41: Urticaria Flashcards
1
Q
What is urticaria?
A
Urticaria is defined as a skin disorder characterized by local transient skin or mucosal edema (wheal) and an area of redness (erythema) accompanied by itchy sensations that diminish within 1 day.
2
Q
What role do mast cells play in urticaria?
A
Mast cells and their histamine play a crucial role in the pathogenicity of urticaria, as they can release histamine which contributes to the symptoms of the condition.
3
Q
What are the clinical features of wheals in urticaria?
A
Wheals are circumscribed, raised, usually pruritic, and evanescent areas of edema that involve the superficial portion of the dermis, and they are highly pruritic.
4
Q
What is angioedema and how does it differ from urticaria?
A
Angioedema is a local and transient skin or mucosal edema that develops in deep tissues, mostly without itching but may accompany pain or burning sensations, whereas urticaria involves wheals that are more superficial and itchy.
5
Q
What are the common triggers that may aggravate urticaria or angioedema?
A
Common triggers include infections, stress, fatigue, and drugs, especially NSAIDs and ACE inhibitors.
6
Q
What is the peak age for chronic spontaneous urticaria (CSU)?
A
The peak age for chronic spontaneous urticaria (CSU) is between 20 to 40 years old.
7
Q
What are the noncutaneous findings associated with urticaria?
A
Noncutaneous findings include discomfort of the stomach and intestine, pharyngeal edema (bradykinin-induced), and anaphylaxis, which is a serious, life-threatening generalized or systemic hypersensitivity reaction.
8
Q
What are the key clinical features that differentiate wheals from angioedema in urticaria?
A
9
Q
How does chronic spontaneous urticaria (CSU) with concomitant angioedema differ from CSU without angioedema?
A
Chronic spontaneous urticaria (CSU) can be categorized based on the presence of angioedema:
- With concomitant angioedema: 33% to 67% of CSU patients experience this, indicating a significant overlap that may complicate management due to the potential for deeper tissue involvement.
- Without angioedema: 29% to 65% of patients, which suggests that a substantial number of CSU cases may not present with this complication, potentially leading to a different therapeutic approach.
10
Q
What are the potential triggers for urticaria and angioedema?
A
Potential triggers include infections, stress, fatigue, medications (especially NSAIDs and ACE inhibitors), and autoantibodies against IgE or its receptor.
11
Q
What are the complications associated with urticaria?
A
Higher incidence of autoimmune thyroid disease, rheumatoid arthritis, Sjogren syndrome, celiac disease, type I diabetes mellitus, and systemic lupus erythematosus.
12
Q
How is urticaria classified?
A
Urticaria is classified by duration, trigger, mode of induction, and underlying causes. Specific subtypes include cholinergic, adrenergic, and aquagenic urticaria.
13
Q
What is the mechanism of Type I hypersensitivity in urticaria?
A
In Type I hypersensitivity, mast cells are activated via high affinity IgE receptors and antigen-specific IgE, leading to urticaria symptoms that develop within 15 minutes to 1 hour.
14
Q
What is the autoimmune mechanism associated with chronic spontaneous urticaria (CSU)?
A
1/3 to ½ of patients with CSU possess functional IgG antibodies against IgE or high affinity IgE receptors, leading to histamine release.
15
Q
What is pseudoallergy in the context of urticaria?
A
Pseudoallergy refers to nonallergic hypersensitivity to dietary pseudoallergens, including naturally occurring food ingredients and additives.
16
Q
What are the findings related to the coagulation system in patients with urticaria?
A
Levels of plasma coagulation markers, such as prothrombin fragment 1+2 and D dimer, are higher in patients with urticaria compared to healthy controls.
17
Q
A patient with urticaria has elevated levels of prothrombin fragment 1+2 and fibrin degradation products. What does this suggest?
A
Elevated levels of these markers suggest increased potential for blood coagulation, which correlates with disease severity in chronic spontaneous urticaria.
18
Q
A patient with urticaria has a history of Sjogren syndrome. What is the relationship between these conditions?
A
Chronic urticaria may be associated with autoimmune disorders like Sjogren syndrome, although the prevalence is low.
19
Q
A patient with urticaria reports symptoms triggered by food additives. What diagnostic approach can confirm pseudoallergy?
A
A 3-week pseudoallergen-free diet can help diagnose food pseudoallergy.
