Integra® -Dermal Regeneration Template and Split-Thickness Skin Grafting: A Therapy Approach to Correct Aplasia Cutis Congenita and Epidermolysis Bullosa in Carmi Syndrome

Dermatology and Therapy
Julian TrahIngo Königs

Abstract

The association of junctional epidermolysis bullosa with pyloric atresia (JEB-PA) and aplasia cutis congenita (ACC) was described by El Shafie et al. (J Pediatr Surg 14(4):446-449, 1979) and Carmi et al. (Am J Med Genet 11:319-328, 1982). Most patients die in the first weeks of life, and no curative treatment options are available so far. We describe a patient with JEB-PA and ACC (OMIM # 226730) who was treated for extensive areas of ACC by Integra®-Dermal Regeneration Template and split-thickness skin grafting (STSG). Clinically, the dermal template changed into well-vascularized neodermis, and after STSG, full take of the transplants was detected. No infections of the huge ACC areas were seen. Further studies must validate this treatment option in severe and acute cases of JEB-PA with ACC. Based on clinical findings, we postulate that placement of Integra®-Dermal Regeneration Template with STSG could be a new treatment option for patients having JEB-PA with ACC to prevent severe infection, compartment-syndrome-like conditions, and deformities. Based on literature findings, we assume that Integra®-Dermal Regeneration Template with STSG could even be able to prevent new blistering and thereby be a treatment option in cases of A...Continue Reading

References

Apr 1, 1986·Journal of the American Academy of Dermatology·I J Frieden
Mar 1, 1982·American Journal of Medical Genetics·R CarmiJ Bar-Ziv
Jun 1, 1994·International Journal of Dermatology·S IkedaH Ogawa
Mar 7, 2001·The Journal of Investigative Dermatology·E PfendnerJ D Fine
Feb 27, 2009·The EMBO Journal·José M de PeredaArnoud Sonnenberg
Dec 1, 2009·Dermatologic Clinics·Hye Jin Chung, Jouni Uitto
Nov 23, 2010·Plastic and Reconstructive Surgery·Naiem MoiemenMartin Freeth
Apr 3, 2014·Journal of the American Academy of Dermatology·Jo-David FineGiovanna Zambruno
Oct 31, 2014·The Journal of Investigative Dermatology·Alexander G Marneros
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May 10, 2017·International Journal of Dermatology·Benjamin M PerryScott D Hadenfeld
Nov 17, 2017·Nature·Tobias HirschMichele De Luca

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Citations

Jul 21, 2020·Pediatric Dermatology·Adrian Martinez-MorenoErika Alba-Rojas
Apr 23, 2020·Neonatal Network : NN·Mary Whalen
Mar 7, 2021·Journal of Controlled Release : Official Journal of the Controlled Release Society·Parinaz AbdollahiyanAhad Mokhtarzadeh
Apr 24, 2021·Signal Transduction and Targeted Therapy·Jiacheng HuangYiting Qiao
Aug 10, 2020·Advances in Neonatal Care : Official Journal of the National Association of Neonatal Nurses·Lauren A Stracuzzi

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Methods Mentioned

BETA
biopsies
transmission electron microscopy

Software Mentioned

Integra

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