Current reviews of allergy and clinical immunology
Chronic urticaria,☆☆

https://doi.org/10.1067/mai.2000.105706Get rights and content

Abstract

Chronic urticaria remains a major problem in terms of etiology, investigation, and management. It is important to identify patients in whom physical urticaria is the principal cause of disability. Once confirmed by appropriate challenge testing, no further investigation is required. Urticarial vasculitis (UV) is a major differential diagnosis of “idiopathic” urticaria (CIU). I perform biopsy of most patients in this category because UV cannot be considered confirmed in the absence of histologic evidence. Patients with confirmed UV need to be thoroughly investigated for paraproteins, lupus erythematosus hepatitis B and C, and inflammatory bowel disease. Of patients with CIU, a few (<5%) prove to have food additive reactivity confirmed by placebo-controlled challenge testing. There is no convincing evidence of the involvement of Helicobacter pylori or parasite infestation as a cause of chronic urticaria, although H pylori could have an indirect role. Recently it has become clear that 27% to 50% of patients with CIU have functional autoantibodies directed against the α-chain of the high-affinity IgE receptor or less commonly against IgG. These antibodies, whose involvement has now been independently confirmed in several centers, are identified by autologous serum skin testing and confirmed by histamine release studies or immunoblotting. Their removal (by intravenous Ig or plasmapheresis) or treatment by cyclosporine has proved highly beneficial in severely affected patients. However, the routine treatment of all CIU patients, irrespective of etiology, remains the judicious use of H1 antihistamines. (J Allergy Clin Immunol 2000;105:664-72.)

Section snippets

CLINICAL FEATURES OF CHRONIC URTICARIA

The cardinal clinical features of urticaria that distinguish it from any other type of inflammatory eruption are the repeated occurrence of short-lived cutaneous wheals accompanied by redness and itching (Fig 1).

. Chronic idiopathic urticaria.

Wheals are lesions ranging from a few millimeters to several centimeters in diameter, although if they run together and become confluent much larger plaques may occur. Individual wheals normally, by definition, last less than 24 hours, although there are

PHYSICAL URTICARIAS

It is most important to distinguish the physical urticarias from CIU. This is because, if it turns out that a physical urticaria is the main cause of chronic urticaria in an individual, it almost invariably obviates the necessity for investigation beyond any challenge testing necessary to confirm the diagnosis. There are rare exceptions; for example, it is desirable to exclude the (rare) presence of plasma cryoproteins in patients with cold urticaria. However, it is my everyday experience that

SYMPTOMATIC DERMOGRAPHISM (FACTITIOUS URTICARIA)

The diagnosis of symptomatic dermographism can be made by drawing the tip of a blunt-pointed instrument firmly across the skin. This causes an immediate linear red wheal that (in contrast to “ordinary” dermographism that can occur in a healthy person) manifests itching.

Any region of the body can be affected. The condition, which occurs at any age, runs on average a course of 2 to 3 years before resolving spontaneously. The wheals, which last for up to 30 minutes, fade, leaving no mark. Unlike

DELAYED PRESSURE URTICARIA

It is not generally appreciated how common delayed pressure urticaria is. Our results show that at least 40% of all patients with CIU have concurrent delayed pressure urticaria.7 Indeed, it is doubtful if it ever occurs in isolation. This explains the frequency of wheals at local pressure sites (waistband, palms, soles, etc) in CIU. It also explains the poor response to H1 antihistamines in some patients with CIU because delayed pressure urticaria is generally poorly responsive to this

COLD URTICARIA

There are a number of rare subtypes of cold urticaria, but for the purposes of this account only 2 subtypes need to be considered: primary acquired cold urticaria (“essential” cold urticaria) and secondary acquired cold urticaria. Compared with most other physical urticarias, these have been intensively studied.

CHOLINERGIC URTICARIA

In its milder presentations, cholinergic urticaria is probably the most common of all the physical urticarias. Often referred to trivially as “heat bumps,” it probably occurs at some time during the lives of at least 15% of the population. It has been the subject of several useful reviews.24, 25

Cholinergic urticaria is a physical urticaria predominantly in teenagers and younger adults and carries a good prognosis for eventual improvement, although I have had patients in whom troublesome

Clinical features

Conventionally, CIU is defined as the daily, or almost daily, occurrence of urticarial wheals for at least 6 weeks. Intermittent urticaria, although a common entity, is less well recognized. It consists of bouts of urticaria lasting days or weeks with intervals of days, weeks, or months in between. It will be considered jointly with classic CIU for the purposes of this discussion. Angioedema occurs concurrently with CIU in about 50% of cases1 and delayed pressure urticaria in about 40%.7

As

ETIOLOGY

The target cell for CIU and angioedema is the dermal mast cell, and any hypothetic etiological mechanism should explain how this cell becomes repeatedly and extensively activated, leading to release of histamine and other mediators. No doubt other cell types are also involved, including the basophil.37 Until recently there has been a paucity of convincing evidence-based causes. Chronic infection has frequently been cited—most recently Helicobacter pylori. However, recent reports have failed to

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    Series editor: Harold S. Nelson, MD

    ☆☆

    Reprint requests: Malcolm Greaves, MD, St John’s Institute of Dermatology, St John’s Hospital, United Medical and Dental School, Lambeth Palace Road, London SE1 7EH, United Kingdom; fax 44 171 401 2008.

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