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Epidermolysis bullosa (EB) is a hereditary mechanobullous disease of animals and humans, characterized by an extreme fragility of the skin and mucous membranes. The main feature of EB in humans and animals is the formation of blisters and erosions in response to minor mechanical trauma. Epidermolysis bullosa is caused by mutations in the genes that code for structural proteins of the cytoskeleton of the basal keratinocytes or of the basement membrane zone. Based on the ultrastructural levels of tissue separation, EB is divided into the following three broad categories: epidermolysis bullosa simplex, junctional epidermolysis bullosa and dystrophic epidermolysis bullosa. Human types of EB are divided into several subtypes based on their ultrastructural changes and the mode of inheritance; subtypes are not fully established in animals. In humans, it is estimated that EB affects one in 17,000 live births; the frequency of EB in different animals species is not known. In all animal species, except in buffalo with epidermolysis bullosa simplex, multifocal ulcers are observed on the gums, hard and soft palates, mucosa of the lips, cheek mucosa and dorsum of the tongue. Dystrophic or absent nails, a frequent sign seen in human patients with EB, corresponds to the deformities and sloughing of the hooves in ungulates and to dystrophy or atrophy of the claws in dogs and cats. This review covers aspects of the molecular biology, diagnosis, classification, clinical signs and pathology of EB reported in animals.

RESUMOCom este trabalho teve-se como objetivo avaliar os parâmetros fisiológicos (freqüência respiratória, freqüência cardí-aca, movimentos ruminais e temperatura retal) de ovinos das raças Dorper, Santa Inês e seus mestiços F 1 , submetidos às condições climáticas do trópico semi-árido nordestino. Foram utilizados 48 ovinos, sendo 16 animais de cada um dos três grupos gené-ticos (Dorper, Santa Inês e mestiço F 1 Dorper x Santa Inês), dos quais 8 do sexo masculino e 8 do sexo feminino. Utilizou-se o delineamento experimental inteiramente casualizado num esquema fatorial de 3 x 2 x 2 (3 grupos de animais, 2 sexos e 2 turnos de coletas). Das três fontes de variações consideradas: sexo, genótipo e turno, a última exerceu efeito significativo (P<0,05) em todos os parâmetros fisiológicos analisados, sem interagir com sexo e genótipo, enquanto o genótipo interagiu com o sexo (P<0,05) em relação à freqüência cardíaca e à respiratória. Concluiu-se que o turno foi o fator que mais influenciou nas respostas dos ovinos ao ambiente térmico, independentemente de genótipo e sexo. O turno da tarde conduziu os animais à situação de perigo, em termos de desconforto térmico, e à condição de elevado estresse calórico. Para a freqüência cardíaca, os machos F1 e as fêmeas Dorper e para freqüência respiratória, os machos e fêmeas Dorper e os machos F1 foram os mais estressados, o que demonstra menor adaptabilidade do genótipo exótico e de seus mestiços. Termos para indexação:Adaptabilidade, estresse térmico, raça, freqüência respiratória, temperatura retal. ABSTRACTThe goal of this work was to evaluate physiological parameters (respiratory and cardiac frequencies, rumen movement and rectal temperature) in Dorper, Santa Inês and Dorper x Santa Inês crossbreed (F1) sheep submitted to typical weather conditions (tropic semi-arid) of Brazilian northwest. Were used 48 sheep, 16 animals from each of the three genetic groups (Dorper, Santa Inês and crossbreed), among them 8 were male and 8 were female. The experimental design was completely randomized in a 3 x 2 x 2 factorial scheme (3 genetic groups, 2 genders and 2 collecting shift). From the three variations sources considered, gender, genotype and shift, the last had a significant (P<0.05) effect on all physiological parameters evaluated without interaction with gender (P<0.05) in relation to respiratory and cardiac frequencies. It was concluded that the shift was the factor that had the higher influence on the sheep s answer to temperature environment, independently of genotype and gender. Evening shift conducted animals to a dangerous situation, in terms of thermal uncomfortable and heat stress. Considering the cardiac frequency of F1 males and Doper female and the respiratory frequency of Dorper males and females and of F1 males, they were more stressed. It demonstrated the reduced capacity of the exotic genotype and their crossbreed for adaptation.

