1
Q
What is the definition of vasculitis
A
Inflammation that occurs initially and primarily in the vascular wall
2
Q
What can cutaneous vasculitis represent
A
- A manifestation of a systemic vasculitis
- A skin-limited or skin-predominant variant of a systemic vasculitis
- A single-organ vasculitis with no detectable systemic involvement
3
Q
Which vessels can be affected
A
- Small vessels = arterioles, capillaries and post capillary venues , found in the superficial and mid dermis of the skin
- Medium-sized vessels = small arteries and veins reside within the deep dermis or subcutis
- Large vessels = aorta and its branches
4
Q
With involvement of which vessels does cutaneous involvement occur
A
Small & Medium sized
5
Q
What is Leukocytoclastic vasculitis
A
A histopathologic term for vasculitis involving the small post capillary venules of the dermis with neutrophilic inflammation
6
Q
What I the classification of vasculitis based on the Chapel Hill Consensus
A
- Large vessel vasculitis
- Medium vessel vasculitis
- Small vessel vasculitis : divided into two major subtypes
A.ANCA-associated vasculitis
B.Immune-complex vasculitis
4.Variable vessels vasculitis - Single organ vasculitis
- Vasculitis associated with systemic disease
- Vasculitis associated with probable aetiology
7
Q
What is the pathogenesis of small vessel vasculitis - Immune Complex Mediated
A
- Immune complexes form in the presence of antigen excess –> antigen + antibody ( IgG,IgM & IgA in IgA vasculitis)
- Immune complexes deposit in the post-capillary venules of the dermis
- These complexes lead to complement-mediated chemotaxis of neutrophils
- Neutrophilc migrate to the vessel wall and release - proteases, myeloperoxidase and reactive oxygen species
- Vessel wall damage with leakage of erythrocytes –> Palpable Purpura
- During this process, neutrophils break down and their nuclear fragments remain in the tissue, those fragments are called Leukocytoclasia ( histological Hallmark of small-vessel vasculitis)
8
Q
What is the pathogenesis of ANCA-associated small vessel vasculitis
A
No immune complex deposition
The key mechanism is = Autoantibody mediated neutrophil activation
1. Formation of ANCA ( antineutrophilic cytoplasmic antibodies) autoantibodies
- c-ANCA –> against Proteinase 3
- p-ANCA –> against Myeloperoxidase (MPO)
- Primary cytokine activation ( release of inflammatory cytokines by innate immune cells after a trigger, such as infection…) & release of TNF alpha, IL1,6,8..
- Make neutrophils partially activated or primed –> in that state, the proteins that are normally hidden inside neutrophil granules (intracellular proteins -PR3,MPO) move toward the cell surface
- Because these neutrophil proteins are now abnormally exposed during inflammation, the adaptive immune system start treating them as foreign
- Soo B cells start producing antibodies against PR3 or MPO –> these antibodies are called ANCA
- ANCA circulates in the blood and binds to the primed neutrophils
- These binding causes the neutrophil to become fully activated
- Activated neutrophils stick to vessel walls –> degranulate, release reactive oxygen species ,proteolytic enzyme –> damage to the endothelium and vessel wall
9
Q
why is ANCA -associated vasculitis, also called pauci-immune vasculitis?
A
Because the vessel damage in ANCA-positive vasculitides is directly mediated by neutrophils rather than by immune complexes, they are referred to as “pauci ( few or very little) immune” vasculitides.
Pauci-immune vasculitis refers to vasculitis with minimal or absent immune-complex deposition on immunofluorescence.
10
Q
What is the most common clinical lesions and the reason behind them of CSVV?
