41 - Urticaria and Angioedema Flashcards
Local transient skin or mucosal edema and an area of redness that diminish within a day
Urticaria
Local and transient skin is mucosal edema that develops in deep tissue mostly without itching but may accompany pain or burning sensations
Angioedema
Nonacute or chronic urticaria
Persist for more than 6 weeks
Chronic spontaneous urticaria CS can be divided into
With concomitant angioedema
Without angioedema
Recurrent angioedema without wheals
Women have urticaria nearly _____ as often as men do
Twice
Edema that involves the superficial portion of the dermis
Wheals
Edema that extends into the deep dermis or subcutaneous and submucosal layers
Angioedema
Y/N: Angioedema may be painful and pruritic
No - painful but not pruritic
The heterogeneity of wheals in size and shape is one of the characteristics of
Spontaneous urticaria
The duration of individual wheals is long in _____ and shorter in _____
Spontaneous urticaria and deep pressure urticaria
Physical urticaria
Angioedema is rare in
Physical urticaria
Flowerlike or annular-shaped wheals are characteristic of
Spontaneous urticaria
(Acute/Chronic) spontaneous urticaria tends to be more severe
Acute
Most common subtype among the physical urticarias
Mechanical urticaria or dermographism
Rare and severe case of symptomatic dermographism with erythematous lines accompanied by punctate wheals characteristic of cholinergic urticaria
Cholinergic dermographism
Wheals of symptomatic dermographism may return in the same site or newly develop 3-6 hours after stimulation and persist for up to 48 hours
Delayed dermographism
Wheals of symptomatic dermographism may be markedly augmented when the skin is chilled
Cold-dependent dermographism
Local skin contact with a cold substance induces wheals and flare
Cold contact urticaria
Erythematous edematous and deep swelling may appear 9-18 hours after cold challenge
Delayed cold urticaria
Widespread wheals and flare develop in response to cooling of the core body temperature
Systemic cold urticaria
Familial cold urticaria syndrome is classified as a subtype of
Cryopyrin-associated periodic syndrome
Cryopyrin-associated periodic syndrome is an autosomal dominant inherited disease which is associated with a genetic mutation of
NLRP3 (CIASI)
Develop wheals and flare that spread in the area of skin exposed to heat, regardless of the core body temperature or sweating
Heat urticaria
Y/N: In solar urticaria, face and hands may develop fewer lesions than skin areas that are usually covered by clothes
Yes - because of hardening due to chronic exposure to sunlight
Deep dermal wheals that appear in a continuously compressed region
Delayed pressure urticaria
Missense mutation of _____ has been reported to be associated with familial vibratory urticaria with autosomal dominant inheritance
ADGRE2
Characterized by small wheals, resembling eruption of cholinergic urticaria, but wheals in this urticaria subtype are generally fewer in number compared with eruptions of cholinergic urticaria
Aquagenic urticaria
Induced by stimuli that cause sweating and distinctive for its small urticarial eruptions
Cholinergic urticaria
Pruritus without wheals induced by conditions that elicit sweating
Cholinergic pruritus
Major antigen in sweat has been identified as _____, a protein produced and released be by _____
MGL_1304
Malassezia globosa
Oral edema and discomfort induced by contact of the oral mucosa with certain foods
Oral allergy syndrome
Those with hereditary angioedema do not develop
Superficial wheals
Most frequent sign of anaphylaxis
Wheal and flare
Classical type I allergy against exogenous antigens, such as food and drugs, is less than _____% of the whole population of patients with urticaria
10
Dietary pseudoallergens that may be relevant as a cause or a precipitating or aggravating factor for CSU
Salicylates and biogenous amines
Food additives
Topical or oral NSAIDs
For a diagnosis of food pseudoallergy, _____ may be followed by well-motivated patients
3-week pseudoallergen-free diet
The increased sensitivity of patients with CSU to _____ in the diet has been suggested as a cause of nonallergic hypersensitivity
Histamine
In patients with CSU, especially those with severe symptoms, the levels of _____ are higher than those in healthy control participants
Plasma coagulation markers, such as
Prothrombin fragment 1+2 (PF 1+2)
Fibrin degradative products (FDPs)
D-dimer
Known to be associated with spontaneous urticaria, especially with the onset of acute urticaria in children and transient aggravation of CSU
Acute infections by viruses and bacteria
_____, which inhibit cyclooxygenase-1, may induced or aggravate wheals and angioedema
Aspirin and other NSAIDs
_____ are the most common signs of immediate reactions to contrast media, but delayed nonimmediate reactions may include a _____
Erythema and wheals with or without angioedema
Maculopapular rash
Drugs that induced histamine release from mast cells or basophils, especially when given at high doses
Opiate analgesics Polymyxin B Curare D-tubocurarine Vancomycin
Drug that does not cause release or production of mediators by themselves but inhibit degradation of bradykinin
ACE inhibitors
CSU may be associated with
Infections
Autoimmune disorders, such as thyroid diseases and collagen diseases
Have a higher risk of developing latex allergy
Health care workers
Individuals with atopic dermatitis
Patients with spina bifida
Flares in disease activity during the evening to early morning is not specific but is a common feature of
Spontaneous urticaria
Wheals of indictable urticaria do not usually last for more than 4 hours expect
Delayed pressure urticaria
_____-mediated angioedema does not usually occur in the larynx
Mast cell
_____-mediated angioedema does not accompany superficial wheals
Bradykinin
Routine laboratory testing recommended for acute spontaneous urticaria
None
Routine laboratory testing recommended for chronic spontaneous urticaria
CBC
ESR or CRP
Hereditary angioedema types
I - lack C1-INH protein
II - lack activity of C1-INH because of genetic point mutations
III - normal C1-INH
Acquired angioedema may develop by
Overconsumption of C1-INH caused by myeloproliferative diseases
Presence of autoantibodies against C1-INH
Characterized by urticaria-like eruptions and monoclonal IgM or IgG gammopathy accompanied by systemic syndrome such as fever and bone and muscle pain
Schnitzler syndrome
Schnitzler syndrome histology resembles
Urticarial vasculitis or neutrophilic urticaria
Easy and comprehensive method of evaluation for all kinds of urticaria and angioedema
Urticaria Control Test
Infiltration of neutrophils is prominent especially in
Acute urticaria
Physical urticaria
Mainstay of treatment for all kinds of mast cell-mediated urticaria
Second-generation nonsedating antihistamines
Adverse effects of first-generation antihistamines
Carry-over effects of sedation in the morning
Paradoxical excitation or possibly epilepsy in children
Second-line therapy for intractable cases resistant to standard doses of antihistamines
Increased dosing of antihistamines up to fourfold
Inhibits circulating IgE and consequently decreases the number of high-affinity Ig E receptor on mast cells and basophils
Omalizumab
Although it takes 2 months or longer for omalizumab to decrease the number of cell surface high-affinity Ig E receptor, the clinical effect of omalizumab on CST may become apparent within
1 week
In most cases of _____, antihistamines are not effective
Delayed pressure urticaria
Urticarial vasculitis
Used for treatment of acute attacks of bradykinin-mediated HAE
Icatibant
Ecallantide
Purified or recombinant C1-INH
Used for prophylaxis of bradykinin-mediated HAE
Purified or recombinant C1-INH
Lanadelumab
BCX7653
Y/N: Antihistamines do not cross the plancenta
No - cross the placenta
Among antihistamine, _____ is often chosen for pregnant women based on its long bioavailability and safety record
Chlorphenamine
All family members of patients with HAE type I and type II who are older than _____ are recommended to undergo screening
1 year old