Vasculitis, Ischemic Dermatopathy, Panniculitis Flashcards by Treasa Clifford

Breeds with familial vasculitis (all in puppies)

1) Beagles (familial necrotizing arteritis). ANCA positive.

2) GSD Familial Cutaneous Vasculopathy. Autosomal recessive. 2’ vaccination? Foot pads, nasal bridge. Self recover, but relapse with future vax.

3) Jack Russel Terrier Familial Cutaneous Vasculopathy. Vaccination? Ulcers, alopecia on boney prominences, pinnae, footpads.

4) Scottish Terrier Hereditary Vasculitis (pyogranuloma and vasculitis of the nasal planum); Autosomal dominant. Ulcer of nasal planum.

5) Shar Pei Vasculitis (life threatening! Idiopathic. Fever, hemorrhagic papules, deep ulcers, epidermal detachment. Tx GC)

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Cryoglobulinemia causes

*Lead poisoning
*Upper respiratory infections
*Neoplasia (Multiple Myeloma, Lymphoma)
*Idiopathic

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Systemic signs of cryoglobulinemia

*IMHA (IgM triggers it)
*Glomerulonephritis
*Thrombosis

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Diagnosing cryoglobulinemia

*Immune complexes will be more severe in cold blood (refrigerator) and dissolve in warm temperature

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Urticarial vasculitis causes

*Drugs
*CAFR
*Insects
*Cold
*Idiopathic

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How to discern regular urticaria vs urticarial vasculitis

Vasculitic form will NOT blanch on diascopy

Normal urticaria WILL blanch on diascopy

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Neonatal vasculitis pathogenesis

Foal ingesting anti-neutrophil and anti-platelet antibodies via colostrum –> forms immune complexes in their body (<4 days old) –> start bleeding.

Tx with GCs for 2 weeks. Usually a good prognosis.

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Solar vasculitis skin color

Unpigmented, lightly pigmented

Often coexists with fDLE, vitiligo

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Traction alopecia causes

Owners putting on cosemetic to tie the hair back (Rubber bands, Barrettes)

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Traction alopecia lesions

Alopecia, thinning, scar –> well demarcated

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Traction alopecia histopath

Follicular atrophy Deep arteritis (hyalinization of vessel wall)

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Traction alopecia treatment

No self cure. Neglect vs pentoxifylline vs surgery

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Cutaneous and Renal Glomerular Vasculopathy (AKA “alabama rot”: Breed

Racing greyhounds in USA

Many breeds in UK

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Cutaneous and Renal Glomerular Vasculopathy (AKA “alabama rot”: etiology

E coli Shiga toxin (verotoxin) in raw beef

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Cutaneous and Renal Glomerular Vasculopathy (AKA “alabama rot”: seasonality

Winter and summer in USA
Nov-May in the UK

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Cutaneous and Renal Glomerular Vasculopathy (AKA “alabama rot”: pathogenesis

Thrombotic microangiopathy –> ischemia

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Cutaneous and Renal Glomerular Vasculopathy (AKA “alabama rot”: clinical signs

Acute, with rapid deterioration

Edema, erythema –> hemorrhage, ulcers

HINDlimbs, footpads, mucosae

25% get acute kidney injury AKI. Usually AKI happens AFTER skin lesions.

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Cutaneous and Renal Glomerular Vasculopathy (AKA “alabama rot”: Labwork findings

THROMBOCYTOPENIA (d/t consumption of platelets due to massive thrombosis)

AKI signs

Proteinuria, casts

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Cutaneous and Renal Glomerular Vasculopathy (AKA “alabama rot”: treatment

If only skin lesions– self cure slowly, goof prognosis

Severe AKI: usually poor prognosis –> euthanize

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Non-immune mediated causes of vasculitis (8)

1) Direct invasion of vascular wall (microorganisms, neoplasia)
2) Embolism (septic embolism)
3) Irradiation (UV)
4) Heat (thermal burns)
5) Water (equine immersion foot syndrome)
6) Trauma, irradiation (IV catheter)
7) Toxins
8) Hemodynamic factors (turbulence)

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Immune mediated causes of vasculitis (4)

1) Immune complexes
*Antigen-antibody complexes –> complement (C3a, C5a, MAC) –> neutrophils –> granule contents, ROS released –> vasculitis

