Dermographism Urticaria

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Background

The term dermographism (or dermatographism) literally means writing on the skin. Firm stroking of the skin produces an initial red line (capillary dilatation), followed by an axon-reflex flare with broadening erythema (arteriolar dilatation) and the formation of a linear wheal (transudation of fluid/edema); these events are collectively termed the triple response of Lewis.

An exaggerated response to this constitutional whealing tendency is seen in approximately 2-5% of the population and is referred to as dermographism. In a minority of people, it is accompanied by itching (symptomatic dermographism).

Dermographism should be distinguished from other types of urticaria. See the following articles:

Pathophysiology and Etiology

The exact mechanism of dermographism remains uncertain. Trauma may release an antigen that interacts with the membrane-bound immunoglobulin E (IgE) of mast cells, which release inflammatory mediators, particularly histamine, into the tissues. This process causes small blood vessels to leak, allowing fluid to accumulate in the skin. Other mediators that may be involved are leukotrienes, heparin, bradykinin, kallikrein, and peptides such as substance P.

Symptomatic dermographism is usually idiopathic, though it may have an immunologic basis in some patients. Passive transfer of the dermographic response with IgE- or immunoglobulin M (IgM)-containing serum has been reported, but no allergen has been identified.

Symptomatic dermographism may be triggered by drugs (eg, penicillin), an insect bite, Helicobacter pylori infection, or an infestation (eg, scabies, Fasciola hepatica). Congenital symptomatic dermographism has been described as the first sign of systemic mastocytosis.[1]

Approximately 75% of patients with hypereosinophilic syndrome, which has multisystem involvement and high mortality, have dermographism. Psychological factors and a history of stressful life events have been implicated as triggering factors in 30% of patients.[2] However, a small prospective study showed no alteration in dermographic reaction after social stress provocation tests.[3]

One case report describes symptomatic dermographism secondary to trauma from a coral reef.[4] Symptomatic dermographism may be a presenting feature of dermatomyositis.[5]

Epidemiology

Dermographism is the most common of the physical urticarias and can occur with other forms of urticaria. An increased incidence has been reported during pregnancy (especially in the second half), at the onset of menopause, in atopic children, and in patients with Behçet disease.[6]

Dermographism can appear in persons of any age but is more common in young adults; the peak incidence is in the second and third decades. Whether a sexual variance in prevalence occurs is unclear. None has been consistently reported, though one study on dermographism in children reported a female predominance.[7] No racial variance in prevalence is known.

Prognosis

Simple dermographism is the most common variant, and patients with this form are asymptomatic. However, other forms are associated with pruritus, and this can significantly affect quality of life. Most people with dermographism are otherwise healthy. An association with thyroid disease has been described in some patients but remains controversial.

The natural history of symptomatic dermographism is unpredictable. It may last for months or years, or be present intermittently. In many patients, the condition gradually improves and clears after several years. Of the chronic urticarias, symptomatic dermographism appears to have the best prognosis in terms of clearance after 5 years (36%) and 10 years (51%).[8]

Patient Education

Reassure patients about the benign nature of the disorder, and inform them of the possible prolonged course. Explain the adverse effects of antihistamine therapy. In particular, warn patients about drowsiness, which is especially problematic when they are driving or handling machinery.

For patient education resources, see the Allergy Center and Skin, Hair, and Nails Center, as well as Hives and Angioedema.

History

Whealing usually develops within 5-10 minutes of stroking the skin and persists for 15-30 minutes. A short refractory period after clearance of the wheal has been reported. Giant wheals can develop if deep extension of the swelling occurs. Intermediate and delayed forms of dermographism are also described. These develop more slowly and can last several hours to days.

In patients with symptomatic dermographism, the skin eruption is associated with itching, which is often most severe at night. Symptoms can be aggravated by heat (eg, from a hot bath), minor pressure (eg, from scratching, from friction with clothes, or from rubbing with towels), exercise, stress, and emotion.