20
Q
What is the role of mast cells in the pathogenesis of urticaria?
A
Mast cells play a crucial role by degranulating and releasing histamine and other vasoactive mediators.
21
Q
How do infections contribute to the development of chronic spontaneous urticaria (CSU)?
A
Acute infections caused by viruses and bacteria are associated with spontaneous urticaria, and chronic infection by Helicobacter pylori is recognized as an important cause of CSU.
22
Q
What factors may induce or aggravate urticarial reactions?
A
Factors include aspirin and other NSAIDs, serum sickness, blood products, contrast media, and other mast cell and basophil activators.
23
Q
What are the risk factors associated with chronic spontaneous urticaria (CSU)?
A
Risk factors include age, gender ratio of 2:1 (F:M), association with infections and autoimmune disorders, and strong association with HLA-DR4.
24
Q
A patient develops urticaria after taking NSAIDs. What alternative medication can be considered safer?
A
COX-2 selective NSAIDs tend to be safer for patients who develop urticaria after taking NSAIDs.
25
A patient with chronic urticaria has a history of autoimmune thyroid disease. What is the relationship between these conditions?
Chronic urticaria may be associated with autoimmune disorders like thyroid disease, although the prevalence is low.
26
A patient with urticaria reports symptoms triggered by emotional stress. What mechanism might explain this?
Stress can lead to excessive release of acetylcholine, which may activate mast cells and contribute to urticaria symptoms.
27
A patient with urticaria has a history of atopic dermatitis. What subtype of urticaria is commonly associated with this condition?
Cholinergic urticaria is often complicated by atopic dermatitis.
28
A patient with urticaria has a history of systemic lupus erythematosus (SLE). What underlying mechanism might link these conditions?
SLE may underlie chronic urticaria by consuming C1 inhibitor, resulting in overproduction of bradykinin.
29
A patient with urticaria reports symptoms triggered by emotional stress. What neuropeptides might be involved in the pathogenesis?
Neuropeptides like substance P, vasoactive intestinal peptide, and somatostatin may activate mast cells and contribute to urticaria symptoms.
30
What are the unique characteristics of mast cells in relation to neurogenic mechanisms?
Mast cells uniquely respond to substances such as substance P, vasoactive intestinal peptide, somatostatin, neurokinin A and B, bradykinin, cGRP, and ACh.
31
What infections are associated with chronic spontaneous urticaria (CSU)?
Acute infections caused by viruses and bacteria are associated with spontaneous urticaria. Chronic persistent infection by Helicobacter pylori is also recognized as an important cause of CSU.
32
What role do autoimmune disorders play in chronic urticaria?
Chronic urticaria may be associated with autoimmune disorders such as thyroid diseases and collagen diseases, though the prevalence is low and not significant. Systemic Lupus Erythematosus (SLE) may lead to angioedema without wheals.
33
What factors can induce or aggravate urticarial reactions?
Factors include Aspirin and NSAIDs, Serum Sickness, Blood Products, Contrast Media, and other mast cell activators like opiate analgesics and vancomycin.
34
What are the risk factors for chronic spontaneous urticaria (CSU)?
Risk factors include occurrence at any age, a male-to-female ratio of 2:1, association with infections and autoimmune disorders, strong association with HLA-DR4 in the white population, and presence of functional autoantibodies.
35
What is the characteristic feature of urticaria?
The characteristic feature of urticaria is the transient nature of individual eruptions.
36
Why is a thorough history essential in diagnosing urticaria?
A thorough history is essential for diagnosing urticaria as it helps elucidate causative or aggravating factors.
37
How do spontaneous urticaria and inducible urticaria differ?
Spontaneous urticaria flares during the evening to early morning, while inducible urticaria wheals do not usually last for more than 4 hours, except for Delayed Pressure Urticaria (DPU).
38
What laboratory tests are recommended for Chronic Spontaneous Urticaria (CSU)?
Recommended tests include CBC, ESR, CRP, and examinations for Helicobacter pylori, Type I allergy, autoantibodies, and thyroid hormone.
39
What biomarkers reflect disease severity in CSU?
Biomarkers include Mean Platelet Volume (MPV), IL-6, D-dimer, FDP, PF 1+2, and CRP.
40
How is the disease activity of angioedema evaluated?
The disease activity may be evaluated by a 4-week-long record of edema in terms of frequency, duration, subjective sensations, and disability.