2) Anti-neutrophil cytoplasmic antibodies (ANCA)
*Ab binds to neutrophil –> activation –> granule contents, ROS released –> vasculitis
*Usual ANCA targets = neutrophil proteinase 3 & Myeloperoxidase

3) Anti-endothelial cell antibodies

4) T cells (CD8 autoreactive cells, NK cells), macrophages (ie FIP, Leishmania)

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Main causes of vasculitis, general

Infection**
**Drug
*Immune-mediated dz
*CAFR
*Neoplasia
*Environmental factors (contact, heat, cold)

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Proliferative (dermal) arteritis of the nasal philtrum: Breed

Saint Bernard

*Basset hound
*Bloodhound
*Dobie
*GSD
*Other large/giant breeds

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Proliferative (dermal) arteritis of the nasal philtrum: Age

Mean 5 yr

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Proliferative (dermal) arteritis of the nasal philtrum: Cause

Idiopathil Familial predisposition in Saint Bernard

Proliferative (dermal) arteritis of the nasal philtrum: Clinical signs

Circular ulcer on nasal philtrum 1/3 of dogs will have PERFUSE bleeding from the lesion

Proliferative (dermal) arteritis of the nasal philtrum: Histopathology

Proliferative arteritis (deep arteries, arterioles)-- Thickening of tunica media/intima: myofibroblasts that produce collagen/mucin around the arteries Tunica interna: elastic lamina failure

Proliferative (dermal) arteritis of the nasal philtrum: Treatment

Prednisone, tacrolimus

Proliferative thrombovascular necrosis of the pinna: treatment

Oclacitinib!

Proliferative (dermal) arteritis of the nasal philtrum: etiology

Idiopathic!! Potentially vaccination, fenbendazole

Proliferative (dermal) arteritis of the nasal philtrum: Predisposed pinnae

Pendulous, folded ears >> Erect ears

Proliferative (dermal) arteritis of the nasal philtrum: differential diagnoses

*Leishmania *Bartonella henselae *Frostbite *Cryoglobulinemia-Cryofibrinogenemia *Familial vasculopathies (GSD, JRT) *Ischemic dermatopathies

Ischemic Teat Necrosis (cows): etiology

Unknown. Usually during 1st lactation cycle of cow LOSS OF TEATS- necrosis, self trauma

Types of Ischemic Dermatopathy (5)

1) Familial dermatomyositis 2) Dermatopmyositis-like in atypical breeds, Juvenile-onset ischemic dermatopathy 3) Post-rabies vaccine vasculitis and panniculitis 4) Generalized vaccine-associated ischemic dermatopathy 5) Adult-onset generalized idiopathic ischemic dermatopathy

Ischemic dermatopathy: histopath

Cell poor interface dermatitis *Basal cell: Hydropic degeneration, basal cell apoptosis. Suprabasilar vesicles, ulcers Pigmentary incontinence *Hair follicles: Hydropic degeneration, apoptosis --> atrophy, fibrosis *Dermis: Collagen pallor, mucinosis, edema, fibrosis *Blood vessels: vasculitis (occasional), endothelial loss, mural hyaline changes

Ischemic dermatopathy differential diagnosis on histopath

Lupus

Familial dermatomyositis: breed

Collie (rough), Shetland sheepdog

Familial dermatomyositis: age

<6 months old for skin Older for myositis

Familial dermatomyositis mode of inheritance

NOT mendelian inheritance (used to be thought autosomal dominance. But MANY genes!)

Familial dermatomyositis: genes

Risk alleles: *PAN2 *MAP3K7CL polymorphisms *MHC cl II haplotypes

Familial dermatomyositis: environmental factors

*Estrus *Sun exposure *Trauma *?? virus, vaccine, drugs, toxins, stress

Familial dermatomyositis: antibody quantity

*Increased total IgG *Increased circulating immune complexes (w/ IgG)

Familial dermatomyositis: complement related?

No difference in complement quantities in dogs But in humans, complement deficiency is important for dermatomyositis

Familial dermatomyositis: clinical lesions, distribution

Alopecia, erythema, dyspigmentation, ulcers, scars, scale Skin, footpads, nails; oral mucosa

Familial dermatomyositis: which muscles are involved

Masseter > skeletal Atrophy Dysphagia Regurgitation Stiff gait

Familial dermatomyositis: nail lesions

*Onychorrhexis *Onychoschizia *Onychomadesis

Which breed has more myositis? Collies or Shelties?

