Urticaria

Question 1

Rare disorder comb of chronic non pruritic urticaria, fever of unknown origin, disabling bone pain, hyperostosis, increased ESR, IGM.
CHED-FM acronym

Answer 1

Schnitzler syndrome

Question 2

schnitzler syndrom gender
age onset
Biopsy.

Answer 2

no gender predilec
29-77 yo.
Neutrophilic perivascular and interstitial infiltrate.

Question 3

tx for schnitzer syn

Answer 3

RAT-Rc

rituximab 
Anakinra 
Tocilizumab 
Rilonacept 
Canakinumab

Question 4

duration of chronic urtaicaria

Answer 4

> 6 weeks

Question 5

Dermatographism has been assoc w what H2 blocker

Answer 5

Famotidine

Question 6

Tx

Answer 6

Antihistamine
Phototherapy- Severe
Omalizumab- refractory

Question 7

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.

Answer 7

Cholinergic urticaria

Question 8

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.

Answer 8

Adrenergic urticaria

ID 3-10ng of norepinephrine

Question 9

seconardy cold urticaria is assoc w

Answer 9

underlying systemic disease such as cryoglobulinemia.

Question 10

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

Answer 10

familial cold autoinflammatory syndrome

Question 11

Provocative test for Vibratory angioedema

Answer 11

laboratory vortex vibration is applied to forearm for 5 mins

Question 12

The primary effector cell in urticartial lesions

Answer 12

Mast cell

Question 13

If individual whels last for longer than 24 hours what should be performed

Answer 13

Skin biopsy

Question 14

Histo for urticaria

Answer 14

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.

Question 15

Mainstay treatment of acute urticaria

Answer 15

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.

Question 16

Hereditary angioedema
age
manif

Answer 16

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

Question 17

Heritance of hereditary angioedema

Answer 17

AD

1:50000 -150000

Question 18

HE THREE PHENOTYPIC FORMS OF DX

Answer 18

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.

Question 19

HE type 1 and 2 C levels

Answer 19

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.

Question 20

Treatment of choice for acute HAE types 1 and 2:

Answer 20

Plasma derived or recombinant C1 inhibitor or contact system modulators such as ecallantide or icatibant.

Question 21

Short term prophylaxis for HAE

Answer 21

C1 inhibitor replacement tx or danazol.

Question 22

HAe Type 3 tx

Answer 22

Danazol.

not respond to C1 E1 replacment.

Question 23

Acquired C1 esterase i hibitor def is indistinguishable w Hereditary angioedema. but the onset is

Answer 23

after 4th decade
HAE is before 20 yrs old

also doesnt have pruritus and urticaria.

Question 24

diff aquired angioedema 1 and 2

Answer 24

1 - assoc w lymphoprpliferative disease.

2- Presence of autoantibodies to C1 E1.

Question 25

mgt of acquired c1.

Answer 25

Acquired angioedema 1- replacement if c1 e1. w plasma derived recombinant c1 inh.

2- Immunosuppresive tx.

systemic steroid may be temporarily effective.

Question 26

effective fir chinuc spontaneous urticaria

Answer 26

Omalizumab - IgE.

150-300 mg every 4 weeks.

Question 27

acQuired C1 esterase inhibitor deficiency diff w HAE

Answer 27

Indistinguishable w HAE ut onset after 4th decade of life and lacking a family history.

as in HAE, no assoc pruritus and urticaria.