A
- Small vessel involvement = accounts for the size of the lesion
- The inflammatory response = for the palpability and symptomatology ( burning, pain, pruritus)
- The red blood cell extravasation from damaged blood vessels = for the non-blanching Purpura
- The effect of gravity on immune complex deposition = for the distribution of lesions on the lower legs and other dependent areas
11
Q
Why in small vessel vasculitis ( especially immune-complex), lesions typically appear on dependent areas , particularly the lower legs
A
- Increased Hydrostatic Pressure
- when standing the lower extremities experience higher venous pressure due to gravity and higher pressure causes slower blood flow and more contact between circulating immune complexes and vessel walls - Slower Blood Flow ( venous stasis)
- In dependent areas, circulation is slower and immune complexes remain longer in the microvasculature - Increased vascular permeability
- Small vessels in dependent areas are more prone to endothelial stress and leakage of proteins
12
Q
What is the Cutaneous Small Vessel Vasculitis (CSVV)
A
A type of single organ, skin-limited vasculitis that involves primarily the dermal postacapillary venues and its histologically chatacterized by LCV
13
Q
What is the etiology of CSVV
A
- Idiopathic 50% of cases
- Infection 15-20%
- Inflammatory disorders 15-20%
- Drug exposure
- Neoplasms
14
Q
Which are the inflammatory diseases associated with CSVV
A
- Autoimmune connective tissue diseases
- RA; SLE ; Sjogren syndrome ; overlap SLE/Sjogren - IBD
- Behcet
4 Rare = Sarcoidosis ; Cystic fibrosis; Primary biliary cirrhosis; HS;
15
Q
Which are the infectious causes of CSVV
A
- Bacterial
- Beta-hemolytic Strep group A ; Mycobacterium TB/Leprae ;
- Rare : Mycoplasma pneumonia - Viral
- Upper Respiratory tract infection; Hepatitis C > B > A + vaccines
- HIV; Parvovirus 19
- Rare = Cytomegalovirus; VZV; Influenza + vaccine
16
Q
Which are the most common inflammatory disorders a/w CSVV
A
Autoimmune connective tissue
diseases
- Rheumatoid arthritis
- SLE
- Sjögren syndrome
- Overlap SLE/Sjögren
syndrome
Less common:
- Inflammatory bowel disease
- Behçet disease
- Hypergammaglobulinemic purpura of Waldenström
- Sarcoidosis
- Cystic fibrosis
- Primary biliary cirrhosis
- Bowel-associated dermatosis arthritis syndrome
- Gluten enteropathy
-Hidradenitis suppurativa
- Systemic sclerosis
- Dermatomyositis
17
Q
Which are the most common drugs a/w CSVV
A
- Antibiotics, esp. β-lactams
Penicillins
Cephalosporins, esp. cefaclor
Sulfonamides
Minocycline
Quinolones
Macrolides - Cardiovascular
Thiazides
Hydralazine§
Quinidine - Other
Allopurinol
Bortezomib
D-penicillamine
G-CSF
NSAIDs
Propylthiouracil/other
antithyroid agents§
Serum (e.g. ATG)
Streptokinase
18
Q
Which are the most common malignancies a/w CSVV
A
Plasma cell dyscrasias
* Monoclonal gammopathies
* Multiple myeloma
Myelodysplasia
Myeloproliferative disorders
Lymphoproliferative disorders
Hairy cell leukemia
19
Q
What is the pathogenesis of CSVV
A
Immune-complex deposition in walls of capillaries and venules
20
Q
What are the clinical features of CSVV
A
- Presents spontaneously or after exposure to an inciting agent ( drug= onset 1-3 wks after initiation ) , with a crop of lesions consisting of non-blanching ( do not disappear with diascopy ) purpuric macule and papules; petechia; urticarial lesions or hemorrhagic vesicles ranging in size from 1mm-several cm
-Occasionally - pustules, targeted lesions and round ulcerations are seen - Usually asymptomatic , but lesions can be associated with burning pain or pruritus
- Residual postinflammatory hyperpigmentation can persist for months after the primary process resolves
21
Q
What are the primary clinical features of small vessel vasculitis?