41
What is the purpose of the Urticaria Control Test (UCT)?
The Urticaria Control Test (UCT) asks four retrospective questions about the patient's condition over the previous 4 weeks.
42
What do elevated D-dimer and CRP levels indicate in chronic urticaria?
Elevated D-dimer and CRP levels reflect disease severity or activity in chronic spontaneous urticaria (CSU).
43
What subtype of urticaria is likely if symptoms worsen during the evening?
Spontaneous urticaria often flares during the evening to early morning.
44
What diagnostic tests should be prioritized for recurrent angioedema without wheals?
For mast cell-mediated angioedema, look for triggers and perform histamine release tests. For bradykinin-mediated angioedema, assess C1-INH levels and activity, and check for C4 levels.
45
What additional diagnostic considerations should be taken into account for Inducible Urticarias?
Consider eliciting specific triggers, conducting threshold tests, and performing differential blood count, ESR, or CRP to rule out other conditions.
46
What are the characteristics of wheals in different subtypes of urticaria?
| Urticaria Type | Spontaneous Urticaria | Type I Allergy Mediated | Cold, Solar, Heat, and Deep Pressure | Symptomatic Dermographism | Cholinergic, Aquagenic, and Adrenergic | Angioedema |
|----------------|-----------------------|------------------------|-------------------------------|--------------------------|--------------------------------|----------------|
| Severity | +++ | ++ | +++ | +++ | +++ | +++ |
47
What are potential causes of episodic or acute urticaria?
| Potential Causes | Examples |
|------------------|---------|
| Foods | Milk, eggs, nuts, seeds, fruits, seafood, pseudoallergens |
| Drugs | Antibiotics, NSAIDs, ACE inhibitors |
| Vaccines | Transfusions |
| Stings | Bee, wasp venoms |
| Contactants | Latex, chemicals |
| Inhalants | Latex powder, chemicals |
48
What is the significance of the Histamine Release Assay in diagnosing spontaneous urticaria?
The Histamine Release Assay detects the presence of IgG autoantibodies against IgE or FcεRI, indicating an autoimmune mechanism.
49
What is symptomatic dermographism and how is it tested?
Symptomatic dermographism involves the development of pruritic wheals in areas exposed to shearing forces. A positive result is indicated by wheal and itching at the site of provocation ≤ 36 g/mm².
50
What does a positive autologous serum skin test (ASST) indicate in chronic spontaneous urticaria?
A positive ASST indicates autoreactivity and is associated with more severe symptoms and a slower response to treatments.
51
What does a positive histamine release assay indicate?
A positive histamine release assay indicates the presence of IgG autoantibodies against IgE or FcεRI, suggesting an autoimmune mechanism.
52
What are the key characteristics of spontaneous urticaria?
Key characteristics include spontaneous occurrence almost daily, wheals often accompanied by angioedema, heterogeneity in size and shape, and acute forms may last for a few days.
53
What diagnostic methods are recommended for spontaneous urticaria?
Diagnosis is based on history and skin inspection, looking for spontaneous wheals, diurnal variation, and long-lasting course. No routine laboratory tests are recommended.
54
What are the characteristics of wheals in urticaria?
Ular-shaped wheals are common. Acute forms tend to be more severe and may last for a few days.
55
How is spontaneous urticaria diagnosed?
Diagnosis is based on history and skin inspection, spontaneous appearance of characteristic wheals, diurnal variation, and long-lasting course of individual wheals. No routine laboratory tests are recommended for acute spontaneous urticaria.
56
What is the significance of the Histamine Release Assay in diagnosing spontaneous urticaria?
The Histamine Release Assay detects the presence of IgG autoantibodies against IgE or FcεRI using the patient’s serum and basophils from healthy donors. It may reveal the specificity of these autoantibodies and their dependence on or competition with IgE for histamine release. However, it is somewhat cumbersome and requires special equipment, which can limit its practical application.
57
How does symptomatic dermographism differ from spontaneous urticaria?
Symptomatic dermographism develops pruritic wheals in response to shearing forces on skin, while spontaneous urticaria occurs spontaneously without apparent cause. Wheals in symptomatic dermographism disappear approximately within 30 minutes, whereas those in spontaneous urticaria may last for a few days.
58
What are the characteristics of cholinergic dermographism?
