; however, its efficacy in children is unclear. We present the case of a boy with Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome who was initially resistant to methylprednisolone monotherapy but improved after receiving the combination of cyclosporine A and methylprednisolone. Published reports on the use of cyclosporine A for pediatric epidermal necrolysis are also briefly reviewed.
RESUMEN
Background: Autologous non-cultured cell (ANCC) spray has been used to treat burns, chronic wounds, and vitiligo, but its use in junctional epidermolysis bullosa (JEB) has not been published previously. Chronic wounds in JEB are caused by mutations of laminin 332 (L322), whose function is to attach and act as a glue in the basal membrane. It is proposed that ANCC applications can provide keratinocytes and fibroblasts required to improve epithelization and spontaneously correct revertant keratinocytes in the wound area. Purpose: To develop a modified procedure of ANCC spray and improve epithelization using silver sulfadiazine covered with plastic wrap to treat chronic wounds of JEB. Patients and Methods: Shave excision of the donor site was performed on a 19-year-old girl with JEB. The ANCC spray was prepared and applied to the chronic wound, which was then covered with silver sulfadiazine occluded with plastic wrap. Results: Following the ANCC spray application, epithelization was successfully initiated. Unfortunately, the wounds recurred after four months of follow-up. Conclusion: The modified application method of ANCC spray provides a good alternative to treat chronic wounds in JEB.
RESUMEN
A 51-year-old man came to the dermatology and venereology outpatient clinic with the complaint of multiple itchy lumps on his body. The patient had a previous history of hemodialysis due to end-stage renal disease. He also had a previous history of hepatitis B, an abnormal liver function test, and high blood sugar. Histopathological examination with Masson's trichrome revealed that the patient had extrusion collagen in the epidermis which had invagination. Acquired perforating disorder is frequently misdiagnosed as other skin diseases, thus this condition is underdiagnosed. In addition, underlying diseases frequently associated with acquired perforating collagenosis are chronic kidney disease, hepatic insufficiency, and diabetes mellitus. The patient in this case had all 3 conditions. Further investigation is needed to determine whether acquired perforating disorder with multiple underlying diseases will have similar severity with single underlying disease.