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Chapter 22 & 23 Urticaria, mastocytoses and rosacea > Urticaria > Flashcards

Urticaria Flashcards

1
Q

Aeitologies of urticaria

A

idiopathic
immunological (autoimmune, immune complex, allergic, complement)
Non immunological: direct mast cell releases, ACEI, aspirin, nsaids etc

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2
Q

Associations with urticaria

A

Thyroiditis, SLE, ?coeliac, H Pylori

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3
Q

Aggravating factors

A

URTIs, drugs e.g. salicylates, nsaids, ACEI, penicillin.
Pressure, overheating, premenstrual
Alcohol, stress, unrelated viral infections
tartrizine, azo dyes

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4
Q

T/F C1 esterase inhibitor deficiency and ACEI induced angioedema are due to mast cells

A

F - acei : kinins

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5
Q

T/F mast cells are responsible for autoinflamamtory syndromes

A

F - IL1 and 18 activated by caspase 1

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6
Q

H2 is responsible for itch, wheal, flare and erythema

A

F H2 for erythema and wheal only

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7
Q

What medication class may help in aspirin sensitive people?

A

leukotriene antagonists.

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8
Q

How are mast cells activated via immunological means?

A

linkage of 2 adjacent alpha subunits of IgE receptors of a mast cell

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9
Q

What type of hypersensitivy reaction is urticarial vasculitis

A

type III

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10
Q

How long does an IgE mediated urticaria last?

A

max 60 min

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11
Q

What are pseudoallergans

A

sensitization of the immune system is not involved, but clinically the same symptoms appear. e.g. aspirin, food additives, natural salicylate, contrast dye, muscle relaxants, plasma expanders

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12
Q

Non allergic causes of acute urticaria

A

histamine liberators (eg codeine, vancomycin)
Pseudoallergans eg aspirin, food additives
Alcohol
foods with vasoactivate amines: cheese, fish, tomatos, pineapple, avocados
Histamine from strawberries, scombroid fish

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13
Q

Tests for Chronic urticaria

A

FBC, ESR/CRP (increased in inflammatory syndromes, UV), thyroid abs and TFTs, ANA
Bx on wheal 12 hours old ?vasculitis
stool OCP
Consider: H pylori, strongyloides
consider pseudo allergen free diet for 2-3 weeks
?Anti-FceR(autoab to Fc receptor of IgE)
consider serum EPP, urine Bence jones - if fever, bony pain, raised CRP, neutrophils in bx.

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14
Q

Tests in acute

A

often none
maybe specific IgE, skin prick
URTI/bacterial screen

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15
Q

Q to ask re chronic urticaria

A 
Can you induce the symptoms?
Fever?Joint pain/malaise? Family hx?
wheals last longer than 24 hours?
Angioedema alone? 
Medications eg NSAID, ACEI
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16
Q

Treatment ladder for chronic urticaria

A

1st line = 2nd generation antihistamines. If persist > 2 weeks:
2nd line:Increase dose up to 4 x max
If persists after 1-4 weeks:
3rd line: omalizumab or cyclosporin A or montelukast

Can use short courses of CS if required.
Consider Doxepin or promethazine nocte

General measures: avoid overheating, stress, alcohol, drugs with potential to worsen e.g. codeine, aspirin, ACEI

1% menthol in aqueous

Other possibilitys: IVIg, plasmapheresis
Thyroxine in euthyroid with thyroid autoimmunity
nbUVB
Colchicine and dapsone if inflammatory infiltrate is neutrophil predominant

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17
Q

Tx of urticarial vasculitis

A

Dapsone, prednisone

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18
Q

How to use CsA in chronic urticaria

A

4mg/kg once daily 16 weeks.

helpful in about 2/3

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19
Q

How to use omalizumab

A

150-300mg sub cut every 4 weeks for 6/12
add on to antihistamines
keep in hospital to observe for 2-4 hours - risk of anaphylaxis
baseline platelets - risk of thrombocytopenia

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20
Q

Investigations if you suspect urticarial vasculitis

A

Bx DIF: vascular C3, fibrin and immunoglobulins
C3, C4, C1q, ANA, ENA, dsDNA, ANCAs
Hep B,C, HIV, EBV
serum EPP - schnitzlers if fever, bone pain, lymphadenopathy
due to risk of systemic involvement: ESR, Urinalysis
chest X-ray if symptomatic, RFTs
elfts

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21
Q

Important points on hx if you suspect UV

A 
sx: burning, pain, how long they last, resolve with bruising?
angioedema?
fevers, malaise, bony pain
abdo pain, nausea, vomiting
Drugs e.g. cimetidine, diltiazem
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22
Q

In what % of patients does chronic urticaria last >5 years?