Cholinergic dermographism is characterized by erythematous lines that may accompany punctate wheals. Reactions should be evaluated 5 to 10 minutes after testing.
59
What is the difference between cold contact urticaria and systemic cold urticaria?
Cold contact urticaria is localized and occurs within minutes after contact with a cold substance, while systemic cold urticaria involves widespread wheals and flare in response to cooling of the core body temperature.
60
What is the provocation test for heat urticaria?
The provocation test for heat urticaria involves applying hot metal, water, or a glass cylinder filled with hot water at 45°C to the skin of the volar forearm.
61
What symptoms are associated with solar urticaria?
Symptoms of solar urticaria include headache, syncope, dizziness, wheezing, and nausea, which develop within minutes after local exposure to certain wavelengths of light.
62
How is delayed pressure urticaria provoked for testing?
Delayed pressure urticaria can be provoked by suspending a 7-kg weight on a 3-cm shoulder strap for 15 minutes or by applying rods supported in a frame to the back, thighs, or forearm.
63
What is the significance of the skin biopsy in delayed pressure urticaria?
Skin biopsies in delayed pressure urticaria typically show an inflammatory infiltrate with prominent eosinophils but no vasculitis.
64
What triggers vibratory urticaria and how is it tested?
Vibratory urticaria is triggered by activities such as jogging or vigorous toweling. Testing involves holding the forearms on a flat plate placed on a vortex mixer running at 780 to 1380 rpm for 10 minutes, followed by assessment for swelling.
65
What is aquagenic urticaria and how is it provoked?
Aquagenic urticaria is induced by local skin exposure to water. It can be provoked by attaching wet clothes at body temperature for 20 minutes or by wiping the test area with an organic solvent and challenging with water.
66
What areas of the body are less likely to develop lesions due to hardening in urticaria?
The face and hands are less likely to develop lesions due to hardening compared to areas usually covered with clothes.
67
What genetic mutation is associated with familial cold urticaria syndrome?
Familial cold urticaria syndrome is associated with a genetic mutation in NLRP3 (CIASI).
68
What subtype of urticaria might a patient indicate if they develop wheals after jogging?
This may indicate vibratory urticaria. To confirm, place the forearms on a flat plate on a vortex mixer running at 780-1380 rpm for 10 minutes and assess for swelling at the site of application 10 minutes after testing.
69
How can you differentiate between cold contact urticaria and systemic cold urticaria?
Cold contact urticaria is confirmed by applying a wrapped ice cube or cold water for 5 minutes, inducing wheal formation. Systemic cold urticaria involves widespread wheals in response to cooling of the core body temperature.
70
What test can confirm solar urticaria?
Perform a provocation test using sunlight or a slide projector with or without filters on covered parts of the trunk or buttock skin.
71
What test can confirm delayed pressure urticaria?
Suspend a 7-kg weight on a 3-cm shoulder strap for 15 minutes or apply rods supported in a frame to the back, thighs, or forearm. Skin reactions should occur after a latent period of 2-4 hours.
72
What test can confirm heat urticaria?
Apply hot metal, water, or a glass cylinder filled with hot water at 45°C to the skin of the volar forearm.
73
What wavelengths should be tested to confirm solar urticaria?
Test using ultraviolet (UV) A or B, or visible light, with or without filters.
74
What genetic mutation is associated with vibratory urticaria?
Vibratory urticaria is associated with a missense mutation in ADGRE2.
75
What test can confirm systemic cold urticaria?
Systemic cold urticaria is not confirmed by local ice cube tests but requires assessment of widespread wheals in response to cooling of the core body temperature.
76
What subtypes of urticaria should be differentiated for symptoms triggered by hot temperatures?
Differentiate between cholinergic urticaria, solar urticaria, and aquagenic urticaria.
77
What histological findings are expected in delayed pressure urticaria?
Skin biopsies show inflammatory infiltrate with prominent eosinophils but no vasculitis.
78
What is the clinical significance of delayed cold urticaria?
Delayed cold urticaria can cause erythematous edematous and deep swelling appearing 9 to 18 hours after cold challenge. It is differentiated from systemic cold urticaria, which involves widespread wheals.
79
How is heat urticaria provoked?
Heat urticaria is provoked by applying hot metal, water, or a glass cylinder filled with hot water at 45°C to the skin of the volar forearm.
80
What distinguishes heat urticaria from cholinergic urticaria?
