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Epidermolysis bullosa
Synonym(s): Inherited, fragile skin
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.
Acute presentation
- Bullae and ulcers typically affect the oral cavity, coronets, mucocutaneous junctions and skin over bony prominences.
Geographic incidence
- Worldwide.
Age predisposition
- Present at birth or soon after.
Breed/Species predisposition
- Simmental Simmental.
- Hereford Hereford.
- Belgian Blue Belgian Blue.
- Charolais Charolais.
- Jersey x Friesian Jersey Friesian.
- Brangus.
- Vorderwald.
- Rotes Howhenvieh.
Cost considerations
- Loss of individual animals and loss of breeding lines.
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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Treatment
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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.