Advanced Topics
The Wheal
An urticarial wheal results from release of histamine and other mediators from mast cells leading to an increase in capillary permeability with the escape of fluid from the circulation into the tissues. Mast cells degranulate as a result of physical, chemical, pharmacological and immunological stimuli. Ttype I hypersensitivity is mediated through the IgE attached to the mast cell which degranulates on exposure to the specific antigen.
Mast cells can degranulate by other signals. Chemical signals include drugs such as morphine, codeine, ethanol, polymyxin B; bacterial, plant and invertebrate toxins stimulate mast cells to degranulate directly. Physical signals include vibration, heat, sunlight, and cold.
In the biopsy of a typical urticarial lesion there is vasodilatation, dermal edema and perivascular infiltration of lymphocytes and eosinophils. Repeated skin biopsies show a predominance of neutrophils and eosinophils without endothelial damage, a "late phase reaction."
Chronic urticaria may signal occult infections and other systemic diseases. Infections such as a dental abscess or urinary tract infection have been reported to cause chronic urticaria.
CHOLINERGIC URTICARIA
An itchy eruption that develops in response to sweating, exercise, emotion and hot foods. Small wheals of less than 2 mm in diameter with surrounding red halo appear and.are more profuse on the upper trunk and proximal parts of the upper limbs. ssociated symptoms include faintness, headache, wheezing and palpitation. The prevailig theory is that increased temperature triggers a neural reflex, releasing acetylcholine from sympathetic nerve endings that triggers mast cells to degranulate. The diagnosis is established from the history and by finding the characteristic wheals during an attack. These lesions can often be reproduced by intradermal injection of cholinergic drugs, metholyl or acetylcholine.
PRESSURE URTICARIA
Skin redness, swelling and subcutaneous edema occur with sustained application of pressure to the skin. The lesions itch and burn and appear between 30 minutes to several hours after the pressure. This phenonenon is often associated with chronic urticaria. The lesions occur after sitting on hard chairs, carrying bags, leaning against furniture, wearing seat belts and lying on hard mattresses. Swelling of the feet and hands, often indistinguishable from angiooedema, occurs after activities such as weight lifting, walking, jogging, and running. During severe attacks, arthralgias and a flu-like illness may accompany the rash.
DERMOGRAPHISM
Dermographism is whealing after direct pressure on the skin. Linear wheals appear as a result of scratching. The itching and whealing reach their maximum in 5-10 minutes after the stimulus and disappear 30-60 minutes later. Lesions frequently appear in areas where clothing is tight and at sites of scratching
SOLAR URTICARIA
Solar urticaria is triggered by sun exposure and may be associated with other light-triggered eruptions, other urticaria and lupus erythematosus. It may also be symptomatic of porphyria cutanea tarda. Patients notice erythema, burning, and urticarial wheals within minutes following exposure to sunlight. Wheals can develop anywhere on the body, mostly in the sun exposed skin. The action spectrum of solar urticaria is broad, ranging from UVC, UVB, UVA to visible spectrum. Avoidance of sunlight is very important. The body should be covered with clothing and the patient should be advised to use an appropriate sunscreen. Antihistamines can give symptomatic relief and may be helpful as a rpeventive measure if taken before sun exposure.
COLD URTICARIA
Whealing occurs with exposure to cold. Characteristic urticaria appears on exposed areas on a cold day. Handling of cold objects causes immediate local reaction. Swelling of the mouth and oesophagus may occur on drinking cold water. Extensive cold urticaria may be associated with systemic symptoms such as faintness, wheezing and palpitations. Syncope can occur when the patient immerses in cold water. Diagnosis is established by placing an ice cube (wrapped in plastic bag) on the skin for 2 to 10 minutes. Wheals form on rewarming. In some cases, cold water at 7C is more effective in bringing out the wheal. Occasionally cold urticaria is associated with circulating cryoglobulin, cold haemolysin or cryofibrinogen.
Avoid swimming in cold water. Antihistamine treatment is partially effective - Cyproheptadine may be the drug of choice. Doxepin and ketotifen may also be useful.
AQUAGENIC URTICARIA
Brief contact of the skin with water of any temperature causes an immediate urticarial eruption on the site of contact. Aquagenic pruritus is a related but distinct condition in which brief contact of skin with water evokes intense local pruritus without any skin lesion - this is common in the elderly. Complete blood count should be checked as this condition may be symptomatic of polycythaemia rubra vera. Both disorder involve histamine release from skin mast cells and respond well to antihistamine.
