JPC SYSTEMIC PATHOLOGY

INTEGUMENT SYSTEM

September 2016

I-M07

 

Signalment (JPC 21474-5/6):  Mature male springer spaniel

HISTORY:  This dog was undergoing treatment for a large gaping skin wound; he also had a striking amount of excessive skin, especially on the lower extremities.  The owner reported that the dog is prone to such wounds and that the skin has been “peculiar” since birth.

HISTOPATHOLOGIC DESCRIPTION:  Haired skin:  Diffusely the dermis is thin with a paucity of collagen fibers and close approximation of adnexa with moderately decreased numbers of sebaceous glands.  Collagen fibers are haphazardly arranged, thin, wispy, pale, often fragmented, and widely separated by clear space.  Apocrine glands are frequently ectatic.  There are low numbers of lymphocytes scattered throughout the dermis.  There is mild, multifocal epidermal thinning to as few as one to two cells, as well as diffuse, mild orthokeratotic hyperkeratosis.

MORPHOLOGIC DIAGNOSIS:  Haired skin, dermis: Collagen hypoplasia and dysplasia, diffuse, marked, springer spaniel, canine

CAUSE:  Hereditary dermal collagen dysplasia

CONDITION:  Collagen dysplasia

SYNONYMS:  Ehlers-Danlos syndrome (EDS), cutaneous asthenia, dermatosparaxis, collagen dysplasia

GENERAL DISCUSSION: 

PATHOGENESIS: 

TYPICAL GROSS FINDINGS: 

TYPICAL LIGHT MICROSCOPIC FINDINGS: 

ULTRASTRUCTURAL FINDINGS:

ADDITIONAL DIAGNOSTIC TESTS: 

DIFFERENTIAL DIAGNOSIS:

Gross differentials:

 Histological differentials:

 COMPARATIVE PATHOLOGY: 

References: 

  1. Badial PR, Oliveira-Filho JP, Pantoja JC, Moreira JC, Conceicao LG, Borges AS. Dermatological and morphological findings in quarter horses with hereditary equine regional dermal asthenia. Vet Dermatol. 2014; 25(6):547-554.
  2. Cooper TK, Zhong Q, et al. The haploinsufficient Col3a1 mouse as a model for vascular Ehlers-Danlos syndrome. Vet Pathol. 2010;47(6) 1028–1039.
  3. Gross TL, Ihrke PJ, Walder EJ, Affolter VK. Skin diseases of the dog and cat.  2nd ed. Ames, IA: Blackwell Publishing Professional; 2005:386-389.
  4. Hargis AM, Ginn PE. The integument. In: McGavin MD, Zachary JF, Pathologic Basis of Veterinary Disease. 5th St. Louis, MO: Mosby Elsevier; 2012:990,1009-1010.
  5. Maudlin EA, Peters-Kennedy J. Integumentary system. In: Maxie MG, ed. Jubb, Kennedy, and Palmer’s Pathology of Domestic Animals. Vol 1. 6th Philadelphia, PA: Elsevier Saunders; 2016: 534.
  6. Miller WH, Griffin CE, Campbell KL. Muller & Kirk"s Small Animal Dermatology. 7th ed. St Louis, MO: Elsevier; 2013:603-604.
  7. Mochal CA, Miller WW, Cooley AJ, Linford RL, Ryan PL, Rashmir-Raven AM. Ocular findings in quarter horses with hereditary equine regional dermal asthenia. J Am Vet Med Assoc. 2010; 237(3):304-310.
  8. Monteagudo LV, Ferrer LM, Catalan-Insa E, Savva D, McGuffin LJ, Tejedor MT. In silico identification and three-dimensional modelling of the missense mutation in ADAMTS2 in a sheep flock with dermatosparaxis. Vet Dermatol. 2015; 26(1):49-52.
  9. Rashmir-Raven A, Lavagnino M, Sedlak A, Gardner K, Arnoczky S. Increased susceptibility of skin from HERDA (hereditary equine regional dermal asthenia)-affected horses to bacterial collagenase degradation: a potential contributing factor to the clinical signs of HERDA. Vet Dermatol. 2015; 26(6):476-80.
  10. Uri M, Verin R, Ressel L, Buckley L, McEwan N. Ehlers-Danlos syndrome associated with fatal spontaneous vascular rupture in a dog. J Comp Pathol. 2015; 152(2-3): 211-216.


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