(SLE-like illness in patients with C1r, C2, & C4 deficiencies)
S8C13VA4-1:Two disorders associated with hypocomplementemia are listed.
Hypocomplementemia and Vasculitis
1. rare in Henoch-Schonlein purpura
2. arthritis
3. urticaria, angiocentric edema, purpura
4. abdominal pain +/_
5. glomerulonephritis +/-
S8C13VA4-2: Signs and symptoms of hypocomplementemic vasculitis are listed.
Fixed Infiltrative Angiitides
1. granuloma faciale (fixed)
a. fixed (locally chronic and active)
b. reticular dermis
c. pleomorphic infiltrate (perivascular)
d. fibrinoid or laminated hyalin
e. sebaceous glands
f. fibrosis
S8C13VA4-3: Granuloma faciale is an example of a fixed vasculitis with a relatively restricted anatomic distribution. It tends to localize in the same areas as those involved in the setting of rosacea.
Allergic (small vessel) Angiitis and Polyarteritis Nodosa
1. mild AA does not progress to severe PN
2. death (PN) - CVA, coronary thrombosis, renal failure
3. death (AA) - uremia or heart failure
4. corticosteroids more effective in AA than PN
5. PN - fever, weight loss, severe neuritis, asthma
6. AA - arthralgias, purpura, edema, dermatitis, GI bleeding, and renal failure
S8C13VA4-4: Small vessel angiitis (microscopic angiitis, or small vessel angiitis of Zeek) is more common than classic polyarteritis nodosa. The two are independent but, certainly, small vessel disease may be
encountered as an additional feature in the setting of classic polyarteritis nodosa.
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