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Exfoliative cutaneous lupus erythematosus (lupoid dermatosus of German Shepherd short-haired pointers)

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01 October 2015, at 1:00am

DAVID GRANT continues the series of dermatology briefs

EXFOLIATIVE cutaneous lupus erythematosus (ECLE) is one of four presentations of lupus erythematosus described in the dog. The other three are systemic lupus erythematosus (SLE), discoid lupus erythematosus (DLE) and vesicular cutaneous erythematosus (VCLE).

ECLE has, to date, only been described in the German short-haired pointer. Examination of the pedigrees of affected dogs suggests an autosomal recessive form of inheritance.

The disease is considered to be an autoimmune problem as the basement membrane zone of the skin is affected (as it is with all types of lupus erythematosus) with antigenantibody complex deposition leading to an inflammatory response.

Clinical features

  • The disease starts early in life, within the first few months in many cases.
  • Waxing and waning of signs is common.
  • Initial lesions are scaling and alopecia affecting the muzzle, pinna and dorsal trunk (Figures 1 and 2).
  • Progression to involve the ventral thorax and limbs may occur.
  • Ulceration and crusting in severe cases.
  • Generalised lymphadenopathy may be a feature.
  • Frequent evidence of pain, which is an important diagnostic clue. There is stiffness and lameness, reluctance to move and the dog may cry out in pain. A hunched up stance may sometimes be adopted.
  • Intermittent pyrexia in some cases.

Differential diagnosis

  • Keratinising defects such as sebaceous adenitis.
  • Systemic lupus erythematosus.
  • Dermatophytosis.
  • Zinc responsive dermatosis.

Diagnosis

  • History and clinical signs. Age and breed of dog, cutaneous signs, waxing and waning, evidence of pain.
  • Haematological investigation. Thrombocytopenia and lymphopenia may be observed.
  • Histopathological examination. There is hyperkeratosis, with an interface dermatitis and mural folliculitis comprising basal cell degeneration and apoptosis of epidermal cells. The infiltrate is predominantly T lymphocytic. There may also be destruction of sebaceous glands.
  • Immunofluorescence studies, when performed, typically demonstrate evidence of immunoglobulin deposits at the basement membrane.

Treatment

  • Treatment to date has been largely symptomatic with no treatment regime showing great promise. The response to treatment therefore is disappointing with euthanasia being the outcome in many dogs.
  • Bryden and others (2006) reported on a series of 25 German short-haired pointers with ECLE in which various treatments were tried. High doses of glucocorticoids elicited a temporary response with frequent relapse. Eightyfive per cent of those dogs available for follow-up were euthanased.
  • Mauldin and others (2010) reported on six affected dogs in which immunomodulatory treatment with three drugs – ciclosporin, hydroxyquinone and adalimumab – was attempted. Of these hydroxyquinone was partially successful. None of the dogs could be described as cured.
  • In milder cases topical therapy with antiseborrhoeic shampoos may alleviate signs in conjunction with systemic drugs.

References and further reading

Bryden, S. L., White, S. D., Dunston, S. M. et al (2006) Clinical, histopathological and immunological characteristics of exfoliative lupus erythematosus in 25 German shorthaired pointers. Vet Derm 16 (4): 239- 252.

Mauldin, E. A., Morris, D. O., Brown, D. C. et al (2010) Exfoliative cutaneous lupus erythematosus in German shorthaired pointer dogs: disease development and evaluation of three immunomodulatory drugs (ciclosporin, hydroxyquinone and adalimumab) in a controlled environment. Vet Derm 21 (4): 373-382.

Miller, W. H., Griffin, C. E. and Campbell, K. L. (2013) In: Muller and Kirk’s Small Animal Dermatology, 7th ed. 461-462; Elsevier.