- Author: Sarah Beggs; Chief Editor: Dirk M Elston, MD more...
Pressure urticaria is an uncommon form of physical urticaria, a subset of chronic urticaria, which presents with erythematous swelling at sites of pressure. Patients who have urticaria for more than 6 weeks are given the diagnosis of chronic urticaria. This distinction is important as an inciting event or etiology is not identified for the majority of patients with chronic urticaria—hence, the term chronic idiopathic urticaria (CIU) is often used. A proportion of patients diagnosed with chronic urticaria have physical urticaria, which is urticaria incited by a physical stimulus, such as mechanical (friction, vibration, pressure), thermal, or electromagnetic waves.
Pressure urticaria may occur immediately (within minutes) after a pressure stimulus. However, more commonly, erythema and cutaneous (and often subcutaneous) edema develops 4-6 hours after a pressure stimulus. For this reason, the term delayed pressure urticaria (DPU) is commonly used. The reaction may last up to 72 hours and can be associated with pruritus, burning, and pain. Pressure sites, including the hands, feet, trunk, buttocks, and legs, are most commonly affected. Lesions can be induced by a variety of stimuli, including standing, walking, wearing of tight clothes, and sitting or leaning on a hard surface.
For further information on urticaria, see Contact Syndrome Urticaria, Dermographism Urticaria, and Solar Urticaria. For patient education resources, see the Allergy Center and the Skin, Hair, and Nails Center, as well as Hives and Angioedema.
The pathogenesis of delayed pressure urticaria (DPU) is relatively unknown. Although the trigger stimulus is typically identified, no allergen has been established. The general pathogenesis of urticaria (hives) can be mediated immunologically or nonimmunologically; usually antibodies against immunoglobulin E (IgE) molecules are involved. IgE molecules bind to mast cells. When an antibody or autoantibody attaches to the antigen-binding site of the IgE molecule, a bridge is formed between two or more IgE molecules. This induces mast cell degranulation, releasing multiple proinflammatory mediators, including histamine. In chronic idiopathic urticaria (CIU), the mast cells are inappropriately activated.
In DPU, mast cells and histamine are believed to play a role in the disease pathogenesis. Injections of compound 48/80, a mast cell degranulator, lead to the formation of wheals in patients with DPU both 15 minutes after injection and at 6-8 hours after injection. Injections in patients with chronic urticaria, but without DPU, produce wheals at 15 minutes that diminish over time. Histamine injections in both patients lead to initial wheal formation with no late-phase reaction.
Histamine levels are increased in the lesional skin, while intracellular histamine levels are decreased in peripheral white blood cells. There is also an increased stimulation of histamine release. Despite these findings, histamine is unlikely to be the sole mediator in pressure urticaria. This is further demonstrated in the relative unresponsiveness of pressure urticaria to antihistamine treatment.
Other mediators are believed to be involved. This includes eosinophils (as suggested by the presence of eosinophilia), eosinophil cationic protein (ECP), and eosinophil cationic factor (ECF) found in biopsy specimens from some patients with DPU, particularly bullous DPU. In addition, elevated concentrations of interleukin (IL)–1a, IL-5, and IL-6; tumor necrosis factor (TNF)–alpha; and leukotrienes have also been found in lesional skin of pressure urticaria patients.[10, 11, 12] Abnormalities in platelets and fibrin or fibrinolysis have also been investigated.[13, 14] Systemic inflammation has also been suggested with elevation seen of C-reactive protein (CRP) and sCD40L, a platelet activator.[15, 16]
Pressure stimuli may include the following:
Standing, walking, or sitting on a hard surface
Using tools (eg, a screwdriver or a hammer)
Carrying a handbag
Wearing tight-fitting clothes (eg, bra straps, belts, shoes, cuffs, or watches)
Occasionally, delayed pressure urticaria (DPU) is aggravated by heat, aspirin, or menstruation. Exacerbation of the condition during medical procedures is a reasonable possibility; urticaria flares following endoscopy have been described.
Delayed pressure urticaria (DPU) is generally considered a rare entity; some investigators suggest that it is less uncommon than is usually assumed but appears to be rare because it is not consistently recognized. One study of 2310 patients with urticaria seen over 32 years found the prevalence of DPU to be 2%. The peak age of onset of DPU is in the 20s and 30s (range, 5-63 y). DPU is slightly more common in men than in women.
Delayed pressure urticaria (DPU) is a chronic disease that can last for years (mean, 9 y; range, 1-40 y). One study reported that 28% and 48% of patients with DPU were free of lesions after 5 and 10 years, respectively. The morbidity of DPU varies, depending on the severity and the response to treatment. In some patients, this condition can be disabling, especially in patients who perform manual labor.