VIBRATORY ANGIOEDEMA
Vibratory angioedema is an acute short-lived itchy swelling of the skin that occurs within minutes of application of a vibratory stimulus to the skin. The lesions can be reproduced by massage, scratching, shivering and running. Clapping, using a vibratory machine such as massage chair, and riding a motor bike may also produce lesions. The severity of symptoms is proportional to the intensity of the provoking stimulus. If the stimulus is sufficiently strong, facial and/or generalized erythema may occur. Headache and faintness are also reported. The urticarial response occurs within minutes of the stimulus, maximal at 5 minutes and disappears within an hour. Treatment with antihistamine is usually effective.
ANGIOEDEMA
Swelling reactions are called angioedema. Edema involves subcutaneous tissues rather than the skin itself and can involve any part of the body such as lips, eyelids, tongue and larynx. Hives and swelling may be associated. Hereditary Angioedema is caused by a deficiency of C1 esterase inhibitor and is inherited as an autosomal dominant trait. An acquired form of C1 esterase inhibitor may develop in patients with lymphoproliferative disorders and systemic lupus erythematosus.
Hereditary AngioedemaHive and swelling attacks are infrequent in childhood, common in adolescence and early adult life and may subside later. Lesions may affect the skin, mucosal surface and intestine. Subcutaneous swelling is not itchy and persists for a few days. Intestinal edema may causes acute abdominal pain and transient intestinal obstruction. Laryngeal edema may lead to upper airway obstruction and death. The Complement 2 (C2) and C4 levels are low in between attacks and C3 is normal. There is a low C1 esterase inhibitor level.
In acute airway obstruction, subcutaneous adrenaline may be life saving. Fresh frozen plasma should be administered by intravenous infusion or, alternatively a purified C1 esterase inhibitor concentrate can be given. For long term management, androgens usc as stanozolol or danazol can be used to stimulate hepatic synthesis of C1 inhibitor and may prevent swelling attacks. Antifibrinolytic agents such as tranexamic acid and epsilon aminocaproic acid are less effective as prophylaxis but can be tried in patient who do not tolerate androgens.
CONTACT URTICARIAS
Contact urticaria is a local immediate or delayed erythema or urticarial reaction at the site of epidermal or mucosal contact with a causative agent. It may be associated with generalized cutaneous reactions, rhinitis , asthma, or anaphylaxis. It is commonly an IgE mediated immediate reaction and non immunological mechanism is also possible. Probably the most important cause of contact urticaria is natural rubber latex present in gloves and other rubber products. Latex contact urticaria symptoms vary from mild itching to bronchial asthma, anaphylaxis, and death. Small molecular weight chemicals may cause contact urticaria. Chemicals, acting as haptens, for example ethylene oxide, isocyanates, chloramine-T, epoxy resins and nickel sulphate have caused IgE-mediated allergies. This can be confirmed by using skin prick testing.
URTICARIAL PIGMENTOSA
This condition is in fact not urticaria but is a disorder of mast cell proliferation. Clinically there is multiple guttate or larger pigmented macules on the trunk and limbs of the baby and urticated lesion may appear on rubbing the pigmented lesions. The biopsy of the skin shows increase in number of mast cells.
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Abstracts
Prevalence of IgE antibodies specific for food allergens in patients with chronic urticaria of unexplained etiology.
- Author
Kaeser P; Revelly ML; Frei PC Address Division of Immunology and Allergy, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland. Source Allergy, 49: 8, 1994 Sep, 626-9 Abstract We investigated whether some cases of chronic urticaria of unexplained cause might be related to food allergy which had remained undetected during routine examination. This investigation was undertaken as the consequence of the availability of a new in vitro assay for specific IgE with increased sensitivity. The following three groups of subjects were studied: 1) a control group of 60 nonatopic subjects, 2) 60 patients with allergy to perennial aeroallergens without skin involvement, and 3) 60 patients suffering from chronic urticaria with no evidence of any triggering factor despite careful clinical investigation. Specific IgE against 19 food allergens frequently involved in urticaria were investigated in all subjects with the new CAP System (Pharmacia). Positive results (CAP > 0.70 kU/l) for one or more food allergens were found in none of the nonatopic controls, in six of the subjects with respiratory allergy, and in 16 of the urticaria patients. The use of an in vitro test with an increased sensitivity allowed us to detect a significant prevalence of IgE specific for food allergens in patients with chronic urticaria of unknown origin. This suggests that, in several of those patients, chronic urticaria might be triggered by a food allergy undetected by the usual methods.