A
Palpable purpura, petechiae, vesicles, pustules
22
Q
Which are the areas of the skin usually affected
A
- CSVV favours dependent areas ( legs most commonly ) + areas affected by trauma ( Koehler phenomenon) + under tight-fitting clothing
- Prolonged sitting or standing, exercise ( walking or hiking in hot weather) can induce CSVV on the lower extremities
23
Q
What are the systemic sx
A
- occurs in 5-25% of patients
- arthralgias, arthritis , genitourinary sings, gastrointestinal involvement
24
Q
What is the histology of CSVV
A
Perivascular neutrophilic infiltrate w/ leukocytoclasis ( nuclear debris from fragmented neutrophils)
centered around post-capillary venules w/ fibrinoid
necrosis of vessel walls and endothelial swelling, and
RBC extravasation
NB! Concomitant involvement of the deeper larger vessels → suggests systemic vasculitis
25
When should the biopsy be taken
within 18 to 48 hours; DIF best even
earlier (within 8–24 hours)
26
DIF findings?
80% w/ perivascular C3 and IgM in first 48 hours;
after 72 hours, only C3 in minority
27
what is the tx of CSVV
1.Supportive care ( as it often resolves without any sp tx) and elimination of potential triggers
- Rest, leg elevation, compression, high potency steroid creams
2. Severe , ulcerating or progressive cutaneous disease
- Systemic steroids 0.5-1mg/kg daily tapered over 4-6 weeks
3. 2nd line = Cochicine 0.5 mg twice or three times per day or Dapson 50-150 mgday
28
What is IgA vasculitis ( Henoch-Schonlein Purpura)
- Type of vasculitis affecting small blood vessels ( mainly post-capillary venules at papillary plexus ) with vascular IgA deposition
- More common in children ( 5 yrs)
29
What is the classic tetrad seen in IgAV
Palpable purpura + Arthritis + Abdominal pain + Hematuria
30
What is the etiology of IgAV
- Presents 1-2 wks following an upper respiratory tract infection ( Streptococcal tonsillitis) especially in children ( this explains the seasonal pattern, with a peak occurrence during winter )
- Other infections: Bartonella henselae, Parvovirus B19, S. aureus, H. pylori, and Coxsackievirus
- May be triggered by medications, like antibiotics used to tx the URT infection
31
What is the pathophysiology of IgAV
- Deposition IgA1 immune complexes in post-capillary venules --> skin; mesangium of the glomeruli; small vessels of the intestinal submucosa/laminate propria ; small vessels of synovium
- Leading to complement activation and neutrophilic vascular damage
32
What is the tetrad of the clinical presentation of Henoch-Sconlein Purpura?
Skin: Palpable purpura on the Buttocks and lower extremities (100)
Musculoskeletal: arthralgias (75%), arthritis of the knees and ankles
GI: Colicky abdominal pain (65%), diarrhea, vomiting, hematochezia
Renal (40-50%): hematuria , proteinuria , w/ risk of nephritis --> end-stage renal failure seen in 1-3%
33
What is the histopathology of IgA
Leukocytoclastic vasculitis
DIF = IgA deposits, C3, and fibrin in dermal small blood vessels
34
What is the tx of IgA vasculitis
Most cases are self-limited so tx is supportive
Elastic bandages on the legs and antihistamines can
reduce the deposition of immunoglobulins in mild
phlebolymphedema by suppressing vascular dilatation and
increasing blood flow
If signs of marked systemic involve-
ment occur in Henoch–Schönlein purpura, such as arthralgia
or severe abdominal pain, systemic glucocorticoids (1–2 mg/
kg bodyweight/day per os or parenteral methylprednisolone
0.8–1.6 mg/kg bodyweight/day)
35
Tell me about Acute Hemorrhagic Edema of infancy
- Form of vasculitis seen in infants between 4 - 24 months of age ( children < 2 years of age )
- Cutaneous lesions usually begin as a plaques with variable degrees of haemorrhage and favour the head and extremities, which subsecuently become oedematous and targetoid in appearance
36
What is the aetiology
- unknown, but up to 85% of pt have an associated infection, drug exposure or immunisation
37
what are the clinical features of AHEI
- clinically child does not appear ill, but fever is present most of the tie
- Sudden onset of large erythematous patches or urticarial lesions that evolve into annular or targeted purpuric plaques ( favours cheeks, ears and extremities)
