OMIA 001609-9615 : Exfoliative cutaneous lupus erythematosus in Canis lupus familiaris

Mendelian trait/disorder: yes

Mode of inheritance: Autosomal Recessive

Considered a defect: yes

Key variant known: no

Species-specific description: Exfoliative cutaneous lupus erythematosus is characterized by scaling and crusting skin lesions that progress over the entire body. There is a marker-based test available.

Inheritance: Wang et al. (2011) confirmed the autosomal recessive inheritance of this disorder in German shorthair pointers.

Mapping: By conducting a GWAS on 13 affected and 21 control German shorthaired pointers, each genotyped with the canine Affymetrix 127 k SNP v2 full set chip (yielding 64,987 informative SNPs for analysis), Wang et al. (2011) mapped this disorder to a 0.5 MB region of chromosome CFA18.

Clinical features: This is an exfoliating skin disease, with significant scaling and crusting that begin on the face, ears and back. Scaling progresses to involve the entire body. Secondary infections are common. Lesions can be scales, crusts, pustules, papules, erythema, and/or alopecia (Wang et al., 2011, Vroom et al., 1995, Mauldin et al., 2010). Most affected dogs present before 10 months of age, but signs can begin as late as 2.75 years. Dogs that live with this condition for several years may develop lupus nephritis. Many dogs show lameness and a hunched stance due to arthralgia. Dogs can also become infertile, with low sperm counts and abnormal estrous cycles (Wang et al., 2011, Mauldin et al., 2010).

Pathology: There is a lymphocytic to cell-poor interface dermatitis with vacuoles in the basement membrane region. Severe orthokeratotic hyperkeratosis is seen in the stratum corneum. IgG deposition occurs at the dermoepidermal junction, sometimes with concurrent IgM, IgA, or C3 deposition. Sebaceous glands can be normal, small, or absent. Reactive lymphoid hyperplasia is present in peripheral lymph nodes (Wang et al., 2011). Although these histologic changes can resemble those of systemic lupus erythematosus, discoid lupus erythematosus, and erythema multiforme, cutaneous lupus erythematosus generally begins at a much earlier age (Wang et al., 2011). This disease is more generalized than discoid lupus erythematosus and unlike erythema multiforme, apoptosis in the lower epidermis is absent (Vroom et al., 1995).

Prevalence: This is a rare disease among German shorthaired pointers (Vroom et al., 1995).

Control: Relatives of affected dogs should be tested. Breeding of affected or carrier dogs should be avoided.

Breeds: German Shorthair Pointer, Mixed breed.


Note: the references are listed in reverse chronological order (from the most recent year to the earliest year), and alphabetically by first author within a year.
2018 Olivry, T., Linder, K.E., Banovic, F. :
Cutaneous lupus erythematosus in dogs: a comprehensive review. BMC Vet Res 14:132, 2018. Pubmed reference: 29669547. DOI: 10.1186/s12917-018-1446-8.
2017 Banovic, F., Robson, D., Linek, M., Olivry, T. :
Therapeutic effectiveness of calcineurin inhibitors in canine vesicular cutaneous lupus erythematosus. Vet Dermatol :, 2017. Pubmed reference: 28439997. DOI: 10.1111/vde.12448.
2011 Wang, P., Zangerl, B., Werner, P., Mauldin, E.A., Casal, M.L. :
Familial cutaneous lupus erythematosus (CLE) in the German shorthaired pointer maps to CFA18, a canine orthologue to human CLE. Immunogenetics 63:197-207, 2011. Pubmed reference: 21132284. DOI: 10.1007/s00251-010-0499-z.
2010 Mauldin, E.A., Morris, D.O., Brown, D.C., Casal, M.L. :
Exfoliative cutaneous lupus erythematosus in German shorthaired pointer dogs: disease development, progression and evaluation of three immunomodulatory drugs (ciclosporin, hydroxychloroquine, and adalimumab) in a controlled environment. Vet Dermatol :, 2010. Pubmed reference: 20374572. DOI: 10.1111/j.1365-3164.2010.00867.x.
2005 Bryden, S.L., White, S.D., Dunston, S.M., Burrows, A.K., Olivry, T. :
Clinical, histopathological and immunological characteristics of exfoliative cutaneous lupus erythematosus in 25 German short-haired pointers. Vet Dermatol 16:239-52, 2005. Pubmed reference: 16101795. DOI: 10.1111/j.1365-3164.2005.00468.x.
1995 Vroom, M.W., Theaker, M.J., Rest, J.R., White, S.D. :
Lupoid dermatosis in five german short-haired pointers Veterinary Dermatology 6:93-98, 1995.
1992 Theaker, A.J., Rest, J.R. :
Lupoid Dermatosis in a German Short-Haired Pointer Veterinary Record 131:495, 1992. Pubmed reference: 1471334.

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  • Created by Frank Nicholas on 28 Sep 2011
  • Changed by Frank Nicholas on 28 Sep 2011
  • Changed by Vicki Meyers-Wallen on 29 Sep 2011
  • Changed by Frank Nicholas on 29 Sep 2011
  • Changed by Frank Nicholas on 21 May 2013