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Background: Ehlers Danlos Syndrome (EDS) is a rare genetic disease characterized by a deficiency in collagen synthesis,which can result in joint laxity. Patellar luxation is one of the main orthopedic conditions that affect the canine knee joint,with limited descriptions of its association with EDS in dogs. The purpose of this report is to describe the surgical management and postoperative evolution of a 1-year-old Chow Chow dog with grade II patellar luxation, tibial valgus and EDS.Case: A 1-year-old Chow Chow dog was referred to the University Veterinary Hospital due to lameness of the left pelvicfor 3 months. At the orthopedic examination were verified severe lameness and lateral deviation of the left stifle joint during the ambulation of the animal. Additionally, it was verified bilateral hyperextension of the tibiotarsal joint and grade IIpatellar luxation of both pelvic limbs with painful hyperextension of the left stifle joint. Radiographic evaluation showedlateral displacement of the patella from both femoral trochlear groove, and a valgus deviation of the proximal left tibialshaft. In addition, it was verified cutaneous hyperextensibility and an extensibility index suggestive of EDS. The animalwas submitted to trochlear block resection technique and medial imbrication, followed by corrective tibial osteotomy.Furthermore, skin biopsies of the scapular and lumbar folds were performed during the corrective tibial osteotomy. Thesamples were sent for histopathological examination, which revealed fragmented and unorganized collagen fibers in thedermis. Histopathological findings were compatible with EDS. The absence of lameness and correct positioning of thepatella in the trochlear sulcus were verified in the post-surgical follow-up. Complete bone consolidation of the closingwedge osteotomy to correct the tibial...(AU)

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RESUMO

Background: Ehlers Danlos Syndrome (EDS) is a rare genetic disease characterized by a deficiency in collagen synthesis,which can result in joint laxity. Patellar luxation is one of the main orthopedic conditions that affect the canine knee joint,with limited descriptions of its association with EDS in dogs. The purpose of this report is to describe the surgical management and postoperative evolution of a 1-year-old Chow Chow dog with grade II patellar luxation, tibial valgus and EDS.Case: A 1-year-old Chow Chow dog was referred to the University Veterinary Hospital due to lameness of the left pelvicfor 3 months. At the orthopedic examination were verified severe lameness and lateral deviation of the left stifle joint during the ambulation of the animal. Additionally, it was verified bilateral hyperextension of the tibiotarsal joint and grade IIpatellar luxation of both pelvic limbs with painful hyperextension of the left stifle joint. Radiographic evaluation showedlateral displacement of the patella from both femoral trochlear groove, and a valgus deviation of the proximal left tibialshaft. In addition, it was verified cutaneous hyperextensibility and an extensibility index suggestive of EDS. The animalwas submitted to trochlear block resection technique and medial imbrication, followed by corrective tibial osteotomy.Furthermore, skin biopsies of the scapular and lumbar folds were performed during the corrective tibial osteotomy. Thesamples were sent for histopathological examination, which revealed fragmented and unorganized collagen fibers in thedermis. Histopathological findings were compatible with EDS. The absence of lameness and correct positioning of thepatella in the trochlear sulcus were verified in the post-surgical follow-up. Complete bone consolidation of the closingwedge osteotomy to correct the tibial...

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Background: Cutaneous asthenia or Ehlers-Danlos syndrome is an inherited and rare disease. This infirmity is from an autosomal mutation that influences the collagen synthesis of the carrier. Thus, its skeleton, formed of fibers, is structurally defective. The disease is characterized by hyperelasticity and skin fragility, leading to lesions throughout the skin. The lesions may manifest in specific places or in a generalized way, being more frequent in the limbs, neck, and back. This disease does not have a specific treatment, only management care to avoid new traumas. Case: A 3-year-old male castrated, no defined race cat, was attended at one veterinary clinic with a history of intense itching. The rapid tests for feline immunodeficiency virus (FIV) and feline leukemia virus (FeLV) were negative. On physical examination, infestation by fleas, lesions all over the body, and skin hyperelasticity were observed. Topical treatment for ectoparasites as well as for body wounds was established. After the treatment, he returned without itching, but with the complaints of new lesions that did not heal. The patient underwent a total shearing to facilitate the treatment of the skin, and then he underwent blood tests, biochemistry, hormonal tests, and ultrasound, which showed no suggestive changes for hyperadrenocorticism and diabetes mellitus. His clinical signs, besides not matching with these diseases, also did not indicate skin fragility due to his history of age and balanced diet. The confirmation of the cutaneous asthenia syndrome was acquired through biopsy of skin fragment, in which it was observed disarray of collagen fibers, hypertrophy, and fibroblast hyperplasia, together with the rate of extensibility of the skin where the value reached the mark of 27.5%. Throughout the hospitalization, it was noted the progression of the disease with the appearance of new lesions, where there was no bleeding and they appeared even with the patient wearing padded clothes. Its progression lasted one year until the patient's euthanasia. Discussion: For the disposal of diabetes mellitus and hyperadrenocorticism as causes of the appearance of lesions by the body in the patient, he was submitted to the suppression tests with dexamethasone, in which he presented normality, in the biochemical examination it was dosed with fructosamine and glucose. The fructosamine was in the reference value, but the glucose was slightly altered, this increase may have been a result of stress at the time of blood collection. In addition to the patient not showing specific clinical signs such as polyphagia, polyuria, polydipsia, and weight loss, these are characteristic clinical signs of the disease. For the diagnosis of Ehlers-Danlos syndrome, histological examination, and calculation of the skin extensibility index were used, where the results obtained confirmed the suspicion. This genetic anomaly has no treatment, being progressive, so only environmental management is done to mitigate the appearance of the lesions and provide animal welfare. This case report contributes to aggregating the scientific literature in the area of veterinary medicine since skin asthenia is a rare disease and when its extent is total it becomes even more atypical. The availability of this article will provide a vision of palliative treatment for other cases, demonstrating the progressive nature of the lesions and the methods of diagnosis.

