Spontaneous autoimmune subepidermal blistering diseases in animals: a comprehensive review.

Autoimmune subepidermal blistering diseases (AISBDs) are rare skin disorders of animals that were first identified in dogs but several AISBDs are now recognised in other companion animal species. Most AISBDs in animals are homologues of the human diseases and are thought to share similar pathomechanisms of epidermal and/or mucosal blister formation caused by autoantibodies targeting structural proteins of the basement membrane zone (BMZ). Disruption of their structural function by the autoantibodies and/or recruited inflammation leads to BMZ fragility, which presents clinically as vesicles, bullae and, later, deep erosions and ulcers. Canine AISBDs are the best characterised, particularly the more common variants such as mucous membrane pemphigoid (48%), epidermolysis bullosa acquisita (EBA) (26%), and bullous pemphigoid (10%). Exceedingly rare AISBDs in the dog are junctional EBA, mixed AISBD, type-1 bullous systemic lupus erythematosus, linear IgA dermatosis, and pemphigus gestationis. The diagnosis of a specific AISBD is made by combining the clinical features (breed, age, lesion distribution) with histological evidence of subepithelial clefting, but not all AISBDs can be differentiated in this manner and specialised immunological testing is required. This latter, unfortunately, is not readily available and, therefore, the specific AISBD diagnosis often remains unconfirmed. While this limits further understanding of these diseases, it does not prevent clinicians from treating their patients, as the treatment approaches are similar for the different AISBDs in dogs. This review primarily focuses on canine AISBDs, the species for which these diseases have been best characterised, and shorter descriptions of variants in other species are also provided.

Correlation between pruritus score and grossly visible erythema in dogs.

The severity of pruritus and the extent and severity of erythema were quantified in 107 dogs presenting with various dermatoses. Pruritus was assessed using a previously validated scale, and erythema was quantified by assessing severity at 72 different body sites. Pruritus scores were either 0, or followed a normal type of distribution, with most dogs having a score in the middle of the range and a few dogs having low or high scores. The median pruritus score was 6.3/10. Erythema scores were heavily skewed towards lower values, with only a few dogs having high scores. The median diffuse erythema score was 6.0/216 and the median score for maculo-papular/pustular erythema was 0/1080. Pruritus and erythema scores were significantly correlated with a Spearman rank correlation coefficient of 0.4062 (P<0.001). However, visual assessment of the data representing the two variables revealed that this was not a consistent biological or clinically relevant correlation. Individual dogs could have a high pruritus score with low erythema score or vice versa. This study raises questions about the use of erythema scoring systems as a primary outcome measure in clinical trials, and also about the role of various inflammatory mediators in the pathogenesis of canine pruritus.

Sarcoptic mange in three alpacas treated successfully with amitraz.

Sarcoptic mange is a serious skin disease in alpacas that can result in high morbidity and even mortality. Three alpacas were presented with sarcoptic mange that had previously failed to respond to repeated topical applications of eprinomectin, and an injection of doramectin. They were moderately to severely pruritic, had extensive lesions of alopecia, erythema, scaling and crusting, and had lost weight. As no drug is currently licensed for the treatment of sarcoptic mange in alpacas in the UK, they were treated with a topical solution of amitraz (50 mL in 10 L) after initial bathing with antibacterial or keratolytic shampoos. The clinical signs completely resolved with no relapse over a 10-month follow-up period. In this small group of alpacas, amitraz was an effective and well-tolerated treatment for sarcoptic mange.

Suspected polymyxin B-induced pemphigus vulgaris in a dog.

A case of pemphigus vulgaris (PV), putatively induced by topical application of polymyxin B ear drops, is described. A 3-year-old, female Tosa Inu, presented with acute onset swelling, blistering and ulceration of the pinnae, nostrils, lips and oral mucous membranes. The dog was depressed, febrile and anorexic. For 7 days prior to the onset of the acute ulcerative disease, polymyxin B ear drops had been applied to both ears to treat an ear infection. Skin and mucosal biopsies showed suprabasilar cleft formation and acantholysis, indicative of PV. The polymyxin B ear drops were discontinued and the dog was treated with intravenous fluids, systemic and topical antibacterial therapy, and immunosuppressive therapy comprising prednisone and azathioprine. Complete remission was noted after 2 weeks, and the immunosuppressive therapy was discontinued one month later. No clinical signs of PV recurred over a 1 year follow-up period. As PV does not usually resolve spontaneously, or enter long-term remission, it was considered that the condition was most likely drug induced due to the aural application of polymyxin B.