Quality-of-life (QOL) tools have demonstrated that patients with urticaria can show impairments in QOL scores similar to those seen in patients with cardiac disease or chronic dermatoses such as psoriasis and atopic eczema. QOL scores were lowest for energy, social isolation, emotional reaction, and sleep disturbance. The dimension of daily living activities was more profoundly impaired in patients with chronic idiopathic urticaria (CIU) than in those with atopic eczema or psoriasis.
Barlow RJ, Warburton F, Watson K, Black AK, Greaves MW. Diagnosis and incidence of delayed pressure urticaria in patients with chronic urticaria. J Am Acad Dermatol. 1993 Dec. 29(6):954-8. [Medline].
Abajian M, Schoepke N, Altrichter S, Zuberbier T, Maurer M. Physical urticarias and cholinergic urticaria. Immunol Allergy Clin North Am. 2014 Feb. 34(1):73-88. [Medline].
Commins SP, Kaplan AP. Immediate pressure urticaria. Allergy. 2002 Jan. 57(1):56-7. [Medline].
Cassano N, Mastrandrea V, Vestita M, Vena GA. An overview of delayed pressure urticaria with special emphasis on pathogenesis and treatment. Dermatol Ther. 2009 Nov. 22 Suppl 1:S22-6. [Medline].
Lawlor F, Black AK. Delayed pressure urticaria. Immunol Allergy Clin North Am. 2004 May. 24(2):247-58, vi-vii. [Medline].
Jain S. Pathogenesis of chronic urticaria: an overview. Dermatol Res Pract. 2014. 2014:674709. [Medline].
Mekori YA, Dobozin BS, Schocket AL, Kohler PF, Clark RA. Delayed pressure urticaria histologically resembles cutaneous late-phase reactions. Arch Dermatol. 1988 Feb. 124(2):230-5. [Medline].
Kaplan AP, Horakova Z, Katz SI. Assessment of tissue fluid histamine levels in patients with urticaria. J Allergy Clin Immunol. 1978 Jun. 61(6):350-4. [Medline].
Kerstan A, Rose C, Simon D, et al. Bullous delayed pressure urticaria: pathogenic role for eosinophilic granulocytes?. Br J Dermatol. 2005 Aug. 153(2):435-9. [Medline].
Lawlor F, Bird C, Camp RD, et al. Increased interleukin 6, but reduced interleukin 1, in delayed pressure urticaria. Br J Dermatol. 1993 May. 128(5):500-3. [Medline].
Frezzolini A, De Pità O, Cassano N, D'Argento V, Ferranti G, Filotico R. Evaluation of inflammatory parameters in physical urticarias and effects of an anti-inflammatory/antiallergic treatment. Int J Dermatol. 2002 Jul. 41(7):431-8. [Medline].
Di Lorenzo G, Pacor ML, Mansueto P, et al. Is there a role for antileukotrienes in urticaria?. Clin Exp Dermatol. Mar 2006. 31(3):327-34.
Kasperska-Zajac A, Brzoza Z, Rogala B. Increased concentration of platelet-derived chemokines in serum of patients with delayed pressure urticaria. Eur Cytokine Netw. 2008 Jun. 19(2):89-91. [Medline].
Kasperska-Zajac A, Jasinska T. Analysis of plasma D-dimer concentration in patients with delayed pressure urticaria. J Eur Acad Dermatol Venereol. 2011 Feb. 25(2):232-4. [Medline].
Kasperska-Zajac A, Jasinska T, Grzanka A, Kowalik-Sztylc A. Markers of systemic inflammation in delayed pressure urticaria. Int J Dermatol. 2013 Mar. 52(3):309-10. [Medline].
Jasinska T, Grzanka A, Machura E, Kasperska-Zajac A. Is delayed pressure urticaria associated with increased systemic release of sCD40L?. Biomed Res Int. 2013. 2013:823798. [Medline].
Czecior E, Grzanka A, Kurak J, Misiolek M, Kasperska-Zajac A. Late Dysphagia and Dyspnea as Complications of Esophagogastroduodenoscopy in Delayed Pressure Urticaria: Case Report. Dysphagia. 2011 Jun 5. [Medline].
Champion RH. Urticaria: then and now. Br J Dermatol. 1988 Oct. 119(4):427-36. [Medline].
Dover JS, Black AK, Ward AM, Greaves MW. Delayed pressure urticaria. Clinical features, laboratory investigations, and response to therapy of 44 patients. J Am Acad Dermatol. 1988 Jun. 18(6):1289-98. [Medline].
Grob JJ, Gaudy-Marqueste C. Urticaria and quality of life. Clin Rev Allergy Immunol. 2006 Feb. 30(1):47-51. [Medline].
Morioke S, Takahagi S, Iwamoto K, Shindo H, Mihara S, Kameyoshi Y. Pressure challenge test and histopathological inspections for 17 Japanese cases with clinically diagnosed delayed pressure urticaria. Arch Dermatol Res. 2010 Oct. 302(8):613-7. [Medline].