Food-induced contact urticaria syndrome (CUS) in atopic dermatitis: reproducibility of repeated and duplicate testing with a skin provocation test, the skin application food test (SAFT).
- Author
Oranje AP; Van Gysel D; Mulder PG; Dieges PH Address Sub-division of Paediatric Dermatology, University Hospital Rotterdam/Sophia & Dijkzigt, Belgium. Source Contact Dermatitis, 31: 5, 1994 Nov, 314-8 Abstract IgE-mediated contact urticaria syndrome (CUS) is one of the manifestations of allergy in childhood atopic dermatitis (AD). Allergens such as foods and animal products penetrate the skin easily. They can then cause urticarial reactions in sensitized individuals. A provocation test system for foods, called the skin application food test (SAFT), has been developed. Over more than 5 years, a group of 175 patients with AD was built-up and investigated in a prospective follow-up study with SAFT. SAFT was more frequently positive in AD children aged 0-2 years than in older children. In several children of this population (Group 1), we repeated SAFT within a period of 1 year. In another unrelated group of children (Group 2-1), we compared the results of 'original' SAFT and SAFT using square chambers (Van der Bend) or Silver patches. In the 3rd group (Group 2-2) we compared 'original' SAFT with SAFT using big Finn Chambers. The agreement between the tests was high: in Group 1, we observed 88 to 93% concordant scores, and in Group 2, the scores were 96% to 100%. Statistically, the kappa coefficient ranged from 0.71-0.87 in Group 1, and from 0.83-1.00 in Group 2. SAFT is therefore highly reproducible. Agreement was at least > or = 88% between the scores (the lowest kappa value observed was at least 0.71). Mechanisms in adverse reactions to food. The skin.
- Author
Sampson HA Address Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA. Source Allergy, 50: 20 Suppl, 1995, 46-51 Abstract Ingested food antigens rapidly cross the gastrointestinal barrier and reach pro-inflammatory cells in the skin. Food allergy provokes urticaria/angioedema by classical, Type I, IgE-mediated hypersensitivity. Food-induced atopic dermatitis is the result of non-classical, IgE-directed hypersensitivity involving resident mast cells, Langerhans cells, CD4+, TH2 lymphocytes and monocytes. A form of gluten sensitivity provokes a characteristic eczematous-like rash and enteropathy (Dermatitis herpetiformis).
Pirenzepine treatment in urticaria-angioedema syndrome caused by adverse reactions to foods.
- Author
- Ciprandi G; Perasso A; Marenco G; Santucci R; Buffa P; Cheli R; Canonica GW
- Address
- Semeiotica Medica R & Allergy Centre, Genoa, Italy.
- Source
- Allergol Immunopathol (Madr), 1989 Jul-Aug, 17:4, 189-92
- Abstract
- An increased gastroenteric mucosal permeability is generally considered a pathophysiological mechanism in the urticaria-angioedema syndrome caused by adverse reactions to foods. Since pirenzepine, an antimuscarinic receptor drug, exerts a cytoprotective activity on digestive mucosa, the authors evaluated the clinical efficacy of pirenzepine and terfenadine (antihistamine), alone or associated, in the treatment of patients with urticaria-angioedema syndrome due to food allergy. Furthermore, additional endoscopy and biopsy studies were performed in order to provide experimental evidence about the cytoprotective activity of this treatment. The results of the present investigation confirm the clinical efficacy, with improvement of histological parameters, of pirenzepine treatment in adverse reactions to foods, as previously demonstrated by our group, and suggest further investigations on the functional mucosal impairment hypothesized in this pathological condition.
Contribution to the etiopathogenesis of urticaria in children.
- Author
- Moreno MV; González de la Cuesta C; Oehling A
- Address
- Department of Allergology, Faculty of Medicine, University of Navarra, Pamplona, Spain.
- Source
- Allergol Immunopathol (Madr), 1988 Jul-Aug, 16:4, 225-30
- Abstract
- For hundreds of years urticaria has been an intriguing problem for researchers. Together with angioedema it constitutes a common condition that affects 20% of the general population. The etiologic diagnosis is obtained in a variable percentage of cases, according to the different studies published. The clinical course and the association with angioedema are also variables in the different works. It was the diversity of results that led us to undertake the present study. We selected 161 histories of children who came to our department of allergology; these children whose ages ranged from 1-12 years were diagnosed of urticaria and/or angio edema. The number of males was slightly higher than females and the most affected age group was that between 7-12 years. The acute and acute intermittent forms predominated especially in atopic children, highly associated with angioedema; chronic urticaria was less frequent. Within the etiologic factors, food allergy played an important role, followed by drug allergy. It was not possible to reach an etiologic diagnosis in 39.13% of cases.
