Diagnosis, treatment and management of epidermolysis bullosa

Br J Nurs. 2016;25(8):428-31. doi: 10.12968/bjon.2016.25.8.428.

Abstract

Epidermis bullosa is a genetically inherited disease in which painful blistering of the skin or mucous membranes occurs after minor trauma. It is a lifelong problem. The diagnosis should be confirmed by a specialist, preferably at a specialist unit where a treatment plan and follow-up arrangements for professionals and families can be put in place. Nurses will be involved in frequent dressings of wounds, after extra analgesia, and may need to be alert to any need for further specialist referral, especially in the case of complications such as infection, deformities, gastrointestinal strictures and possible skin cancers. Genetic counselling should also be offered to families, especially when considering the possible risks to future pregnancies.

Keywords: Cause; Complications; Epidermolysis bullosa; Genetic counselling; Nursing care; Symptoms.

Publication types

  • Review

MeSH terms

  • Bandages*
  • Carcinoma, Squamous Cell / diagnosis
  • Carcinoma, Squamous Cell / etiology
  • Carcinoma, Squamous Cell / nursing
  • Cicatrix / etiology
  • Cicatrix / nursing
  • Constipation / etiology
  • Constipation / nursing
  • Dehydration / etiology
  • Dehydration / nursing
  • Disease Management
  • Epidermolysis Bullosa / complications
  • Epidermolysis Bullosa / diagnosis
  • Epidermolysis Bullosa / nursing*
  • Genetic Counseling
  • Home Care Services*
  • Humans
  • Pain Management / nursing*
  • Pruritus / etiology
  • Pruritus / nursing
  • Referral and Consultation*
  • Skin Care / nursing*
  • Skin Diseases, Bacterial / etiology
  • Skin Diseases, Bacterial / nursing
  • Skin Neoplasms / diagnosis
  • Skin Neoplasms / etiology
  • Skin Neoplasms / nursing