Barlow RJ, Ross EL, MacDonald D, Black AK, Greaves MW. Adhesion molecule expression and the inflammatory cell infiltrate in delayed pressure urticaria. Br J Dermatol. 1994 Sep. 131(3):341-7. [Medline].
Barlow RJ, Ross EL, MacDonald DM, Kobza Black A, Greaves MW. Mast cells and T lymphocytes in chronic urticaria. Clin Exp Allergy. 1995 Apr. 25(4):317-22. [Medline].
Magerl M, Borzova E, Giménez-Arnau A, Grattan CE, Lawlor F, Mathelier-Fusade P. The definition and diagnostic testing of physical and cholinergic urticarias--EAACI/GA2LEN/EDF/UNEV consensus panel recommendations. Allergy. 2009 Dec. 64(12):1715-21. [Medline].
Zuberbier T, Bindslev-Jensen C, Canonica W, et al. EAACI/GA2LEN/EDF guideline: management of urticaria. Allergy. 2006 Mar. 61(3):321-31. [Medline].
Lawlor F, Black AK, Ward AM, Morris R, Greaves MW. Delayed pressure urticaria, objective evaluation of a variable disease using a dermographometer and assessment of treatment using colchicine. Br J Dermatol. 1989 Mar. 120(3):403-8. [Medline].
Vena GA, Cassano N, D'Argento V, Milani M. Clobetasol propionate 0.05% in a novel foam formulation is safe and effective in the short-term treatment of patients with delayed pressure urticaria: a randomized, double-blind, placebo-controlled trial. Br J Dermatol. 2006 Feb. 154(2):353-6. [Medline].
Liu RH, Werth VP. What is new in the treatment of steroid-induced osteoporosis?. Semin Cutan Med Surg. 2007 Dec. 26(4):203-9. [Medline].
Maurer M, Rosén K, Hsieh HJ, Saini S, Grattan C, Gimenéz-Arnau A, et al. Omalizumab for the treatment of chronic idiopathic or spontaneous urticaria. N Engl J Med. 2013 Mar 7. 368(10):924-35. [Medline].
Metz M, Ohanyan T, Church MK, Maurer M. Retreatment with omalizumab results in rapid remission in chronic spontaneous and inducible urticaria. JAMA Dermatol. 2014 Mar. 150(3):288-90. [Medline].
Hartmann K, Hani N, Hinrichs R, Hunzelmann N, Scharffetter-Kochanek K. Successful sulfasalazine treatment of severe chronic idiopathic urticaria associated with pressure urticaria. Acta Derm Venereol. 2001 Jan-Feb. 81(1):71. [Medline].
Kozel MM, Sabroe RA. Chronic urticaria: aetiology, management and current and future treatment options. Drugs. 2004. 64(22):2515-36. [Medline].
Grundmann SA, Kiefer S, Luger TA, Brehler R. Delayed pressure urticaria - dapsone heading for first-line therapy?. J Dtsch Dermatol Ges. 2011 Nov. 9(11):908-12. [Medline].
Kulthanan K, Thumpimukvatana N. Positive impact of chloroquine on delayed pressure urticaria. J Drugs Dermatol. 2007 Apr. 6(4):445-6. [Medline].
Dawn G, Urcelay M, Ah-Weng A, O'Neill SM, Douglas WS. Effect of high-dose intravenous immunoglobulin in delayed pressure urticaria. Br J Dermatol. 2003 Oct. 149(4):836-40. [Medline].
Metz M, Altrichter S, Ardelean E, Kessler B, Krause K, Magerl M, et al. Anti-immunoglobulin E treatment of patients with recalcitrant physical urticaria. Int Arch Allergy Immunol. 2011. 154:177-80.
Mitzel-Kaoukhov H, Staubach P, Müller-Brenne T. Effect of high-dose intravenous immunoglobulin treatment in therapy-resistant chronic spontaneous urticaria. Ann Allergy Asthma Immunol. 2010 Mar. 104(3):253-8. [Medline].
Lenormand C, Lipsker D. Efficiency of interleukin-1 blockade in refractory delayed-pressure urticaria. Ann Intern Med. 2012 Oct 16. 157(8):599-600. [Medline].
Nettis E, Colanardi MC, Soccio AL, Ferrannini A, Vacca A. Desloratadine in combination with montelukast suppresses the dermographometer challenge test papule, and is effective in the treatment of delayed pressure urticaria: a randomized, double-blind, placebo-controlled study. Br J Dermatol. 2006 Dec. 155(6):1279-82. [Medline].
|1||Mild (< 20 wheals/24 h)||Mild|
|2||Moderate (21-50 wheals/24 h)||Moderate|
|3||Intense (>50 wheals/24 h) or large confluent areas||Intense|
|*Score = wheal score (0-3) + pruritus score (0-3); thus, the overall score for severity ranges from 0 to 6.|