angioedema urticaria

8/12/2019 Angioedema Urticaria

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Urticaria

Urticaria refers to a group of disorders affecting adults and children, in which

red patches and weals occur in the skin. A weal is a swelling of the surface

skin. It may be spelled 'wheal'. The name urticaria is derived from the

common European stinging nettle 'Urtica dioica'.

The skin swelling seen in urticaria is due to the release of chemicals such as

histamine from mast cells and basophils in the skin, which causes small

blood vessels to leak. The weals can be a few millimetres or several

centimetres in diameter, coloured white or red, often surrounded by a red

flare, and frequently itchy. Each weal may last a few minutes or several

hours, and may change shape. Weals may be round, or form rings, a map-

like pattern or giant patches.

The surface weals may be accompanied by deeper swelling ofeyelids,lips,

hands and elsewhere. The deeper swelling is

called angioedema.Angioedemamay occur with or without urticarial weals

(10%).

Rarely, urticaria results from an autoinflammatory disease such as

systemiclupus erythematosusorSchnitzler syndrome,or an inherited

condition such asMuckle-Wells syndromeor acryopyrin-associated periodic

syndrome.

Urticaria & angioedema

Image provided by Dr

Shahbaz A Janjua

More images of urticaria ...

Generalised ordinary urticaria
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Generalized ordinary urticaria (hives) presents with spontaneous weals

anywhere on the body. It is often classified according to how long it has been

present. Ordinary urticaria is also known as spontaneous urticaria or

idiopathic urticaria.

Acute urticaria is of recent onset (hours, days or a few weeks).

Episodic urticaria describes intermittent attacks of urticaria, which may

last a few days or a few weeks.

Chronic urticaria has persisted for several months or years.

Urticaria may not be present all the time. Some find it more noticeable at

certain times of day, or when they are warm or emotionally upset.

Acute urticariais sometimes due to allergy. Allergy depends on previous

exposure to the material, and the development of an immune reaction to it.

An immunoglobulin called IgE is involved, which attaches itself to a receptor

on the mast cell and causes it to release its chemical mediators.

The cause of an allergic urticaria may be:

Drug eruption: most often anantibiotic,but many other drugs have

been reported as well asvaccines.

Food allergy: tiny amounts of fish, eggs, nuts and kiwifruit (many

others have been reported less often).

Bee or wasp stings.

While most allergies involve ingestion,injection or inhalation of the

allergen, sometimes allergic urticaria can result from skin or mucosal

contact with an allergen e.g.rubber latex.

Most allergies are mild, but very allergic individuals may develop

serious anaphylactic shockwithin a few minutes of exposure. The most

frequent causes are antibiotic injections,bee stingsor ingestion of

peanuts.Anaphylaxisresults in urticaria, a tight chest, wheezing, faintness

and collapse. Medical attention must be sought urgently. A subcutaneous

adrenaline (epinephrine) injection will usually be given. Those prone to

anaphylaxis should carry an emergency supply (e.g. an EpiPen).

Most cases of urticaria are NOT due to allergy. Histamine and other

vasoactive chemicals can be released into the skin for many reasons. In

these cases urticaria can occur the first time that a person is exposed to the

material.

Non-allergic causes of acute urticaria include:
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Infection, including sinusitis,helicobacter(a cause of stomach ulcers),

dental abscess,viral hepatitis,mycoplasmapenumonia,infectious

mononuclosisandcandida(thrush).

Serum sickness,due to blood transfusion, viral infection or medicines

(e.g. Ceclor); urticaria is accompanied by fever, swollen lymph

glands, painful joints and nausea. It is thought to result from immune

complexes of the allergen and antibodies lodging in small blood

vessels.

Non-allergic release of mast-cell granules by medicines, especially

morphine, codeine, other opiates, and radiocontrast agents. Urticaria

provoked by aspirin and other non-steroidal anti-inflammatory drugs

usually involves leukotriene formation, although allergy involving IgE

antibodies may also occur with aspirin.