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Dermatosparaxis is a genetic disease that affects collagen maturation. This disease is characterized by marked impairment of the resistance of collagen fibers that leads to skin fragility, and it may affect several species. This is the first report of dermatosparaxis in sheep in Brazil. Clinically, two White Dorper lambs had multiple skin lacerations in the neck, dorsum and limbs. Transmission microscopy demonstrated irregular collagen fibers arranged in hieroglyphic shape and scanning electron microscopy demonstrated thin collagen fibrils that were not arranged in bundles as observed in the normal dermis. These findings are consistent with the diagnosis of dermatosparaxis.

A dermatosparaxis é uma doença genética que afeta a maturação do colágeno. Essa doença é caracterizada por redução acentuada da resistência das fibras colágenas que leva à fragilidade da pele. Esse é o primeiro relato de dermatosparaxis em ovinos no Brasil. Clinicamente, dois cordeiros da raça White Dorper tiveram múltiplas lacerações na pele do pescoço, dorso e membros. A microscopia de transmissão demonstrou fibras de colágeno irregularares arranjadas em formato de hieroflifo, e a microscopia de varredura demonstrou fibras finas de colágeno não arranjadas em bandas como na derme do animal normal. Esses achados são consistentes com o diagnóstico de dermatosparaxis.

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Dermatosparaxis is a genetic disease that affects collagen maturation. This disease is characterized by marked impairment of the resistance of collagen fibers that leads to skin fragility, and it may affect several species. This is the first report of dermatosparaxis in sheep in Brazil. Clinically, two White Dorper lambs had multiple skin lacerations in the neck, dorsum and limbs. Transmission microscopy demonstrated irregular collagen fibers arranged in hieroglyphic shape and scanning electron microscopy demonstrated thin collagen fibrils that were not arranged in bundles as observed in the normal dermis. These findings are consistent with the diagnosis of dermatosparaxis(AU)

A dermatosparaxis é uma doença genética que afeta a maturação do colágeno. Essa doença é caracterizada por redução acentuada da resistência das fibras colágenas que leva à fragilidade da pele. Esse é o primeiro relato de dermatosparaxis em ovinos no Brasil. Clinicamente, dois cordeiros da raça White Dorper tiveram múltiplas lacerações na pele do pescoço, dorso e membros. A microscopia de transmissão demonstrou fibras de colágeno irregularares arranjadas em formato de hieroflifo, e a microscopia de varredura demonstrou fibras finas de colágeno não arranjadas em bandas como na derme do animal normal. Esses achados são consistentes com o diagnóstico de dermatosparaxis(AU)

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Background: Localized Scleroderma (morphea-like) is a rare disease in dogs and extremely rare in cats and it is an uncommon chronic connective tissue disease. There are only two reported cases of cats, dating from 1998 and 2012 and the cause is unknown. The clinical progression of the disease is unpredictable, but usually features moderate and self-limiting course. In the previously reported cases of cats, there was a clinical cure. The objective of this case report is to describe a case of Localized Scleroderma (morphea-like) in a cat that had historical and clinical progression of the disease in a distinct way from the two previously reported cases mentioned. Case: A 4 years old male cat, neutered, which belongs to the Persian breed, was presented with a history of chronic pruritus and alopecic lesions, without involving other organs. The lesions appeared with no history of trauma neither medications applied. Several treatments have been performed, but the only response found was a clinical improvement of pruritus. The presence of bruises and sores on the skin has never been observed, even before any treatment. Three lesions were observed in a specific clinical examination and they were all with the same pattern: the lesions were circular, irregular, alopecic, with peripheral halo slightly hyperemic and raised, and the lesion center with smooth, thin, opaque white skin...(AU)

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Background: Localized Scleroderma (morphea-like) is a rare disease in dogs and extremely rare in cats and it is an uncommon chronic connective tissue disease. There are only two reported cases of cats, dating from 1998 and 2012 and the cause is unknown. The clinical progression of the disease is unpredictable, but usually features moderate and self-limiting course. In the previously reported cases of cats, there was a clinical cure. The objective of this case report is to describe a case of Localized Scleroderma (morphea-like) in a cat that had historical and clinical progression of the disease in a distinct way from the two previously reported cases mentioned. Case: A 4 years old male cat, neutered, which belongs to the Persian breed, was presented with a history of chronic pruritus and alopecic lesions, without involving other organs. The lesions appeared with no history of trauma neither medications applied. Several treatments have been performed, but the only response found was a clinical improvement of pruritus. The presence of bruises and sores on the skin has never been observed, even before any treatment. Three lesions were observed in a specific clinical examination and they were all with the same pattern: the lesions were circular, irregular, alopecic, with peripheral halo slightly hyperemic and raised, and the lesion center with smooth, thin, opaque white skin...

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Hereditary collagen dysplasias comprise a complex group of connective-tissue disorders that result in the reduced tensile strength of affected tissues. These processes are called cutaneous asthenia in the skin of dogs and cats. We report here the case of a crossbred male cat, aged 6 months, that presented with two skin wounds in the region of the right thorax and right iliac tuberosity. The skin of these regions and of the animal's dorsum was hyperextensible, smooth to the touch, and easily torn with minor trauma. Microscopic examination of skin samples revealed reduced dermal connective tissue consisting of shortened and fragmented collagen fibers. Normal fibers were intermingled with altered fibers. Ultrastructural changes in collagen fibers included disorientation of fibrils within the same bundle, marked spacing differences, and variation in the diameter of transverse sections. The fibrils maintained the transverse striations characteristic of normal collagen.

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