Chapter 38 :: Urticaria and Angioedema

:: Allen P. Kaplan
.IJRTJCARIA AND ANGIOEDEMA
ATA GLANCE
Occurs acutely at some time in 20% of the population; incidence of chronic urticaria/ angloedeina is approximately 0.5%.
Acute Ur&aria/angjoede is caused by drugs, foods, occasionaily infection in association with immunoglobulin E-dependent
mechajms (allergy), or metabolic factors.
Chronic urticaria/a.ngioedema is an
autoimmune disorder in 45% of patients.
In the absence of urticaria, angioedema can be due to overproduction or impaired breakdown of bradykinin.
Treatment of acute urticaria/angioedema relies on antihjstamjne and short courses of corticosteroids, and identification and
elimination of endogenous and exogenous causes.
Treatment of Cl inhibitor deficiency includes androgenic agents, antifibrinolytic agents, and Cl inhibitor (Cl INH)
concentrates, a kallikrejn inhibitor, and bradykinin receptor antagonist.
Treatment of physical Urticaria/angioedema includes high-dose antthistan-jne prophylaxis, except for delayed pressure
urticaria.
Treatment of chronic idiopathic or
autoim.mune urticaria/angioedema includes antihistamines (nonsedating preparations primarily), low-dose daily or alternate
day corticosteroids, or cyclosporine.
Urticaria is defined as a skin lesion consisting of a wheal-and-flare reactiOn in which localized intracutan eous edema
(wheal) is surrounded by an area of redn ess (erythema) that is typically pruritic. Individual hives can last as briefly as 30
minutes to as long as 36 hours. They can be as small as a millimeter or 68 inches in diameter (giant urticaria). They blanch
with pressure as the dilated blood vessels are compressed, which also accounts for the central pallor of the wheal. The dilated
blood vessels and increased permeability that characterize urticaria are present in the superficial dermis and involves the
venular plexus in that locat ion. Angioedema can be caused by the same pathog enic mechanisms as urticaia but the
pathology is in

the deep dermis and subcutaneous tissue and swelling is the major manifestation. The overlying skin may be erythematous or
normal. There is less pruritus (fewer type C nerve endings at the deeper Cutaneous levels) but there may be pain or burning.

EPIDEMIOLOGY
Urticaria and angioedema are common. Age, race, sex, occupation, geographic location, and season of the year may be
implicated in urticaria and angioedema only insofar as they may contribute to exposure to an elici ting agent. Of a group of
college students, 15%20% reported having experienced urticaria, while 1 %3% of the patients referred to hospital
dermatology clini cs in the United Kingdom noted urticaria and angio edema. In the National Ambulatory Medical Care
Survey data from 1990 to 1997 in the United States, women accounted for 69% of patient visits. There was a bimodal age
distribution in patients aged birth to 9 years and 30-40 years.1
Urticaria/angioedema is considered to be acute if it lasts less than 6 weeks. Most acute episodes are due to adverse reactions
to medications or foods and in child ren, to viral illnesses. Episodes of urticaria/angjo edema persisting beyond 6. weeks are
considered chronic and are divided into two major subgroups: (1) chronic autojmmujje urticarja (45%) and (2) chronic
idiopathic urticaria (55%) with a combined incid ence in the general population of Q5%2 Physically induced
urticaria/angioedema is not included in the definition. Various types of physical urticaria/angio edema may last for years, but
the individual lesions last fewer than 2 hours (except delayed pressure urtic aria) and are intermittent. Whereas 85% of
children experience urticaria in the absence of angioedema, 40% of adult patients with urticaria also experience angioedema.

Approximately 50% of patients with chronic urtic aria (with or without angioedema) are free of lesions within 1 year, 65%
within 3 years, and 85% within 5 years; fewer than 5% have lesions that last for more than 10 years. Angioedema alters the
natural history, and only 25% of patients experience resolution of lesions within 1 year. There are no data regarding the
remission rate in patients with only angioedema, The hereditary group is considered to be life long once the diagnosis
becomes clinically manifest.
PATHOGENESIS
The mast cell is the major effector cell in most forms of urticaria and angioedema, although other cell types undoubtedl.y
contribute. Cutaneous mast cells adhere