Test Detail

Autoimmune skin disease of the dog characterised by generalised exfoliative dermatitis and, frequently, onychomadesis (detachment of the claws). The lesions are of the cutaneous lupus erythematosus type, with interface dermatitis on histology. It appears in adulthood, is chronic and usually progresses. The predisposed breeds are the German Shorthaired Pointer and the Vizsla, which suggests a hereditary basis.

Predisposed breeds with documented variant (OMIA001609-9615): German Shorthaired Pointer, Vizsla and Braque du Bourbonnais; carriers have also been found in German Longhaired Pointers. There are no reliable published figures for incidence or carrier frequency.

- A genetic test for the UNC93B1 variant is available: test breeding animals of predisposed breeds.\n- Do not mate two carriers: 25 % risk of affected homozygotes.\n- A carrier may be mated to a clear animal; test the offspring intended for breeding.\n- Withdraw affected animals from breeding.\n- Document the pedigree of the cases and inform the buyer of offspring from affected lines.

Differential diagnosis with other exfoliative dermatitides (primary seborrhoeic, zinc-responsive, generalised demodicosis, leishmaniosis, endocrinopathies) and with pemphigus foliaceus. Biopsy showing interface dermatitis and immunofluorescence/immunohistochemistry compatible with lupus are decisive; onychomadesis points towards the suspicion. The UNC93B1 genetic test allows confirmation of the diagnosis in predisposed breeds and guidance of breeding. Symptomatic treatment with immunomodulators (glucocorticoids, fatty acids, etc.) according to dermatological judgement, with variable response.

1. Wang P, Zangerl B, Werner P, Mauldin EA, et al. Familial cutaneous lupus erythematosus (CLE) in the German shorthaired pointer maps to CFA18, a canine orthologue to human CLE. Immunogenetics 2011. PMID:21132284.
2. Leeb T, Leuthard F, Jagannathan V, Kiener S, et al. A Missense Variant Affecting the C-Terminal Tail of UNC93B1 in Dogs with Exfoliative Cutaneous Lupus Erythematosus (ECLE). Genes (Basel) 2020. PMID:32028618.
3. OMIA:001609-9615 Exfoliative cutaneous lupus erythematosus (Canis lupus familiaris).