Non-allergic food reactions, from salicylates in fruit, azo dye food

colouring agents, benzoate preservatives and otherfood additives,orfrom histamine due to bacterial decomposition e.g.scombroid fish

poisoning.

Chronic urticariais often due to autoimmune disease (allergy to one's self),

and may be associated with other autoimmune conditions such as thyroid

disease and coeliac disease. Circulating anti-idiotypic antibodies can be

detected in 10% of patients with chronic urticaria. These activate IgE bound

on mast cells to cause excessive release of chemicals. More commonly there

is no evidence for autoantibodies, and the patient is said to have chronic

idiopathic urticaria. In most cases, chronic urticaria clears up, but 15% still

have wealing at least twice weekly after 2 years.

Recurrent angioedema without urticariamay be due to C1 esterase

deficiency (the protein C1 INH is missing or abnormal); there is often a

family history of similar problems. It may also be caused by angiotensin

converting enzyme (ACE) inhibitors such as captopril, quinapril, enalapril and

others, which are used to treat heart failure and hypertension. These drugs

inhibit kinin breakdown. Angioedema may also be idiopathic (of unknown

cause).

Urticaria should be distinguished fromurticarial vasculitis,in which weals

persist for longer than 24 hours andvasculitisis found onskin biopsy.It

results from immune complex deposition.

Physical urticaria

Physical urticaria refers to urticaria induced by external physical influences.

The weals take about 5 minutes to develop, and last 15 to 30 minutes. Some
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people suffer from a mixture of different types of physical urticaria and

generalized urticaria. The cause is unknown.

Dermographismmeans skin writing. Stroking the skin causes it to weal in

the line of the stroke. This is very itchy, but scratching causes more wealing.Dermographism usually starts quite suddenly. Weals come up where clothes

or furniture touch, especially when the affected person is hot or upset. A

warm shower followed by rubbing with a towel can result in itchy weals all

over.

Cholinergic urticariaresults from sweating. In severe cases, hundreds of tiny

red itchy spots develop after running, when warm, or when concentrating.

Cold urticariaaffects skin warming up after a reduction in temperature,

especially in winter. Weals can be widespread and may cause faintingattacks. Affected individuals should not expose large areas of the skin to the

cold or wind. They should be advised never to swim alone.

Contact urticariaresults from absorption of an elicting substance through the

skin or through a mucous membrane. It may be allergic or non-allergic in

origin. It may result in wealing confined to the site of contact or spreading

more widely. IgE antibodies on mast cells react to chemicals in white flour,

cosmetics, and textiles, or to proteins in latex rubber, saliva, meat, fish and

vegetables may cause contact urticaria. Non-allergic examples include the

stinging reaction of certain plants (e.g. nettles), animals (hairycaterpillar)

and medicines.

Localised heat urticaria, aquagenic urticaria (water contact),solar

urticaria(sunlight), vibratory angioedema anddelayed pressure urticariaare

less common.

Investigations in urticaria

In most cases of urticaria, there is no need for specific investigations.

However, the following tests may be helpful in some cases.

Full blood count to identify eosinophilia caused by allergy or parasitic

infestation, and low white blood count from systemic lupus

erythematosus.

Thyroid antibodies and function in chronic urticaria if autoimmune

origin is considered likely.

Skin prick testing and blood tests for specific allergy (RAST, or

radiollergosorbent tests, or CAP fluoroimmunoassay).

Autologous serum skin prick test (if available) in chronic urticaria.
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Complement tests in case of angioedema without urticaria or urticarial

vasculitis.

Skin biopsy if weals are prolonged, to identify vasculitis.

Treatment of urticaria

Treatment depends on the type of urticaria, its severity and how long it has

been present. If a medicine is thought to be the cause, it should be stopped.

Oral antihistaminescontrol wealing and itching for the majority of patients

with urticaria. The effective dose is variable. Antihistamines do not affect the

underlying cause of the rash. They may need to be taken intermittently or

continuously until the underlying tendency to urticaria disappears. Luckily,

most people eventually recover.