- Lesions may be asymptomatic, painful or less commonly pruritic
- Arcuate / polycyclic/ scalloped or rosette - shaped lesions and vesicobullae occur less commonly = heal with atrophic scars
- Tender, non-pitting edema o the face, ears, extremities ( + hands and feet) and scrotum are typical
38
Tx
supportive
39
What is urticarial vasculitis
Its an entity consisting of persistent urticarial lesions ( >24 hours) that demonstrate the histopathologic features of LCV
40
what are the 2 major subtypes
1. Normocomplementemic vasculitis = normal complement levels ; usually limited to the skin ; systemic sx rare being course and self-limited
2. Hypocomplementemic urticarial vasculitis = more severe form; low complement level ( C3/C4) ; high risk of systemic disease ; a/w autoimmune connective tissue diseases (SLE & Sjogren)
41
What are the clinical features
Erythematous , indurated wheals, with or without angioedema
Favours trunk & proximal extremities
42
how is urticarial vasculitis distinguished from chronic urticaria
Lesions > 24 hours
Burning pain, rather than pruritus
Presence of purpura and/or postinflammatory hyperpigmentation
43
What are the specific diagnostic criteria for hypocomplementic urticarial vasculitis syndrome
2 major:
1. Urticaria > 6mo
2. Hypocomplementemia
PLUS
2 or more minor
1. Vasculitis on skin biopsy
2. Arthralgia or arthritis
3. Uveitis
4. Glomerulonephritis
5. Recurrent abdominal pain
6. positive C1q
precipitin test with a low C1q level.
Patients with hypocomplementemia who do not meet the criteria for
HUVS are considered to have hypocomplementemic urticarial vascu-
litis but not HUVS.
44
What are the most common abnormal laboratory findings
elevated ESR, low serum C3 and C4 levels, and
a positive ANA
45
TX
Antihistamines may reduce the swelling and pain associated with cutaneous lesions but do not alter the course of the disease
Oral corticosteroids
46
General pathology of ANCA-associated small vessel vase ulitivasculititied
Characterised by :
- vasculitis of small to medium sized vessels
- + ANCAs
- overlapping spectrum of organ involvement
47
what type of antibodies are ANCAS
predominantly IgG autoantibodies directed against the components of neutrophilic granules
1. Cytoplasmic (c) ANCAs - directed against the antigen proteinase 3 ( PR3)
2. Perunuclear (p) ANCAS -directed against the antigen myeloperoxidase (MPO)
48
in what other conditions can this autoantibodies be found
1. Certain medications - propylthiouracil, hydralazine
2. Underlying chronic infections
3. Rheumatologist or inflammatory disorders and malignancies
49
What are the ANCA- associated small vessel vasculitis
1. Cytoplasmic ANCA (cANCA) = granulomatosis with polyangitis ( Wegener granulomatosis) ; eosinophilic granulomatosis with polyangitis (Churg-Strauss syndrome)
2. Perinuclear ANCA ( pANCA) = Microscopic polyangitis
50
What is Wegener granulomatosis
Described as the triad of granulomatous inflammation of the upper and lower respiratory tracts, systemic necrotising vasculitis and pauci-immune ( non immune complex-mediate vasculitis) glomerulonephritis
51
what is the etiology of wegeners
unknown, its speculated that an infectious agent triggers the immune response agains the PR3
52
What are the systemic findings in WG
Biphasic course
1. Early phase
- localised inflammation of the sinuses or upper airways, with rhinitis sinusitis , otitis media
2. Later, the pt. develops all the features of systemic vasculitis with fever, malaise, weight loss and multi-organ disease
Hallmark --> necrotic ulcerative granulomas:
- upper airways with tissue destruction, including saddle nose and septal perforation
- Pulmonary infiltrates a/w chest pain, hemoptysis and cavitary change
- Renal disease - focal segmental glomerulonephritis
- Ocular - conjunctivitis, episcleritis , dacryocystitis
53
What are the cutaneous findings
Non specific
- Urticarial and maculopapular exanthems; may ulcerate
-Purpura and pyoderma-gangrenosum like lesions
- Subcutaneous nodules
- Papulonecrotic lesions are common and usually occur on extensor surfaces, particularly the elbows = so times referred to a Churg-Strauss nodules
- Oral mucosal ulcerations, especially on hard palate
54
What is the clinical triad seen in granulomatosis w/ polyangiitis?