Heat urticaria develops wheals in the area exposed to heat regardless of core body temperature or sweating, while cholinergic urticaria is associated with an increase in body temperature and sweating.
81
What are the key features of delayed pressure urticaria?
Delayed pressure urticaria appears in a continuously compressed region with a latency of 30 minutes to several hours after the release of compression, often accompanied by burning sensation or pain rather than itching.
82
What is the mechanism behind vibratory urticaria?
Vibratory urticaria is associated with a missense mutation of ADGRE2 and can be triggered by activities such as jogging or vigorous toweling.
83
What are the characteristics of cholinergic urticaria?
- Induced by sweating
- Distinctive for small urticarial eruptions
- Develops in 30 minutes and disappears in a few hours
- Usually pruritic, may be painful or sting
- Common in children, adolescents, and young adults
- May be triggered by physical exercise, hot temperature, emotional or gustatory excitation
- Can present with angioedema in females with atopic diathesis or sweat allergy
- May lead to anaphylaxis
84
How is contact urticaria diagnosed?
Contact urticaria is diagnosed by immediate development of wheal and flare reaction at the site of contact with specific substances. Diagnosis involves applying the suspected substance on a normal area of skin for 15 to 20 minutes.
85
What are the clinical features of urticarial vasculitis?
- Recurrent urticarial lesions lasting more than 24 hours, leaving pigmentation
- Associated with leukocytoclastic vasculitis
- May develop livedo reticularis, Raynaud phenomenon, and angioedema
86
What are the types of angioedema and their characteristics?
- Mast cell mediated angioedema: Induced by specific stimuli, can be severe but mostly nonlethal.
- Bradykinin-mediated angioedema: Occurs spontaneously without wheals, can be lethal.
87
What is the significance of skin testing in diagnosing cholinergic urticaria?
Skin testing with intradermal injection of acetylcholine at 100 μg/mL is used to reinforce diagnosis. A positive result shows satellite wheals around the injection site.
88
What is the next step if a patient with suspected contact urticaria has a negative skin test?
Apply an occlusive application followed by a prick test. If the patient has a history of severe symptoms, start with sufficiently diluted substances.
89
What condition should be suspected if a patient presents with urticarial lesions lasting more than 24 hours?
Urticarial vasculitis should be suspected. A skin biopsy is essential to confirm the diagnosis.
90
What test can help confirm cholinergic urticaria in a patient with pruritus without wheals?
Perform a provocation test appropriate for the patient’s age and condition, such as exercise on a treadmill or stationary bicycle.
91
How can you differentiate aquagenic urticaria from cholinergic urticaria?
Aquagenic urticaria is characterized by small wheals surrounded by wide flares and is induced by water regardless of temperature, while cholinergic urticaria is induced by sweating.
92
What antigen is associated with cholinergic urticaria in females with atopic diathesis?
MGL_1304 antigen, released by Malassezia globosa, is associated with cholinergic urticaria.
93
What test can confirm latex allergy in a patient with urticaria?
In vitro tests for Type I allergy can confirm latex allergy.
94
How can you differentiate between cholinergic urticaria and exercise-induced anaphylaxis?
Perform a passive warming test to record core body temperature. An increase of ≥1.0°C supports cholinergic urticaria.
95
What solvent can increase reactivity in aquagenic urticaria testing?
Wiping the test area with an organic solvent before challenging with saline instead of tap water may increase reactivity.
96
What are the distinguishing features of cholinergic urticaria?
Cholinergic urticaria is characterized by induction by sweating, small urticarial eruptions within 30 minutes, and pruritus that may be painful or sting.
97
How does contact urticaria differ from other types of urticaria?
Contact urticaria is characterized by immediate wheal and flare reaction at the site of contact with specific substances and can be immunologic or nonimmunologic.
98
What are the clinical features and diagnostic considerations for urticarial vasculitis?
Urticarial vasculitis is characterized by recurrent urticarial lesions lasting more than 24 hours, often leaving pigmentation, and is associated with leukocytoclastic vasculitis.
99
What are the associated symptoms of leukocytoclastic vasculitis?
Symptoms may include livedo reticularis, Raynaud phenomenon, and angioedema.
100
What characterizes hypocomplementemic urticarial vasculitis syndrome?
It involves hypocomplementemia and IgG autoantibody against C1q.
101
What diagnostic considerations are there for urticarial eruptions?
