Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
Home Print this page Email this page Small font size Default font size Increase font size Users Online: 93
Year : 2017  |  Volume : 18  |  Issue : 4  |  Page : 267-273

Inherited epidermolysis bullosa: A multisystem disease of skin and mucosae fragility

Department of Dermatology, National Institute of Pediatrics, Mexico City, Mexico

Correspondence Address:
María Teresa García-Romero
National Institute of Pediatrics, Insurgentes Sur 3700c, Col. Insurgentes Cuicuilco, México DF 04530
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_16_17

Rights and Permissions

Epidermolysis bullosa (EB) is a blistering disorder that can be autosomic or dominantly inherited and has a wide spectrum of clinical presentations. The most recent classification divides EB into four basic subtypes: (1) EB simplex, (2) junctional EB, (3) dystrophic EB and (4) Kindler syndrome; depending on the level of the epidermal-dermal junction where the formation of blisters is present. With the use of immunohistochemistry and molecular biology, new mutation genes and proteins have been identified and more than thirty subtypes of EB have been described according to text phenotype, each affecting different key proteins for the structural integrity of the skin. The phenotype can be a mild one where blisters occur after minor trauma, but quality of life is preserved or a severe one with multisystemic manifestations affecting skin, mucous membranes, bones, joints, nutritional status, and cancer development leading to early death. We describe the clinical manifestations, diagnostic approach, and multidisciplinary management of EB subtypes.

Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)

 Article Access Statistics
    PDF Downloaded558    
    Comments [Add]    
    Cited by others 2    

Recommend this journal