Necrotizing granulomas of the upper and lower respiratory tract: cough hemoptysis, SOB,
Nasal/sinus inflammation: rhinorrhea, sinusitis, and purulent or bloody nasal discharge
Systemic vasculitis
Musculoskeletal: arthralgias
Ocular: conjunctivitis, proptosis, and keratitis
CNS: peripheral neuropathy and CVA
Glomerulonephritis
- Death from renal disease if left untreated (>80% 1
year mortality)
55
What are the most common skin findings in granulomatosis w/ polyangiitis?
Palpable purpura in dependant areas
Oral ulcers common, gingival hyperplasia w/ strawberry gums is less common but near pathognomonic
Can have “pyoderma-like” nodules or necrotic ulcers
Cutaneous dz can be a/w wearlier onset and more widespread dz
56
What is the histology of granulomatosis w/ polyangiitis?
LCV + palisading granulomas of the dermis, with giant cells, fibrin and degenerated collagen
57
What laboratory abnormalities can be seen in patients w/ granulomatosis with polyangiitis?
Can see an increase in ESR and WBC, can also see c-ANCA (sensitivity up to 90% and specificity 80-100%)
Abnormal UA: microscopi hematuria or RBC casts
Abnormal CXR: nodules, infiltrates, and cavities often found
Sinus involvement: abnormal sinus X-ray, CT sinus or nasal bx
58
What ist he treatment for granulomatosis with polyangiitis?
Prednisone (1mg/kg/day) + cyclosphosphamide (2mg/kg/day)
Maintence: MTX (20-25mg/kg) and or oral steroids, azothioprine (2mg/kg/day)
59
What is microscopic polyangitis
vasculitis of small sized blood vessels, most commonly affecting the skin, lungs and kidneys
>90% of pt are ANCA + ( specifically p-ANCA ) with no granulomatous inflammation ( difference between Wegener )
60
what are the cutaneous finings
20-70% of pt have cutaneous involvement
- Most commonly = petechia and purpura
- Livedo reticular and Livedo racemosa are seen more often in MPA than GPA go EGPA
61
What are the most common systemic sx’s seen with microscopic polyangiitis?
Renal (70-90%): focal segmental necrotizing glomerulonephritis
Pulmonary (25-50%): pulmonary capillaritis ( with dyspnea and pulmonary infiltrates) , can result in diffuse alveolar haemorrhage
Neurological (up to 33%): Mononeuritis multiplex, peripheral neuropathy
62
What is the specific antibody seen in microscopic polyangiitis most commonly?
+ANCA –> specific antibody will be Anti-MPO most commonly (60%)
63
What is the histology of microscopic polyangiitis?