Differentiation from urticarial eruptions lasting longer than 24 hours may require a skin biopsy for confirmation.
102
What clinical features may aid in diagnosing urticarial eruptions?
Features such as fever, malaise, arthralgia, and signs of diffuse glomerulonephritis or pulmonary disease may aid in diagnosis.
103
What are the key differences between mast cell-mediated angioedema and bradykinin-mediated angioedema?
| Feature | Mast Cell-Mediated Angioedema | Bradykinin-Mediated Angioedema |
|-------------------------------|--------------------------------|---------------------------------|
| Trigger | Induced by specific stimuli | Occurs spontaneously |
| Wheals | Often occurs with wheals | Does not occur with wheals |
| Severity | Can be severe but mostly nonlethal | Can be lethal |
| Duration | Lasts hours to a few days | Lasts hours to a few days |
104
What are the types of hereditary angioedema and their characteristics?
| Type | Characteristics |
|------|----------------|
| Type I | Lack C1-INH protein; decreased C1-INH activity and C4 |
| Type II | Lack C1-INH activity |
| Type III | Normal C1-INH; typically occurs in females; associated with gain of function mutation of Factor XII |
105
What is the mechanism behind acquired angioedema?
Acquired angioedema is mediated by bradykinin and may develop due to overconsumption of C1-INH caused by myeloproliferative diseases or presence of autoantibodies against C1-INH.
106
What are the clinical features of Schnitzler syndrome?
Schnitzler syndrome is characterized by urticaria-like eruptions, monoclonal IgM or possibly IgG gammopathy, systemic symptoms such as fever and bone/muscle pain, and histology resembling urticarial vasculitis or neutrophilic urticaria.
107
What are the key features of Cryopyrin Associated Periodical Syndrome?
Key features include familial cold-autoinflammatory syndrome, Muckle-Wells syndrome, chronic infantile neurologic cutaneous articular syndrome eruption, periodic fever, arthralgia, amyloidosis, and nerve deafness. Gain of function mutations in NLRP3 (CIASI) lead to overproduction of IL-1.
108
What distinguishes exercise-induced anaphylaxis (EIA) from food-dependent exercise-induced anaphylaxis (FDEIA)?
| Condition | Characteristics |
|-----------|----------------|
| EIA | Develops urticaria and anaphylaxis regardless of food intake |
| FDEIA | Symptoms develop only when exercising after eating certain foods; specific food allergen-reactive IgE may be present but not always sufficient for diagnosis |
109
What test can confirm food-dependent exercise-induced anaphylaxis (FDEIA)?
Perform provocation testing for FDEIA, which has a sensitivity of 70%.
110
What diagnostic marker is highly specific for food-dependent exercise-induced anaphylaxis (FDEIA) caused by wheat?
Anti-ω5-gliadin IgE is an exceptionally good marker for FDEIA.
111
What type of hereditary angioedema (HAE) is most likely in a patient with erythema marginatum?
Type I HAE is characterized by low C1-INH protein and activity, and low C4 levels.
112
What are the characteristics of Type I, II, and III angioedema related to C1-INH deficiency?
| Type | Characteristics |
|------|----------------|
| I | Lack C1-INH protein; decreased C1-INH activity and C4 |
| II | Lack C1-INH activity |
| III | Normal C1-INH; typically occurs in females; associated with gain of function mutation of Factor XII |
113
How does acquired angioedema differ from hereditary angioedema in terms of pathophysiology?
Acquired angioedema is primarily bradykinin-mediated and can develop due to overconsumption of C1-INH or presence of autoantibodies against C1-INH, while hereditary angioedema is due to genetic deficiencies in C1-INH.
114
What are the clinical features of Schnitzler syndrome and how does it relate to urticaria?
Schnitzler syndrome is characterized by urticaria-like eruptions, monoclonal IgM or possibly IgG gammopathy, systemic symptoms such as fever and bone/muscle pain, and histology resembling urticarial vasculitis or neutrophilic urticaria.
115
What is the significance of gene analysis in diagnosing Cryopyrin Associated Periodical Syndrome?
Gene analysis is the most definitive method for diagnosing Cryopyrin Associated Periodical Syndrome, identifying gain of function mutations in NLRP3 (CIASI) that lead to overproduction of IL-1.
116
What distinguishes exercise-induced anaphylaxis (EIA) from food-dependent exercise-induced anaphylaxis (FDEIA)?
