Urticaria Flashcards
Rare disorder comb of chronic non pruritic urticaria, fever of unknown origin, disabling bone pain, hyperostosis, increased ESR, IGM.
CHED-FM acronym
Schnitzler syndrome
schnitzler syndrom gender
age onset
Biopsy.
no gender predilec
29-77 yo.
Neutrophilic perivascular and interstitial infiltrate.
tx for schnitzer syn
RAT-Rc
rituximab Anakinra Tocilizumab Rilonacept Canakinumab
duration of chronic urtaicaria
> 6 weeks
Dermatographism has been assoc w what H2 blocker
Famotidine
Tx
Antihistamine
Phototherapy- Severe
Omalizumab- refractory
type
Type of urticaria produced by the action of acetylcholine on mast cell. characterized by minute highly pruriotix phnctate wheals or papules 1-3 mm. surr by distinct erythematous flare.
occur on trunk and face primarily.
spares palms and sole
persist for 30-90 mins ff by refractory period of upto 24 hrs.
Cholinergic urticaria
urticaria mediated by norepinephrine.
eruption consist of small 1-5 ‘m red macules and papules w a pale halo appearing w/in 10-15 mins of emotional upset coffee chocolate .
Provocative test.
Adrenergic urticaria
ID 3-10ng of norepinephrine
seconardy cold urticaria is assoc w
underlying systemic disease such as cryoglobulinemia.
is group with other of the inflammatory symptoms discussed earlier deletions produce a burning sensation rather than itching they may have cyanotic centers and surrounding white halo and last for 24 to 48 hours they may be accompanied by fever chills headache arthralgia myalgia abdominal pain a prominent is leukocytosis which is the first observable response to cold
familial cold autoinflammatory syndrome
Provocative test for Vibratory angioedema
laboratory vortex vibration is applied to forearm for 5 mins
The primary effector cell in urticartial lesions
Mast cell
If individual whels last for longer than 24 hours what should be performed
Skin biopsy
Histo for urticaria
Mild dermal edema
margination if neutrophils within post capillary venules. later neutrophils mugrate through the vessel wall into the interstitium and eo and lympho are noted in infiltrate.
karyorrhexis and fibrin deposition within vessel walls are absent.
Mainstay treatment of acute urticaria
Antihistamine
Severe rxns:
0.3 ml dose of a 1:1000 diutuon of epinephrine every 10-20 mins.
In young children half strength dilution is used.
Adj:
IM antihistamine 25-50 mg hydroxyzine or diphenhydramine every 6 hours as needed.
systemic steroid 250mg hydrocortisone or 50mg Methyprednisolone IV every 6 hrs for 2-4 doses.
Hereditary angioedema
age
manif
Before 20 yrs okd.
sudden attacks of angioedema
Osler as frequently as every two weeks to out the patient’s life lasting for 2 to 5 days swelling is typically a symmetric and urticaria or itching does not occur. patients experience local swelling in the subcutaneous tissue abdominal organs mimicking surgical emergency and the upper airway which can be life-threatening. Minimal response to antihistamines epinephrine or corticosteroids
other name Quincke edema
Heritance of hereditary angioedema
AD
1:50000 -150000
HE THREE PHENOTYPIC FORMS OF DX
Type I - low antigenic and functional plasma levels of a normal C1 esterase inhibitor.
Type 2- Normal or elevated antigenic levels of a dysfunctional protein.
Type 3- normal C1-E1 function and normal compliement.
HE type 1 and 2 C levels
C4 low
Low C1 C1q C2
Screeninh test of choice for type 1 and2: c4 level.
Low c1 l-e1 in presence of normal c4 should raise suspicion of sample decay.
Treatment of choice for acute HAE types 1 and 2:
Plasma derived or recombinant C1 inhibitor or contact system modulators such as ecallantide or icatibant.
Short term prophylaxis for HAE
C1 inhibitor replacement tx or danazol.
HAe Type 3 tx
Danazol.
not respond to C1 E1 replacment.
Acquired C1 esterase i hibitor def is indistinguishable w Hereditary angioedema. but the onset is
after 4th decade
HAE is before 20 yrs old
also doesnt have pruritus and urticaria.
diff aquired angioedema 1 and 2
1 - assoc w lymphoprpliferative disease.
2- Presence of autoantibodies to C1 E1.
mgt of acquired c1.
Acquired angioedema 1- replacement if c1 e1. w plasma derived recombinant c1 inh.
2- Immunosuppresive tx.
systemic steroid may be temporarily effective.
effective fir chinuc spontaneous urticaria
Omalizumab - IgE.
150-300 mg every 4 weeks.
acQuired C1 esterase inhibitor deficiency diff w HAE
Indistinguishable w HAE ut onset after 4th decade of life and lacking a family history.
as in HAE, no assoc pruritus and urticaria.
