Lapis ISSN 2398-2969

Cutaneous asthenia

Synonym(s): Ehlers-Danlos syndrome, Ehlers-Danlos-like syndrome, Dermatosparaxis (dEDS)

Contributor(s): Beatrice Funiciello, Kristina Hunter

Introduction

  • Cause: a rare collagen defect similar to that described in human Ehlers-Danlos syndrome.
  • Signs: skin fragility with repeated spontaneous wounds and scarring.
  • Diagnosis: history, clinical examination, collagen alterations detected on electron microscopy.
  • Treatment: no treatment protocols are reported but vitamin C has been used in other species.
  • Prognosis: poor.

Pathogenesis

Etiology

  • The genetic disease is best described in humans and is reported in other animals like sheep, cattle, dog, cat, mink and horse with some differences. In animals it is often referred to as Ehlers-Danlos-like Syndrome due to these genetic variants.
  • A genetic defect is suspected also in rabbits as lesions occur in very young subjects and in siblings.

Predisposing factors

General

  • Suspected gene mutations.

Pathophysiology

  • Mutations in different collagen genes and enzyme deficiencies lead to alterations in collagen fibrils and their organization.
  • Mutations can occur in any of the several types of collagen recognized in the species of interest:
    • Formation of the normal triple-helix structure of the collagen fibrils may be impeded which may interfere with wound healing.
    • The triple-helices form covalent-bond crosslinks and function to provide tensile strength to the skin – defects in these can cause skin fragility.
  • The abnormal collagen formation results in marked skin fragility and hyperextensibility.

Timecourse

  • Defects present at birth and condition is lifelong.

Epidemiology

  • Very young rabbits (4 months to 1 year).
  • Can be seen in siblings.

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.
  • Paciello O, Lamagna F, Lamagna B & Papparella S (2003) Ehlers-Danlos-Like Syndrome in 2 Dogs: Clinical, Histologic, and Ultrastructural Findings. Vet Clin Pathol 32 (1), 13-18 PubMed.
  • White S D, Bordeaux P J & Meredith A (2002) Dermatologic problems of rabbits. Sem in Avian and Ex Pet Med 11 (3), 141-150 SciDirect.
  • Iglauer F, Wilmering G, Huidinga E, Wölm M & Lorke D E (1999) Cutaneous asthenia (Ehlers-Danlos-like syndrome) in a pet rabbit. Dtsch Tierarztl Wochenschr 106 (12), 500-505 PubMed.
  • Sinke J D, van Dijk J E & Willemse T (1997) A case of Ehlers-Danlos-like syndrome in a rabbit with a review of the disease in other species. Vet Q 19 (4), 182-185 PubMed.
  • Brown PJ, Young RD & Cripps PJ TJ (1993) Abnormalities of collagen fibrils in a rabbit with a connective tissue defect similar to Ehlers-Danlos syndrome. Res Vet Sci 53 (3), 346-350 SciDirect.
  • Harvey RG, Brown PJ, Young RD & Whitbread TJ (1990) A connective tissue defect in two rabbits similar to the Ehlers-Danlos syndrome. Vet Rec 126 (6), 130-132 PubMed.

Other sources of information

  • Halper J (2014) Connective Tissue Disorders in Domestic Animals. In: Progress in Heritable Soft Connective Tissue Diseases. Advances in Experimental Medicine and Biology. Vol 802. Springer, Netherlands. pp 231-240.
  • Meredith A (2014) Dermatoses. In: BSAVA Manual of Rabbit Medicine & Surgery. Eds: Meredith A & Lord B. BSAVA, UK. pp 255-263.
  • Miller W H, Scott D W, Griffin C E & Campbell K L (2013) Dermatoses of Exotic Small Mammals. In: Muller and Kirk’s Small Animal Dermatology. 7th edn. Elsevier, USA. pp 844-887.
  • Hess & Tater (2012) Chapter 18 Dermatologic Diseases. In: Ferrets, Rabbits, and Rodents Clinical Medicine and Surgery, 3rd ed.Eds: Quesenberry K E & Carpenter J W. Elsevier, USA. pp 232-244.


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