EIA is characterized by development of urticaria and anaphylaxis regardless of food intake, while FDEIA occurs only when exercising after eating certain foods.
117
What types of inflammatory cells are involved in the edema of the dermis and perivascular areas in urticaria?
The inflammatory cells involved include lymphocytes, eosinophils, and neutrophils.
118
What is the significance of neutrophil infiltration in urticaria?
Neutrophil infiltration is especially prominent in acute urticaria and physical urticarias, indicating an inflammatory response.
119
What role do extracellular eosinophil major basic protein play in urticaria?
Extracellular eosinophil major basic protein is frequently deposited in spontaneous wheals, indicating its involvement in the inflammatory process.
120
What histological evidence is associated with urticarial vasculitis?
Urticarial vasculitis is characterized by histologic evidence of vasculitis (venulitis).
121
What findings may be sufficient for a diagnosis in difficult cases of urticaria?
In difficult cases, leukocytoclasis or fibrin deposition with or without erythrocyte extravasation may be sufficient for a diagnosis.
122
What is the significance of neutrophil infiltration in acute urticaria and physical urticarias?
Neutrophil infiltration is prominent in acute urticaria and physical urticarias, indicating an inflammatory response.
123
How do infiltrated lymphocytes in urticaria express cytokines?
Infiltrated lymphocytes in urticaria express cytokines such as IL-4, IL-5, and IFN-γ, suggesting a complex immune response.
124
What histological features are associated with Neutrophilic Urticaria?
Neutrophilic Urticaria is characterized by urticaria-like eruptions that persist for longer than a day and involve predominantly neutrophilic infiltration.
125
What role does extracellular eosinophil major basic protein play in urticaria, particularly in spontaneous wheals?
Extracellular eosinophil major basic protein is frequently deposited in spontaneous wheals, indicating its potential role in the pathophysiology of urticaria.
126
What are the histological findings in Urticarial Vasculitis?
Urticarial Vasculitis shows histologic evidence of vasculitis (venulitis), aiding in diagnosis.
127
What imaging techniques may be useful in cases of edema related to bradykinin-mediated angioedema?
Imaging techniques such as endoscopy, radiographs, CT scans, and MRI examinations may be useful.
128
What are the two basic approaches to managing urticaria?
1. Identification and removal of the cause. 2. Continuous use of antisymptomatic treatment with medications.
129
What factors are associated with a prolonged prognosis in patients with chronic spontaneous urticaria (CSU)?
Factors include initial disease severity, presence of angioedema, combination of CSU and physical urticaria(s), and autoreactivity.
130
What treatments are ineffective for mast cell-mediated urticaria?
Antihistamines, corticosteroids, and adrenaline are ineffective in controlling symptoms without identifying the cause.
131
What alternative treatments may be considered for refractory cases of urticaria?
Alternative treatments may include histamine H2-blockers, dapsone, antifibrinolytics, methotrexate, tacrolimus, hydroxychloroquine, IVIg, plasmapheresis, and narrow-band UVB.
132
What is the reported improvement rate for patients with acute spontaneous urticaria after treatment within one week?
Approximately 85% of patients improve shortly after treatment.
133
What type of angioedema is likely if a patient reports no response to antihistamines, corticosteroids, or adrenaline?
This is likely bradykinin-mediated angioedema. Treatments like icatibant or ecallantide should be considered.
134
What are the two basic approaches to managing urticaria, and how do they differ?
1. Identification and removal of the cause focuses on eliminating aggravating factors. 2. Continuous use of antisymptomatic treatment involves ongoing medication.
135
In cases of bradykinin-mediated angioedema, what treatments are considered ineffective?
Antihistamines, corticosteroids, and adrenaline are ineffective.
136
What alternative treatments may be considered for refractory cases of urticaria, and what are their potential risks?
Alternative treatments may include histamine H2-blockers, dapsone, antifibrinolytics, methotrexate, tacrolimus, hydroxychloroquine, IVIg, plasmapheresis, and narrow-band UVB. These treatments have weak evidence supporting their efficacy and may cause serious side effects.
137
What is the reported improvement rate for patients with chronic spontaneous urticaria (CSU) at 60 months?
The reported improvement rate at 60 months is 66.1%.
138
What is the mainstay of treatment for all kinds of mast cell-mediated urticaria?
