ISSN 2398-2969      

Skin: cutaneous asthenia

icanis

Synonym(s): Ehlers Danlos Syndrome, Dermatosparaxis, skin hyperextensibility/fragility, collagen dysplasia


Introduction

  • Rare.
  • Cause: genetic.
  • Signs: hyperextensible smooth skin that tears easily.
  • Diagnosis: breed history (young age of onset), clinical signs, histopathology.
  • Treatment: wound management.
  • Prognosis: guarded to poor.

Pathogenesis

Etiology

  • A delay or arrest in maturation of collagen fibers was recently proposed as possible pathogenesis in a case of cutaneous asthenia in a Fila Brasiliero dog.
  • A defect in packing of collagen into fibrils has also been hypothesized to be due to mutations of structural proteins. In affected animals increased hyaluronic acid, increased proteodermatan sulfate levels and an altered iduronic to glucuronic acid ratio have been reported.

Pathophysiology

  • Collagen dysplasia that results in reduced tensile strength of affected tissues.
  • In dogs disease appears to be autosomal dominant. Two dogs with this syndrome were bred and only half of the offspring had fragile and hyperextensible skin.

Diagnosis

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Treatment

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Prevention

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Further Reading

Publications

Refereed papers

  • Recent references from PubMed and VetMedResource.
  • Jelínek F & Karban J (1998) Cutaneous asthenia in one dog. Acta Veterinaria Brno 67 (2), 109-113 VetMedResource.
  • Danielson K G, Baribault H, Holmes D F, Graham H, Kadler K E & Iozzo R V (1997) Targeted disruption of decorin leads to abnormal collagen fibril morphology and skin fragility. J Cell Biol 136 (3), 729-43 PubMed.
  • Minor R R, Lein D H, Patterson D F, Krook L, Porter T G & Kane A C (1983) Defects in collagen fibrillogenesis causing hyperextensible, fragile skin in dogs. JVMA 182 (2), 142-8 PubMed.
  • Poulsen P H, Thomsen M K & Kristensen F (1983) Cutaneous asthenia in the dog - a report of two cases. Nord Vet Med 37 (5), 291-7 PubMed.

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