Non-sedating antihistamines include:

loratadine

desloratadine

fexofenadine

levocetirizine

cetirizine.

Cetirizine is the quickest acting antihistamine, and desloratadine is the most

long-lasting.

Conventional antihistamines such as chlorpheniramine or promethazine may

be preferred at night as they tend to have a sedative effect. Hydroxyzine or

diphenhydramine may be taken during the day and in some people they

appear more effective than newer, non-sedating antihistamines.

Response and tolerance varies, so if the first antihistamine is not effective,

consult your doctor. You may need to increase the dose up to four times (dothis slowly in case of unexpected sedation), or use a different drug. Usually

any sedative effect wears off in a week or so. Sometimes a combination of

antihistamines works better than a single type alone.

Other treatments may be tried for urticaria that fails to clear with

antihistamines. A combination of medicines may be required.

Off-license addition of H2 blockers, such as cimetidine, famotidine or

ranitidine, can also reduce urticaria but these medications are more

often prescribed to reduce stomach acidity.
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Oral steroids(prednisone) in moderate dose for a few days are useful

for severe acute urticaria. They are rarely recommended long term

because of serious adverse effects. In rare cases, alternate day

steroids may be warranted.

Tricyclic medications such asamitriptyline,nortriptyline and doxepin

are thought to help because of antihistaminic and neuropathic

properties.

Antileukotriene agents,such as montelukast and zileuton, occasionally

help; they are usually prescribed for asthma.

Antimalarials such ashydroxychloroquinemay be used.

Ultraviolet radiationtreatment (narrowband UVBandPUVA)reduces

the severity of wealing in some patients.

Antibiotics,dapsone,sulfasalazine,mesalazine andantifungal

agentsare used to clear an assumed underlying infection or for theirpresumed anti-inflammatory action.

Some patients respond tocolchicine.

Immunosuppressive medications. The most effective of these appears

to beciclosporin.Methotrexate,mycophenolate,

plasmapheresis,intravenous immunoglobulinsand off-label use of TNF-

alpha inhibitors (biologicssuch

asinfliximab,adalimumabandetanercept) are reported to help but

may have serious side effects.

Antifibrinolytic agents (tranexamic acid, androgenetic steroids such as

danazol) are mainly used for treatment-resistant angioedema orangioedema due to C1 esterase inhibitor deficiency. In emergency

situations, C1 esterase inhibitor concentrate or fresh frozen plasma

may be transfused.

Abiologic agent,omalizumabhas been reported to be effective for

chronic idiopathic urticaria. This drug is a monoclonal antibody that

targets IgE and affects mast cell and basophil function. It is currently

registered for the treatment of allergic asthma, and is not funded by

PHARMAC in New Zealand.

Intramuscular injection of adrenaline (epinephrine) is reserved for life-

threateninganaphylaxisor swelling of the throat.

Desensitisation to certain essential medications has been reported to be

effective in the case of urticaria caused by drug allergy. This involves careful

monitoring with medical supervision in case the procedure causes

anaphylaxis. A tiny dose of the drug is taken, with repeated and increasing

doses over several hours to days.

General measures useful for urticaria
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Do not take the medications your doctor has told you to avoid.

Minimise or avoid use of aspirin and codeine. It is usually safe to take

paracetamol to relieve pain.Nonsteroidal anti-inflammatoriesshould

be avoided in those that react adversely to aspirin. ACE inhibitors

should be avoided in those with angioedema.

Dietary changes may help. Some urticaria is aggravated by salicylates

in certain fruits, or additives including amines, tartrazine (102),

benzoates (210-220)and other food chemicals. Whether or not these

need to be avoided can be determined by appropriate food challenge

tests.

Avoid alcohol (it causes the surface blood vessels to dilate). Try not to

overheat. Cool the affected area with a fan, cold flannel, ice pack or

soothing moisturising lotion.
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