A

15%

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23
Q

% causes of acute urticaria

A

20% allergic e.g. drugs/food
30% infections
50% unknown

24
Q

% causes of chronic urticaria

A

20% physical

80% unknown

25
Q

Types of physical urticarias

A

Mechanical: delayed pressure, symptomatic dermatographism, vibratory
Thermal: cholinergic, cold contact, localised heat, systemic cold urticaria
Other: aquagenic, solar, exercise induced anaphylaxis

26
Q

What treatment is recommended in delayed pressure urticaria

A

poorly responsive to antihistamines, but some help with 10mg tads cortisone as it also inhibits eosinophil migration, dapsone, colchicine, sulfasalzine, montelucast, nsaids (but may aggravate ordinary urticaria), topical steroids under occlusion

27
Q

How to test for aquagenic urticaria

A

water in bath for 15min or water soaked towels at body temp for 30min

28
Q

How to test for solar urticaria

A

Check porphyrins
Uusally fade in 2 hours cf to PMLE which appears hours later and lasts days
exposure to sunlight or solar stimulator - takes a few min, lasts less than 1 hour.

29
Q

How to test for cholinergic urticaria

A

warm in hot bath 42 degrees for 15 min - raises core temp 0.7-1degree, or exercise until sweating.

30
Q

How to test for delayed pressure urticaria

A

weight of 7kg hanging by broad strap, wheal at site 4-6 hours later, or 4.5kg weight rods with a 1.5cm diameter applied for 15 min.

31
Q

How to test for systemic cold

A

ice cube test negative

cold room at 4 degrees for 30 min in lightweight clothes.

32
Q

How to test for cold contact urticaria

A

consider cryoglobulins, cold agglutinins, cryofibrinogens, hep B, C, FBC, ANA
ice cube in a thin plastic bag for up to 20min to skin. Whealing occurs within 15 min usually during rewarming.

33
Q

Angioedema without wheals - causes

A

Idiopathic
Drugs usually ACEI, Angiotensin II receptor blockers
Hereditary: C1 esterase inhibitor deficiency or impaired function or females with oestrogen dependant angioedema
Acquired C1 esterase inhibitor deficiency.

34
Q

Causes of acquired C1 esterase inhibitor deficiency angioedema

A

1) lymphoproliferative: b cell lymphoma, nHL, multiple myeloma
2) SLE, autoimmune
3) Abs directed against inhibitor but no evidence of lymphoma

35
Q

Different types of hereditary angioedema

A

1) Reduced amts of C1 esterase inhibitor
2) inactive form : 15% normal, or increased but dysfunctional
3) women with family hx but normal levels - oestrogen dependent

36
Q

Treatment of hereditary angioedema

A 
avoid oestrogen
check for SLE, sjogrens
Stanozolol 2mg alt days to 10mg daily
Danazol 200mg mond to fri to 400mg daily
Tranexamic acid 0.5-3mg daily 
C1 esterase inhibitor concentrate
FFP
Icatibant (bradykinin 2 receptor antagonist) 30mg SC
37
Q

Ix of angioedema

A

check meds
C3, C4, C1q, CH50, C1 esterase inhibitor and function
ANA, ENA, dsDNA (associations with SLE, Sjogrens)
If C1q is LOW: consider acquired: serum EPP, hence ones, lymphoma,

38
Q

What do you expect in results of complement studies in hereditary angioedema type 1

A

Low C1 INH
Low C4
normal C1q
CH50 may reduce during attacks

39
Q

What do you expect in results of complement studies in hereditary angioedema type 2

A

Normal or elevated C1 INH but dysfunctional
low C4
normal C1q

40
Q

What do you expect in results of complement studies in hereditary angioedema type 3