Shelties

Familial dermatomyositis: progression of disease

*Skin lesions worst at 1 year old *Wax/wane clinical signs (stressful periods) *May spontaneously cure, or PR

Familial dermatomyositis: how to assess muscle involvement

*CK levels (only elevated if acute flare of myositis) *Electromyography *Muscle biopsy (atrophy, necrosis, fibrosis, mixed inflammation)

Familial dermatomyositis: treatment

*Avoid triggers (sun, trauma; spaying if estrus trigger) *Topical GC, tacrolimus *Systemic GC, cyclosporine, MMP, pentoxifylline, vit E, OFA, doxycycline/niacinamide *Apoquel (works for other ischemic dermatopathies!) --> inhibition of Type 1 IFNs (which are a major cytokine for dermatomyositis)

Non-familial ischemic dermatopathies (Juvenile, adult onset, vaccine-associated [local, generalized]): signalment

*SMALL DOGS > MEDIUM DOGS *64% of dogs are <10kg!! *Age: all *Chihuahua *Toy/Mini poodle *Maltese *Chinese crested *Fox terrier *Jack russel terrier *Yorkshire terrier *Schipperke *Rat terrier

Non-familial ischemic dermatopathies (Juvenile, adult onset, vaccine-associated): vaccine-associated signalment

*Toy poodle *Young dogs *Larger size dogs

Non-familial ischemic dermatopathies (Juvenile, adult onset, vaccine-associated): localized vaccine-associated timeline

2-3 months (up to 8 mo) after rabies vaccine *IgG and Rabies Antigen can be found in vessels and hair follicles!!!

Non-familial ischemic dermatopathies (Juvenile, adult onset, vaccine-associated): Generalized vaccine-associated timeline

*up to 5 months after localized lesion (but don't NEED a localized lesion first) *C5b-9 membrane attack complex in muscle vessels

Sterile nodular panniculitis breeds

Dachshunds Poodles Australian shepherds Bristtany spaniels Chihuahuas Dalmation Pomeranians

Body regions for sterile nodular panniculitis

Neck, trunk

Other systemic signs that may be related to sterile nodular panniculitis

IMPA

Histo patterns of panniculitis

*Nodular >>> *Septal *Diffuse *Intralobar

Treatment for sterile nodular panniculitis

*GC + 2' immunosuppressive agent *Needed lifelong Only 15% achieve CR with GC monotherapy

Most common disease associated with cutaneous vasculitis in horses

Photo-aggravated dermatitis (non-pigmented skin, normal liver enzymes, no exposure to photosensitizing agents) BUT previous studies indicate Purpura hemorrhagica (2' Streptococcus equi or Corynebacterium pseudotuberculosis). SQ limb edema; MM hemorrhage.

Breed with higher rate of cutaneous vasculitis in horses

Paints Pintos (more unpigmented skin)

Signalment with LOWER rates of cutaneous vasculitis in horses

Thoroughbreds Stallions

Most commonly affected site for cutaneous vasculitis in horses

Legs (crust, scale, edema)

What clinical sign is indicative of a poorer prognosis in horses with cutaneous vasculitis

Fever

T or F: Vasculitis can occur 2' PF and equine sarcoidosis

True. Antigenic stimulation cause those 2 diseases. If antigen is similar to vascular endothelium-- vasculitis will occur OR Antigenic stimulation causes antigen-antibody complex deposition

What type of hypersensitivity reaction occurs to trigger purpura hemorrhagica in horses

Type 3. Antibody-antigen complexes that deposit in vessel walls

Labwork findings in horses with cutaneous vasculitis

Anemia Neutrophilia Hyperglycemia Hyperglobulinemia (2' inflammation, stress, GC administration)

T or F: Cutaneous vasculitis is likely to recur in horses

True. 44% have recurrence

Most common treatments for cutaneous vasculitis in horses

TMS Corticosteroids Pentoxifylline (antiinflammatory, rheological)

Dermatomyositis genes

MAP 3K7CL PAN2

Factors associated with worse prognosis for ischemic dermatopathy in dogs

*<10kg *increased age *More lesion sites *Systemic signs *Pinnae, paw pad lesions