Felis ISSN 2398-2950

Skin: cutaneous asthenia

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

Contributor(s): David Godfrey, Rosanna Marsella, Ian Mason, David Scarff, Susan E Shaw

Introduction

  • Rare.
  • Cause: a group of genetic, congential connective tissue diseases.
  • 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 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.
  • In one case, a deficiency in procollagen-N-peptidase enzyme was considered responsible.
  • 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.

Pathophysiology

  • Collagen dysplasia that results in reduced tensile strength of affected tissues.
  • Various forms of cutaneous asthenia in different species can be recessive or autosomal dominant.
  • In the recessive form of the disease, the fibrils are twisted and this abnormality seems to be caused by a deficiency of the enzyme procollagen terminal peptidase.
  • In the dominant form both normal and disorganized fibers are mixed.
  • The homozygotic form is probably lethal.

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.
  • Olivry T, Dunston S M & Marinkovich M P (1999) Reduced anchoring fibril formation and collagen VII immunoreactivity in feline dystrophic epidermolysis bullosa. Vet Path 36 (6), 616-618 PubMed.
  • Sequeira J L, Rocha N S, Bandarra E P et al (1999) Collagen dysplasia (cutaneous asthenia) in a cat. Vet Pathol 36 (6), 603-606 PubMed.
  • Danielson K G, Baribault H, Holmes D F et al (1997) Targeted disruption of decorin leads to abnormal collagen fibril morphology and skin fragility. J Cell Biol 136 (3), 729-743 PubMed.
  • Regnier A & Pieraggi M T (1989) Abnormal skin fragility in a cat with a cholangiocarcinoma. JSAP 30 (7), 419-23 VetMedResource.
  • Rest J R (1989) Pathology of two possible genodermatoses. JSAP 30 (4), 230-5 VetMedResource.
  • Freeman L J, Hegreberg G A, Robinette J D (1987) Ehlers-Danlos syndrome in dogs and cats. Semin Vet Med Surg Small Anim (3), 221-7 PubMed.
  • Collier L L, Leathers C W, Counts D F (1980) A clinical description of dermatosparaxis in a Himalayan cat. Feline Practice 10 (5), 25-36 VetMedResource.
  • Counts D F, Byers P H, Holbrook K A (1980) Dermatosparaxis in a Himalyan cat I. Biochemical studies of dermal collagen. J Invest Dermatol 74 (2), 96-9 PubMed.
  • Holbrook K A, Byers P H, Counts D F (1980) Dermatosparaxis in a Himalyam cat II. Ultrastructural studies of dermal collagen. J Invest Dermatol 74 (2), 100-4 PubMed.
  • Patterson D F & Minor R R (1977) Hereditary fragility and hyperextensibility of the skin of cats. A defect in collagen fibrillogenesis. Lab Invest 37 (2), 170-9 PubMed.
  • Scott D V (1974) Cutaneous asthenia in a cat, resembling Ehlers-Danlos syndrome in man​. Vet Med Small Anim Clin​ 69 (10), 1256-8 PubMed.

Other sources of information

  • Hetton-Rhodes (1993) Advances in Veterinary Dermatology. Vol 2. Pergamon.


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