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Congenital and Hereditary Skin Diseases


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Discuss the lesions of color mutant alopecia including the pathogenesis.
-Scaly, dry and brittle hair coat
-Papule may be present
-Puppies are usually normal at birth and later the skin becomes diseased
-There are defects in the melanization and cortical structures of the affected hair follicles
-Melanin clumps in the hair bulbs and shafts and makes it brittle
Describe your therapeutic plan for color mutant alopecia.
-There is no cure, just medical management (do not breed these animals)
-Use follicle flushing shampoos (benzoyl peroxide or ethyl lactate)
-Antibiotics for secondary infections
-Topical emollients and humectants
-Thyroid therapy if T4 is low
Describe a diagnostic plan to confirm a diagnosis of dermatomyositis.
-Skin biopsies (looking for liquefaction necrosis of basal cell layers of epidermis, vesicles)
-Muscle biopsies
-Skin scrapings
What are some breeds predisposed to dermatomyositis?
What are options for treatment for Dermatomyositis?
-Avoid trauma
-Vit. E
-Tetracycline and Niacinamide
-Spay and Neuter
Describe the pathogenesis of acral mutilation syndrome.
-It is a sensory neuropathy w/ decreased numbers of spinal ganglia and dorsal roots
-Occurs as a slowly progressive postnatal degeneration
Describe the pathogenesis of cutaneous asthenia.
-Cutaneous asthenia (a.k.a. Rubber puppy syndrome)
-Defect in collagen formation
-Increased fragility and laxity of skin
-In cats the deficiency is of procollagen peptidase
What breeds are predisposed to lethal acrodermatitis?
Bull Terriers
What are some clinical signs of lethal acrodermatitis?
-Growth retardation
-Progressive acrodermatitis
-Chronic pyoderma
-Chronic paronychia
-Abnormal behavior
-Lighter than normal skin pigmentation
What are some diagnostic options for lethal acrodermatitis?
-Breed predisposition
-PE findings
-Low serum zinc
-Decreased lymphocyte blast transformation
-Skin biopsy
-Parakeratotic hyperkeratosis w/ ulceration and superficial pyoderma
What is the prognosis for LETHAL acrodermatitis?
-Median survival 7 months
-Most puppies die of bronchopeumonias
What are the clinical signs of epidermal dysplasia?
-Lesions begin in puppyhood
-Pruritis (feet, ventrum at first and then becomes generalized)
-Seborrhea oleosa
-Peripheral lymphadenopathys
What breeds are predisposed to epidermal dysplasia?
West highland white terrier
What is the diagnostic plan to diagnose epidermal dysplasia?
-Rule out these diseases (atopy, food allergy, sarcoptes, demodex, ichthyosis, endcrinopathies, other seborrheic diseases)
-Skin biopsy: epidermal dysplaisa w/ hyperchromasia
-Increased mitosis, crowding of basal keratinocytes w/ “ buds”, parakeratosis
What is the treatment for Sebaceous adenitis?
-No cure
-Life long management
1. Keratolytic shampoo (Sialycylic acid)
2.Tetracycline +/- Niacinamide
3.Vit. A
4.Retinoids ($$$)
What is the predisposition for Sebaceous adenitis?
-Standard poodle (black and apricot)
What is the prognosis for Sebaceous adenitis?
-Trying to minimize symptoms and manage the disease (there is no cure)
How do you diagnose Sebaceous adenitis?
-Granulomatous sebaceous adentitis
-Chronic cases have complete absence of sebaceous glands
What is the treatment for ichthyosis?
-Life long (no cure)
-Topical antiseborrheias/keratolytic agants
-Propylene glycol
-Vit. A
How do you diagnose Ichthyosis?
-Rule out other causes of seborrhea
-Skin biopsy:
-Prominent granular layer
-Numerous mitotic figures in keratinocytes
-Orthokeratotic hyperkeratosis
-Follicular keratosis
Which breed of cats is predisposed to primary seborrhea oleosa?
What is follicular dysplasia?
What is pattern baldness?
What is Ehlers-Danlos syndrome?
-It is cutaneous asthenia (a.k.a.Rubber puppy syndrome)
-The skin hangs in loose folds
What is the normal skin extensibility index for dogs and cats?
Extensibilty index = (vertical height of skin fold)/(body length) * 100
-Normal 9-16% and Affected is 18-24 %
What is epidermolysis bullosa?
-Blisters and ulcerations at areas of trauma (footpad, mouth, anus, elbows)
How do you diagnose epidermolysis bullosa?
-Histopath: shows dermoepidermal vesicles and bullae
-Electron microscopy
What are the clinical signs of “Schnauzer Comedone Syndrome”?
-Multiple comedones on the back that become a crusted papule
What is the differential diagnoses need to be ruled out to confirm a diagnosis of Schnauzer Comedone Syndrome?
-Staph pyoderma

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