LCV w/ segmental small > medium vessel vasclulitis
No granuloma formation, unlike WG or Churg-Strauss syndrome
64
Treatment for microscopic polyangiitis?
nduction:
Cyclophosphamide (2 mg/kg per day) + oral steroids
(1 mg/kg per day)
Rituximab + cyclophosphamide
Remission:
-MTX or azathioprine, similar to WG
Localized
TMP-SMX + oral steroids
65
What is Eosinophilic granulomatosis with polyangitis ( Churg-Strauss syndrome ) characterised by
Vascular and extravascular granulomas + eosinophil-rich pulmonary infiltrates + necrotising vasculitis of small-medium vessels within multiple organ systems
66
What is it associated with
Asthma and eosinophilia
67
What are the various triggering factors in CSS
vacation , leukotriene inhibitors, omalizumab , rapid discontinuation of steroids
68
What are the 3 classic clinical phases of Churg-Strauss syndrome?
1. Prodromal phase - sx of adult onset asthma , allergic rhinitis, nasal polyps , may persist for years
2. Eosinophilic phase - peripheral eosinophilia and eosinophilic infiltration ( Loffler infiltrates ) of internal organs, especially lungs and GI tract ( N/V, abdominal pain)
3. Vasculitis phase - systemic necrotizing vasculitis with granulomatous inflammation, can occur several years to decades after the initial sx
- 2 major internal organs: Heart ( coronary artieries and myocardium ) & Nervous system
- neurologic: mononeurtiis multiplex, symmetric polyneuropathy
- cardiac: pericarditis, valvular disease, endocardiomyopathy
69
What are the clinical presentations ( cutaneous findings) of Churg-Strauss Syndrome?
Palpable purpura on the lower extremities, painful symmetric subcutaneous nodules of the extremities and scalp
70
What is the histology of Churg-Strauss Syndrome?
LCV w/ mixed infiltrate of eosinophils, neutrophils, lymphocytes, and macrophages
Palisading neutrophilic and eosinophilic extravascular granulomas with degenerated collagen fibers (“red granulomas”)
71
What laboratory findings can be seen in Churgg-Strauss Syndrome?
+ ANCA (linked to neurologic and renal dz)
- ANCA (more linked to cardiac dz)
- p-ANCA > C-ANCA in leukotriene-associated dz
- Eosinophilia (eos > 1500)
- Leukocytosis
- Increased IgE
- CXR: patchy infiltrates, interstitial dz, and nodular masses
72
Treatment for Churg-Strauss syndrome?
Oral steroids (1 mg/kg a day)
Internal organ involvement:
- Cyclophosphamide (2 mg/kg a day) + oral steroids
73
What is the primary cause of death in Churg-Strauss syndrome?
Endocardiomyopathy
74
which vessels are affected in Polyarteritis Nodosa
medium-sized blood vessels
75
what is the etiology of PN
- most cases are idiopathic
- Associated with:
1.Infections = HBV & HCV; Streptococcus ( esp in children) ; HIV/AIDS
2. Inflammatory diseases = IBD
3. Malignancies = hairy cell leukemia
4. Medications = minocycline
76
what are the 2 forms of PAN
1. Classic PAN = systemic dz w/ multi-system vasculitis
2. Cutaneous PAN = mainly skin-restricted variant with limited systemic involvement ( occasional muscle or nerve involvement)
77
What are the clinical features of Classic PAN
- Long-prodromal period = weight loss , fever, malaise, arthralgia
- Kidney involvement ( >70%) = hypertension & renal failure ; no glomerulonephritis ( as interlobar renal arteries are involved )
- GI tract ( 50%) = ulcerations, bowel perforation, ischemic colitis & enteritis , GI bleeding
- Neurologic = peripheral neuropathy, dysesthesias, ischemic injury, cranial nerve paralysis psychoses, seizures
- Cardiac dz = uncommon, coronary artery damage or conduction defects
- Orchitis !!! = unilateral, very characteristic for PAN
- 1/3 have cutaneous findings = very variable; most commonly Livedo-racemosa -like changes often with ulcerations
78
What is the clinical presentation in Cutaneous PAN
Skin predominant form of the disease, represents about 10% of cases
- Livedo-racemosa ( starburst pattern) and painful tender subcutaneous nodules , which can ulcerate on the lower extremities
79
What are the laboratory findings in systemic PAN
- Leukocytosis, elevated ESR , thrombocytosis, eosinophilia
-Abdominal and renal angiography
80
What is the histology of PAN?