Epidermolysis bullosa (EB) is a hereditary mechanobullous skin disease of animals and human beings, characterized by marked skin and mucous membrane fragility, which triggers the formation of blisters and ulcers in response to minor trauma. The lesions occur due to insufficient connections at the dermis-epidermis junction, due to defects at the basement membrane zone. 4,6 In addition to the formation of blisters, there is a loss of nails, claws, or hooves, and detachment of the epithelium of the oral mucosa. 7,8 The different EB types in domestic animals follow human classification, based on the ultrastructural lesion at the skin basement membrane zone. There are 3 types of EB: epidermolysis bullosa simplex (EBS), characterized by cytolysis of basal keratinocytes producing intraepidermal clefts; junctional epidermolysis bullosa (JEB), in which the separation occurs in the lamina lucida; and dystrophic epidermolysis bullosa (DEB) with cleavage within or below the lamina densa.3 Most types of EB are caused by autosomal recessive genes and appear with low frequency. It is estimated that, in human beings, the disease affects 1 in 17,000 live births in the entire world population. 8In animals, the frequency of EB is not estimated, but there are few reports of the disease. The aim of the current study was to report the clinical, histological, and ultrastructural aspects of a case of EB in a 2-month-old calf, examined in April 2010, at the Veterinary Hospital of the Federal University of Campina Grande (HV/UFCG), Paraíba, Brazil. The calf belonged to a herd of 18 animals. It was descendent from a Gir bull and a Gir crossbreed cow. According to the farmer, a male calf (sired by the same bull) with similar lesions died previously on the farm.The calf had exungulation of all hooves, widespread erosions, and crusts on the skin (Fig. 1A, 1B) and ulcers in the oral cavity (Fig. 1C). The epidermis loosened easily when a little pressure was exerted on the intact skin (positive Nikolsky sign 8 ). Due to the severity of clinical signs and unfavorable prognosis, the animal was euthanized and necropsied. Varying degrees of alopecia, erosions, and crusts were observed on the skin of the metacarpal and metatarsal regions, elbows, knees, ventral thorax, and abdomen and gluteal region. There were also multifocal ulcers on the gums, hard and soft palates, mucosa of the lips, cheek mucosa, and dorsum of the tongue. The other organs showed no significant lesions.Tissue samples from liver, spleen, kidney, adrenal, lymph nodes, thymus, lung, heart, skeletal muscles, thyroid, central nervous system, esophagus, fore stomachs, abomasum, large and small intestine, oral mucosa, tongue, and skin were fixed in 10% buffered formalin, embedded in paraffin, and stained with hematoxylin and eosin, periodic acid-Schiff, and Gomori trichome. Histologically, there was subepidermal separation (Fig. 2), forming blisters filled occasionally with clear eosinophilic fluid, cellular debris, or neutrophils. Epidermal cells showed degeneration evolving to necro...

The aim of this study was to elucidate the cause of a neurological syndrome characterized by stridor in adult goats with clinical signs of copper deficiency. The main clinical signs consisted of apathy, emaciation, pale mucous membranes, mucous nasal discharge, dyspnea, severe achromotrichia, diffuse alopecia, torpor, ataxia, and stridor. When the goats were forced to move, the stridor increased. In a herd of 194 Toggenburg goats, 10 adult goats with clinical signs of copper deficiency were removed from the herd and divided into 2 groups: group 1, which consisted of 4 nannies and 1 buck with stridor, and group 2, which consisted of 4 nannies and 1 buck without stridor. Group 3, used as a control, consisted of 5 adult goats from another flock without any clinical signs of disease. The mean serum copper concentrations were 1.3 ± 0.3 μmol/L in group 1, 8.1 ± 1.1 μmol/L in group 2, and 11.3 ± 2.2 μmol/L in group 3. The mean serum iron concentrations were 42.3 ± 14.2 μmol/L in group 1, 39.1 ± 8.2 μmol/L in group 2, and 20.6 ± 6.1 μmol/L in group 3. The main histological lesions in goats from group 1 were axonal degeneration of the recurrent laryngeal nerves and atrophy of the muscles of vocal folds and of the dorsal cricoarytenoid and right and left cricothyroid muscles. Goats with ataxia had neuronal degeneration and necrosis of cerebellar Purkinje cells and of the cranial cervical ganglion. We concluded that the stridor was caused by axonal degeneration of the recurrent laryngeal nerves due to the severe copper deficiency.