Disease overview

Exfoliative Cutaneous Lupus Erythematosus, also known as Lupoid Dermatosis, is a rare early-onset autoimmune condition that affects the "basement membrane zone" of the skin. There is deposition of antigen-antibody complexes in the skin, which leads to an inflammatory response.

Gene name

UNC93B1

Inheritance

Autosomal recessive

Signs and symptoms

Affected dogs will present with signs early in life, typically before one year of age. These signs may come and go in increasing or decreasing severity and include skin lesions, lameness, scaling, reddening of the skin, ulceration and crusting, and joint pain. This is a severe disease that can be painful.

How is it diagnosed

A veterinarian will conduct a thorough exam, including a review of health history and any clinical signs. A skin biopsy is needed for a definitive diagnosis. Genetic testing is necessary to determine if there is an underlying genetic cause, which can assist veterinarians with diagnosis and help breeders identify affected and carrier dogs.

Breeds at higher risk

German Short-Haired Pointer, ,

Citations

Leeb T, Leuthard F, Jagannathan V, Kiener S, Letko A, Roosje P, Welle MM, Gailbreath KL, Cannon A, Linek M, Banovic F, Olivry T, White SD, Batcher K, Bannasch D, Minor KM, Mickelson JR, Hytönen MK, Lohi H, Mauldin EA, Casal ML. A Missense Variant Affecting the C-Terminal Tail of UNC93B1 in Dogs with Exfoliative Cutaneous Lupus Erythematosus (ECLE). Genes (Basel). 2020 Feb 3;11(2):159. doi: 10.3390/genes11020159. PMID: 32028618; PMCID: PMC7074252.

Wang P, Zangerl B, Werner P, Mauldin EA, Casal ML. Familial cutaneous lupus erythematosus (CLE) in the German shorthaired pointer maps to CFA18, a canine orthologue to human CLE. Immunogenetics. 2011 Apr;63(4):197-207. doi: 10.1007/s00251-010-0499-z. Epub 2010 Dec 4. PMID: 21132284; PMCID: PMC3230530.

Disclaimer

Disease diagnosis and treatment should always be performed by a veterinarian. The following information is for educational purposes only.