Urticaria and angioedema: a clinical spectrum.
- Author
- Charlesworth EN
- Address
- Department of Allergy and Dermatology, Brenham Clinic Association, Texas, USA.
- Source
- Ann Allergy Asthma Immunol, 1996 Jun, 76:6, 484-95; quiz 495-9
- Abstract
- OBJECTIVE: The objective of this review is to give the reader a global insight into the spectrum of urticaria, focusing on differential diagnosis and pathogenic mechanisms. This review will define the role of the mast cell, explore a possible autoimmune basis for urticaria, and examine the purported role of food allergy in chronic urticaria. Last, the work-up and treatment of urticaria will be discussed in the context of the histologic diagnosis. STUDY SELECTION: The relevant past medical literature will be reviewed in the context of new and novel research into the mechanisms of chronic urticaria. RESULTS: Urticaria can be classified histopathologically into the following three categories: (1) neutrophilic vasculitis, (2) polymorphous perivascular infiltrate, and (3) sparse perivascular lymphocytic infiltrate. Each of the above histologic patterns correlates with a distinct clinical entity and the work-up and treatment of urticaria will be related to each of the above histologic types. CONCLUSIONS: Urticaria and angioedema are frustrating problems for both physicians and their patients; however, the problem can best be approached by considering urticaria as a symptom that may be part of a larger clinical spectrum. The physical examination and medical history remain the two most important pieces of information. The allergist frequently overlooks the value of a skin biopsy as an aid in sorting out the pathophysiology of urticaria and the biopsy results may help to classify urticaria into subgroups which respond differently to treatment.
Cell-mediated immune responses to artificial food additives in chronic urticaria.
- Author
- Warrington RJ; Sauder PJ; McPhillips S
- Source
- Clin Allergy, 1986 Nov, 16:6, 527-33
- Abstract
- In some cases of chronic urticaria it is suspected that food additives such as tartrazine and sodium benzoate or salicylates may play a role in the pathogenesis of the condition. Since, at times, chronic urticaria may appear histologically similar to a mild cell-mediated immune response, the release of the T cell-derived lymphokine leucocyte inhibitory factor (LIF), in response to incubation with these additives and with acetylsalicylic acid (ASA), was measured in vitro using cells from normal controls, from patients with chronic urticaria with or without clinically associated additive sensitivity and from patients with asthma with or without associated ASA sensitivity. It was found that significant production of LIF occurred in response to tartrazine and sodium benzoate in those individuals with chronic additive induced urticaria. In addition, tartrazine caused LIF release from mononuclear cells of ASA-sensitive asthmatics. These results may indicate a possible role for additive-induced cell-mediated immune responses in the pathogenesis of some cases of chronic urticaria and suggest a potential diagnostic test for this condition.
Food-induced contact urticaria syndrome (CUS) in atopic dermatitis: reproducibility of repeated and duplicate testing with a skin provocation test, the skin application food test (SAFT).
- Author
- Oranje AP; Van Gysel D; Mulder PG; Dieges PH
- Address
- Sub-division of Paediatric Dermatology, University Hospital Rotterdam/Sophia & Dijkzigt, Belgium.
- Source
- Contact Dermatitis, 1994 Nov, 31:5, 314-8
- Abstract
- IgE-mediated contact urticaria syndrome (CUS) is one of the manifestations of allergy in childhood atopic dermatitis (AD). Allergens such as foods and animal products penetrate the skin easily. They can then cause urticarial reactions in sensitized individuals. A provocation test system for foods, called the skin application food test (SAFT), has been developed. Over more than 5 years, a group of 175 patients with AD was built-up and investigated in a prospective follow-up study with SAFT. SAFT was more frequently positive in AD children aged 0-2 years than in older children. In several children of this population (Group 1), we repeated SAFT within a period of 1 year. In another unrelated group of children (Group 2-1), we compared the results of 'original' SAFT and SAFT using square chambers (Van der Bend) or Silver patches. In the 3rd group (Group 2-2) we compared 'original' SAFT with SAFT using big Finn Chambers. The agreement between the tests was high: in Group 1, we observed 88 to 93% concordant scores, and in Group 2, the scores were 96% to 100%. Statistically, the kappa coefficient ranged from 0.71-0.87 in Group 1, and from 0.83-1.00 in Group 2. SAFT is therefore highly reproducible. Agreement was at least > or = 88% between the scores (the lowest kappa value observed was at least 0.71).
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