ISSN 2398-2993      

Epidermolysis bullosa

obovis
Contributor(s):

Catherine Fraser

Ben Dustan

Synonym(s): Bullous epidermolysis, epidermolysis bullosa simplex, junctional epidermolysis bullosa, dystrophic epidermolysis bullosa, skin blistering


Introduction

  • Epidermolysis bullosa is a heterogeneous group of inherited diseases characterized by skin blistering and fragility following trivial trauma.
  • Cause: hereditary disease of the basement membrane involving abnormal keratin intermediate filaments and/or abnormal anchoring mechanisms.
  • Signs: blistering skin disease affecting oral cavity and frictional areas.
  • Diagnosis: signalment, history, clinical signs and biopsy.
  • Treatment: none effective.
  • Prognosis: poor.

Pathogenesis

Etiology

  • Hereditary defect of various components of the epidermis and/or basement membrane.
  • It is further classified as:
    • Epidermolysis bullosa simplex defect is in the intermediate filaments (keratin 5 or 14).
    • Junctional epidermolysis bullosa defect in integrins ±6 or ²4 , type XVIII collagen, or laminin 5.
    • Dystrophic epidermolysis bullosa defect in type VII (anchoring fibrils).

Predisposing factors

General

  • Genetically inherited disease.

Pathophysiology

  • Due to defect in proteins important for anchoring the basement membrane, cleavage occurs either in the deeper epidermis or in the basement membrane. 
  • Vesicles or bullae develop. 
  • As these lesions are very fragile, rupture easily occurs → ulcers.

Timecourse

  • Calves may be born with lesions or they become noticeable in the first few days after birth.
  • Calves rapidly develop secondary infection of the extensive ulcers and are euthanized.

Epidemiology

  • Epidermolysis bullosa in Charolais cattle Charolais has been shown to be associated with a 4.4kb deletion in the integrin beta gene
  • A stop-gain in the laminin, alpha 3 gene causes recessive junctional epidermolysis bullosa in Belgian Blue cattle Belgian Blue.
  • In Hereford Hereford calves it is caused by a deletion in LAMC2 gene.
  • A COL7A1 mutation causes dystrophic epidermolysis bullosa in Rotes Hohenvieh and Vorderwald  cattle.

Diagnosis

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Prevention

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Outcomes

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Further Reading

Publications

Refereed Papers

  • Recent references from PubMed and VetMedResource.
  • Medeiros G X & Riet-Correa F (2015) Epidermolysis bullosa in animals: a review. Vet Derm 26 (1).
  • Foster A P et al (2010) Epidermolysis bullosa in calves in the UK. J Comp Pathol 142 (4), 336-40 PubMed.
  • Bruckner-Tuderman L, McGrath J A, Robinson E C & Uitto J (2010) Animal models of epidermolysis bullosa: Update 2010. J Invest Dermatol 130 (6), 1485-8 PubMed.
  • Matthews G & Allen M (2005) Epidermolysis bullosa in cattle in the UK. Vet Rec 157 (15), 456 PubMed.

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