1. Inflammation of the arteries of the deep dermal plexus, initially with fibrinoid necrosis and later with dense neutrophilic and eosinophilic infiltrates and thrombosis
2. Later in the stage both granulation tissue and fibrosis
81
what is the tx of systemic PAN
Prednisone 1-2 mg/kg/day 4 wks than reduction
If renal insufficiency, new or increasing hypertension or other symptomatic arterial stenoses (on the extremties, heart, gastrointestinal, CNS) or aneurysms are added, an
additional immunosuppressant is required --> Cyclophosphamide 2mg/kg/dat
82
Tx for Cutaneous PAN
Cutaneous subtype:
Intralesional steroids, NSAIDS, and oral steroids for 3 o 6 months if severe skin involvement
Children: consider penicillin, given the association with streptococcal infection
83
What is the definition of of Kawasaki disease ( mucocutaneous lymph node syndrome )
Acute disease of infancy involving a high fever + mucous membrane lesions + exanthema + painful cervical lymphadenopathy
84
What is the epidemiology of Kawasaki dz?
Mostly seen in kids <5 y/o; M>F
Increased incidence in Japanese
85
what is the etiopathogenesis
unknown
sudden onset and fever point towards an infection - with likely suspects being Staph and Strep toxins
86
What are the clinical findings
- Starts with fever up to 40C, febrile convulsions and marked lymphadenopathy, with no response to antibiotic tx
- 2-3 days later, conjunctival injection (dilation and congestion of the blood vessels of the conjunctiva - red eye) + photophobia and swollen cracking lips
- Tongue is with enlarge inflamed papillae ( like in scarlet fever )
By the end of the 1st week all sx disappear EXCEPT Swollen lips
- >90% develop widespread rash, on day 3-5 of the illness
- 1st sign: erythema of the palms and soles --> rash spreads to involve the trunk and extremities
- Lesions are red macules, that may coalesce
- May remain maculopapular or evolve into --> urticarial plaques ; scarlatinifrom macules with prominent flexural involvement; truncal target lesions (EM like)
- Very characteristic is Desquamative scarlatiniform perianal rash
- After 2 weeks the classic Acral Desquamation occurs, as large sheets of skin are shed, beginning just beneath the tip of the nails !!!!
- Almost all pt develop Beau line or horizontal nail furrows, reflecting damage to the nail matrix
87
What systemic complications can be seen Kawasaki dz?
Cardiac: rythm disturbances, heart failure, valvular dz, sudden death from coronary artery occlusion or aneurysm
Musculoskeletal: arthritis/arthralgias
Pulmonary: pneumonitis
CNS: aseptic meningitis and facial nerve palsies
Ophtho: anterior uveitis
GI: gastroenteritis, hepatomegaly, bile duct inflammation/hepatitis, jaundice, and pancreatitis
88
Laboratory findings
Thrombocytosis - correlates with the highest risk of coronary disease
Elevated neutrophil and eosinophil count
89
What must be serially checked in Kawasaki’s dz?
Echocardiogram and ECG, at diagnosis, 2,6 and 8 weeks
Coronary artery occlusion or aneurysm appears after day 10;
The earlier sign is thickening of the coronary arteries seen on echo
Cardiac problems appear as the fever, rash and other signs and sx have resolved
90
Treatment of Kawasaki’s dz?
High-dose Aspirin (80–100 mg/kg a day) + IVIG (2 g/kg)
-If given within first 10 days leads to a decreased rate of coronary artery issues
Resistant cases: IVIG + steroids, cyclophosphamide, cyclosporine/CIs, plasma exchange, TNF-α inhibitors, MTX, rituximab, and anakinra
Maintenance: aspirin
91
What is the epidemiology of Temporal arteries ( Giant cell arteritis )
elderly 70 years
females
92