Second-generation nonsedating antihistamines are the mainstay of treatment.
139
What is recommended for intractable cases of urticaria resistant to standard doses of antihistamines?
Increased dosing of antihistamines up to fourfold is recommended.
140
What is the third line therapy for intractable chronic spontaneous urticaria (CSU)?
Omalizumab is the third line therapy, administered at 300 mg/month.
141
What precautions should be taken regarding systemic treatment in pregnant women?
Systemic treatment should generally be avoided in pregnant women, especially in the first trimester.
142
What is recommended for family members of patients with HAE type I and type II?
All family members older than 1 year old are recommended to undergo screening for the diagnosis of HAE.
143
What is the recommended first-line treatment for all kinds of mast cell-mediated urticaria?
Second-generation nonsedating antihistamines are the mainstay of treatment.
144
What is the recommended approach for intractable cases of urticaria that are resistant to standard doses of antihistamines?
Increased dosing of antihistamines up to fourfold is recommended.
145
What is the role of Omalizumab in the treatment of chronic spontaneous urticaria (CSU)?
Omalizumab is a third-line therapy for intractable CSU, effective for virtually all subtypes of urticaria mediated by mast cells.
146
What precautions should be taken when treating pregnant women with antihistamines?
Systemic treatment should generally be avoided in pregnant women.
147
What is the role of Omalizumab in the treatment of chronic spontaneous urticaria (CSU)?
Omalizumab is a third-line therapy for intractable CSU, administered at **300 mg/month** regardless of serum total IgE levels. It inhibits circulating IgE and shows clinical effects within **1 week**, effective for virtually all subtypes of urticaria mediated by mast cells.
148
What precautions should be taken when treating pregnant women with antihistamines?
Systemic treatment should generally be avoided in pregnant women, especially in the **first trimester**. Antihistamines cross the placenta, and while there is no reliable evidence that the medications are teratogenic, treatment principles are suggested for both pregnant and lactating women with urticaria.
149
What screening is recommended for family members of patients with hereditary angioedema (HAE)?
All family members of patients with **HAE type I and type II** who are older than **1 year old** are recommended to undergo screening for the diagnosis of HAE.
150
Most common subtype of physical urticaria?
Symptomatic dermographism (urticaria factitia / dermographic / mechanical urticaria)
151
Most frequent sign of anaphylaxis?
Wheal and flare
152
T or F: Mast cell-/non-bradykinin-mediated angioedema usually occurs in the larynx.
False. Mast cell-/non-bradykinin-mediated angioedema does not usually occur in the larynx.
153
T or F: Bradykinin-mediated angioedema does not accompany superficial wheals.
True.
154
Wheals of inducible urticaria do not usually last for more than _____, except for those of _____.
4 hours
Deep pressure urticaria
155
Potential triggers for episodic/acute urticaria?
156
Diagnostics for urticaria and angioedema?
157
There is a strong association between HLA-___ and patients with CSU with functional autoantibodies against IgE or FcεRI.
DR4
158
Risk factors for having a latex allergy?
Health care workers
Atopic dermatitis
Spina bifida
159
Role of ACE inhibitors in angioedema?
Angiotensin-converting enzyme (ACE) inhibitors do not cause release or production of mediators by themselves but inhibit degradation of bradykinin which causes angioedema.
160
Acquired angioedema is caused by overconsumption of _____ caused by _____.
C1-INH
myeloproliferative diseases
161
_____ are the mainstay of pharmaceutical therapy for urticaria and angioedema.
Nonsedative second-generation antihistamines
162
Medication recommended for intractable CSU?
Omalizumab 300 mg/month. Clinical effects may become apparent within 1 week.
163
Treatment for acute attacks of and prophylaxis against bradykinin-mediated HAE?
Acute attacks:
Icabitant
Ecallantide
Purified or recombinant C1-INH (Lanadelumab)
Prophylaxis:
Purified or recombinant C1-INH (Lanadelumab)
BCX7653
164
_____ and _____ for urticaria in pregnant women are desirably used after the 1st trimester.
Cetirizine
Loratadine
165
Describe the treatment algorithm for chronic urticaria.
166
4 factors associated with prolonged prognosis of disease?
1. Initial disease severity
2. Angioedema
3. Combination of CSU and physical urticarias
4. Autoreactivity (positive ASST result)
167
Prognosis of acute and chronic urticaria?
