Literature DB >> 17098678

Junctional epidermolysis bullosa.

Chuan-Hong Kao1, Sue-Jen Chen, Betau Hwang, An-Hang Yang, Chih-Yi Hsu, Cheng-Hung Huang.   

Abstract

Epidermolysis bullosa (EB) encompasses a heterogeneous group of genodermatoses, characterized by fragility and blistering of the skin, often associated with extracutaneous manifestations. The level of vesiculation within the skin defines 3 major subtypes of EB: EB simplex, junctional EB, and dystrophic EB. We present the case of a male neonate of 36 weeks of gestation, who was born with a few blisters with erosions and who rapidly developed extensive blistering of the skin. Histopathology revealed subepidermal blistering. Electron microscopy confirmed the cleavage of epidermis from dermis within the lamina lucida. Junctional EB was the diagnosis. The patient was discharged after hospitalization for 28 days. The development of new blisters with erosions were gradually improved after AQUACEL Ag dressing, and the general condition was much better than at admission. The patient likely has a subtype of junctional EB termed generalized atrophic benign EB that clinically improves with age. He has the potential to father children and has a normal life expectancy.

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Year:  2006        PMID: 17098678     DOI: 10.1016/S1726-4901(09)70318-1

Source DB:  PubMed          Journal:  J Chin Med Assoc        ISSN: 1726-4901            Impact factor:   2.743


  2 in total

1.  Occurrence of epidermolysis bullosa along with Amelogenesis imperfecta in female patient of India.

Authors:  A P Javed; Prashanth Shenai; Laxmikanth Chatra; K M Veena; Prasanna Kumar Rao; Rachana Prabhu
Journal:  Dent Res J (Isfahan)       Date:  2013-11

2.  Otorhinolaryngological and esophageal manifestations of epidermolysis bullosa.

Authors:  Rodrigo Santana Fantauzzi; Mariana Oliveira Maia; Flávia Coelho Cunha; Rodrigo Vidal Simões; Denise Utsch Gonçalves; Amélio Ferreira Maia
Journal:  Braz J Otorhinolaryngol       Date:  2008 Sep-Oct
  2 in total

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