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*Affects puppies of collies and shetland sheepdogs from 8 weeks of age
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Also known as: '''Canine familial dermatomyositis'''''
*Lesions - vesiculating dermatitis
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**Face, lips, external ears, later distal extremities
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*Microscopically: interface dermatitis and basal cell degeneration of epidermis and follicular wall, atrophy of follicles, epidermal vesicles and pustules, dermal scarring
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*Hereditary in Collies and Shetland sheepdgs
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==Introduction==
*Lesions resemble [[Canine Polymyositis|canine polymyositis]]
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Canine familial dermatomyositis is a '''hereditary inflammatory disease of skin and muscle''' characterised by symmetrical scarring alopecia of the face and limbs and atrophy of the muscles of mastication.
*Also degeneration of blood vessels -> muscle [[Muscle Ischaemia|microinfarcts]]
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*Usually generalised but temporalis and other masticatory muscles may be involved the most
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*Microscopically: inclusion bodies in endothelial cells
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*Best place for biopsy is anterior and most superficial temporalis muscle
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*Also involves skin, may involve the oesophagus
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It affects '''Shetland Sheepdogs''' and '''Collies''' from as early as '''7 weeks of age''' and has an autosomal dominant mode of inheritance with variable expressivity. It has also been known to occur in other breeds such as the Chow Chow and Corgi.
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The aetiopathogenesis of the condition is unknown. It is thought that '''immunological damage occurs to blood vessels''' resulting in ischaemic damage to the skin and muscles.
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==Clinical Signs==
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Lesions have usually developped by '''6 months of age''', but they can also occur in adults.
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The rate of development of lesions is very variable, and the disease may '''wax and wane''' and may undergo spontaneous regression.
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Typical lesion distribution is the '''face''' (bridge of nose, tips of ears and around the eyes), '''carpal and tarsal areas''', '''digits''' and '''tip of the tail'''.
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There is '''scarring alopecia, erythema, scaling and crusting'''.
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'''Myositis''' is a feature of the disease and its severity correlates with the severity of the skin lesions. There is commonly temporal and masseter muscle atrophy.
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Severely affected dogs may present with '''dysphagia''', may have growth retardation, '''megaoesophagus''', lameness and widespread muscle atrophy.
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The condition is not usually pruritic nor painful.
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==Diagnosis==
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A clinical diagnosis can be made based upon the presentation, history and clinical signs.
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Other common causes of follicular dermatitis in young dogs, such as dermatophytosis, demodecosis and pyoderma, should be '''ruled out''' with skin scrapes, cytology and culture.
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'''Skin biopsies''' can provide supportive evidence, and there is hydropic degeneration of basal cells, intrabasalar or subepidermal clefting, pigmentary incontinence, follicular atrophy and possibly vasculitis.
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'''Muscle biopsies''' reveal inflammatory exudates, muscle fibre necrosis and muscle atrophy. The best place for a biopsy is the anterior and most superficial temporalis muscle.
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'''EMG''' can be performed and will reveal abnormalities consisting of positive sharp waves, fibrillation potentials and bizarre high-frequency discharges of affected muscles.
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==Treatment==
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It is difficult to determine the effectiveness of a treatment as lesions can '''wax and wane''' on their own.
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Mild lesions may not need treatment and may '''spontaneously resolve'''.
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'''Pentoxifylline''' is the treatment of choice and it improves circulation and decreases levels of inflammatory mediators. 2-3 months may be needed before any benefits are seen.
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'''Vitamin E''' can lead to an improvement in the skin lesions, but not in muscle.
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'''Prednisolone''' can be given during acute flare-ups but prolonged use is discouraged as it can contribute to muscle atrophy.
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As the condition is heritable, affected animals should '''not be used for breeding'''.
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==Prognosis==
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'''Complete resolution is rarely possible''' and treatment only minimises the development of new lesions and lessens the severity of those present. Scarring and muscle atrophy may never resolve. Animals may suffer from secondary infections and aspiration pneumonia from megaoesophagus. This means prognosis can be '''difficult to predict'''.
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Mild cases generally have a '''good prognosis''' and may resolve spontaneously.
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{{Learning
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|flashcards = [[Small Animal Orthopaedics Q&A 05]]
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}}
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==References==
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Moriello, K. (2005) '''Self-assessment colour review of small animal dermatology''' ''Manson Publishing''
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Harvey, R. (2009) '''A colour handbook of skin diseases of the dog and cat''' ''Manson Publishing''
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[[Category:To Do - Helen]]
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[[Category:To Do - Review]]
    
[[Category:Integumentary System - Autoimmune Reactions]]
 
[[Category:Integumentary System - Autoimmune Reactions]]
 
[[Category:Muscles - Developmental Pathology]]
 
[[Category:Muscles - Developmental Pathology]]
 
[[Category:Immune-Mediated Myositis]]
 
[[Category:Immune-Mediated Myositis]]
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