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Dermatomyositis (DMS) - Collie and Shetland Sheepdog

Dermatológico · Dog

Complex immune disease affecting the vasculature of skin and muscle in the Collie and Shetland Sheepdog (Sheltie). It produces scaling and erosive skin lesions in areas overlying bone (face, ears, limbs, tail tip) and, in a proportion of cases, myositis with muscle weakness. Onset usually occurs between 7-12 weeks and one year of age, often after a stressful trigger (vaccination, lactation, stress). Intensity is variable and some dogs improve with age.
Inheritance patternPolygenic with epistasis; the risk is modulated by three loci (PAN2, MAP3K7CL and the DLA-DRB1*002:01 haplotype).
Gene / MutationPAN2 p.Arg492Cys + MAP3K7CL c.383_392ACTCCACAAA>GACT + DLA-DRB1*002:01 (loci A, B and C)
PenetrancePenetrance modulated by the genotypic combination of the three loci: low risk (0-5 %), moderate (33-50 %) or high (90-100 %) depending on the combination. AABB genotypes with DLA-DRB1*002:01 homozygosity reach 100 % penetrance.
Codelmwb
Turnaround time15 days
Price61,11 €

Incidence

Characteristic of the Collie and the Shetland Sheepdog, with sporadic cases in related breeds (Beauceron, Corgi). Most purebred Collies are homozygous for DLA-DRB1*002:01; in the Sheltie, ~78 % carry at least one copy. The frequency of PAN2 and MAP3K7CL risk alleles is high in both breeds, but the actual clinical incidence is lower than the prevalence of risk genotypes.

Breeder management

- Test the three loci (PAN2, MAP3K7CL, DLA) before mating\n- Prioritise low-risk combinations; avoid matings of two individuals with PAN2/MAP3K7CL risk alleles, especially if both are homozygous for DLA-DRB1*002:01\n- Remember that the disease is complex: a 'high-risk' genotype does not equate to guaranteed disease, but it does mean a high probability\n- Progressively reduce PAN2 and MAP3K7CL risk alleles without removing DLA-DRB1*002:01 all at once (very widespread in the Collie) so as not to narrow the gene pool\n- In a dog with a high-risk genotype, monitor stressful triggers

Specialist notes

Definitive diagnosis requires a skin biopsy. The differential diagnosis includes demodicosis, dermatophytosis, pemphigoid, cutaneous lupus and drug reactions. Muscle involvement may be mild in the Sheltie and marked in the Collie. Treatments described: pentoxifylline, corticosteroids, vitamin E and oclacitinib in those over 12 months. Extensive lesions heal with irreversible atrophic alopecia. The risk genetics is complex: two 'high-risk' dogs may not develop the disease, and vice versa.

References

1. Evans JM et al. (2017). Beyond the MHC: a canine model of dermatomyositis shows a complex pattern of genetic risk involving novel loci. PLoS Genet 13(2):e1006604. PMID: 28158183
2. OMIA:000270-9615. Dermatomyositis in Canis lupus familiaris (dog).

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