A

normal C1 INH, normal C4, normal C1q

mutation in factor XII gene

41
Q

Diagnostic criteria for schnitzlers

A 
Major criteria (must have these) and 2 minor criteria
Major: chronic urticarial rash, monoclonal IgM or IgG
Minor: recurrent fever, abnormal bone remodelling, neutrophilic dermal infiltrate, leucocytoisis and/or elevated CRP
42
Q

What are Cryopryin associated periodic syndromes

A

NLRP3 gene mutation. Rare hereditary inflammatory disorder with 3 phenotypes. Due to a gain of function mutation affecting the cryopyrin protein

1) familial cold auto inflammatory syndrome AD
2) Muckle Wells - AD
3) Neonatal onset multisystem inflammatory disease/chronic infantile neurological cutaneous and articular syndrome

43
Q

If you suspect a CAPS what do you do?

A 
check FBC - neutrophilic
CRP, ESR - elevated
urinary protein to detect amyloid 
call renal 
Bx: perivascular and sometimes peregrine neutrophilic infiltrate of the reticular dermis without mast cells or vasculitis 
Tx is anakinra
44
Q

What is familial mediterranean fever?

A

AR, MEVR1 gene
self limiting attacks of fever, peritonitis, pleurisy and synovitis. Erysipelas like lesions on the lower leg. 1/4 may develop renal amyloidosis.

45
Q

Types of hereditary auto inflammatory syndromes

A

CAPS
familial mediterranean fever
hyperIgD syndrome
TRAPS

46
Q

What are TRAPS

A

TNF receptor associated periodic syndrome
AD disorder.
Migratory erythema, febrile epidsodes, migratory muscle pain, amyloidosis

47
Q

Clinical features of muckle wells

A

sensioneural hearing loss in 2/3, headaches, abdominal pain, mouth ulcers, amyloidosis in 25% leading to kidney failure and death, delayed puberty.

48
Q

urticarial/angioedema like conditions associated with paraproteins

A

schnitzlers, acquired C1 esterase inhibitor deficiency angioedema, systemic papillary leak syndrome

49
Q

People with auto inflammatory syndromes do not have angioedema as an associated symptom T/F

A

T

50
Q

Diseases which may manifest urticarial lesions (according to the curriculum)

A

Arthropod bites - immediate, hypersensitivity reaction (papular urticaria)
contact dermatitis, exanthematous drug eruption
urticarial dermatitis
Polymorphic eruption of pregnancy
Mastocytosis in children
Autoimmune bullous diseases
Rare: autoimmune progesterone/oestrogen dermatitis, interstitial granulumatous dermatitis, eosinophilic cellulitis, neutrophilic eccrine hidradenitits, urticaria like follicular mucinosis.

51
Q

Angioedema not associated with urticaria

A

hereditary angioedema
acquired C1 esterase inhibitor deficiency
idiopathic recurrent angiodema

52
Q

Systemic diseases that can present with urticarial skin lesions (curriculum)

A

Vasculitides eg churg strauss, wegeners, PAN
urticarial vasculitis
SLE, Sjogrens, dermatomyositis
Mixed connective tissue disease
Juvenile RA
Haematologic disease
NHL (b cell), cryoglobulinaemia, hypereosinophilic syndromes, polycythaemia vera.

53
Q

In angioedema without wheals, but normal C4 - consider what?

A

idiopathic, drug induced, episodic angiodema with eosinophilia, hereditary type III, capillary leak syndrome

54
Q

What is urticarial dermatitis

A

Usually in elderly with both urticaria like and eczema like lesions. Often reported as dermal hypersensitivity reaction with minimal spongiosis, mixed inflammatory cells in the dermis. May be an early sign of BP, but could be related to drug, malignancy

55
Q

Neutrophilic urticarial dermatoses

A

Urticaria with neutrophilic dermatoses histopathologically
Strongly associated with a systemic disease (schnitzler, adult onset stills, LE, hereditary auto inflammatory fever syndromes)

56
Q

Clinical features of adult onset Still’s disease

A

High fevers often occur late afternoon, arthritis with carpal ankylosis, asymptomatic macular exanthema which accompanies the fevers (salmon pink in colour) and frequently koebnerises
F>M (usually presents 60)
Patients often have very elevated ferreting
Think of it in 3 weeks of fever, elevated CRP, ESR, platelets, arthritis

Chapter 22 & 23 Urticaria, mastocytoses and rosacea (4 decks)

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