Physical Examination

Itching and whealing can affect all body surfaces, but the scalp and genitalia are less frequently involved. However, dyspareunia and vulvodynia have been reported in patients with symptomatic dermographism.[9] Rarer forms of dermographism include the following:

Approach Considerations

The results from hematologic and biochemical screening tests are normal. In some patients, an increase in blood histamine levels is seen after experimental scratching.

The diagnosis of dermographism is usually made by observing the clinical response after using moderate pressure to stroke or gently scratch the skin. The site is important because areas protected from regular pressure and environmental influences (eg, the back) typically are more reactive than more exposed areas (eg, the buttocks and limbs).

A dermographometer (spring-loaded stylus) can be used to apply graded, reproducible pressure (eg, 3600 g/cm2) and record skin responses. The use of this instrument is mostly limited to research settings.

Biopsy specimens show dermal edema with a few perivascular mononuclear cells.

Approach Considerations

Dermographism can be distressing but is not life threatening. Patients with simple dermographism are asymptomatic and require no therapy. Patients with symptomatic dermographism should be treated until the problem is adequately controlled or resolved.

Recognition of the problem, avoidance of precipitating physical stimuli, reduction of stress and anxiety are important factors in medical care. In addition, scratching because of dry skin can be reduced through good skin care and the use of emollients.

Pharmacologic therapy

H1 antihistamines are the drugs of choice. In some patients, a combination of 2 or more antihistamines may be required. Sedating antihistamines such as hydroxyzine can be helpful. Regular treatment may have to be continued for several months. The addition of H2 -receptor antagonists appears to result in little symptomatic benefit, though some studies have shown a further small reduction in the whealing response.[12] Physical urticarias are usually unresponsive to systemic corticosteroids.

Omalizumab, a recombinant humanized monoclonal antibody against immunoglobulin E (IgE), has been successfully used in patients with physical urticaria, including symptomatic dermographism. It is administered subcutaneously at an initial dosage of 150 mg/mo, but the dosage can be increased to 300 mg/mo. Some patients have reported complete symptom resolution within days after the first injection.[13] Omalizumab was successfully used in a woman with 3 subtypes of chronic urticaria, including symptomatic dermographism.[14] Treatment was continued throughout the patient's pregnancy without any complications or obvious adverse effects, although the frequency of injections had to be increased to every 15 days.

Light therapy

Narrowband ultraviolet (UV)-B phototherapy and oral psoralen plus UV-A light therapy have both been used as treatments for symptomatic dermographism. Subjective relief of pruritus and whealing and objective reduction of wheals have been reported.[15] However, the improvement is short-lived, and most patients relapse within 2-3 months of completing phototherapy.

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications. Antihistamines are the mainstay of pharmacologic therapy for dermographism urticaria.

Cetirizine (Zyrtec, Zyrtec Chewable Tablets)

Clinical Context:  Cetirizine forms a complex with histamine for H1-receptor sites in blood vessels, the gastrointestinal (GI) tract, and the respiratory tract.

Loratadine (Claritin)

Clinical Context:  Loratadine selectively inhibits peripheral histamine H1 receptors.

Desloratadine (Clarinex)

Clinical Context:  Desloratadine is a long-acting tricyclic histamine antagonist that is selective for H1 receptors. It relieves nasal congestion and alleviates the systemic effects of seasonal allergy. It is a major metabolite of loratadine, which, after ingestion, is metabolized extensively to the active metabolite 3-hydroxydesloratadine.

Levocetirizine (Xyzal)

Clinical Context:  Levocetirizine is an H1-receptor antagonist, an active enantiomer of cetirizine. It is a second-generation prescription antihistamine.

Fexofenadine (Allegra)

Clinical Context:  Fexofenadine competes with histamine for H1 receptors in the GI tract, blood vessels, and the respiratory tract, reducing hypersensitivity reactions. It does not sedate.

Class Summary

Second-generation antihistamines, also known as less-sedating or low-sedation antihistamines, produce less sedation than traditional H1 blockers because they are less lipid-soluble and only cross the blood-brain barrier in small amounts. They also have longer half-lives, allowing less-frequent dosing.

Antihistamines act through competitive inhibition of histamine at the H1 receptor, the H2 receptor, or both. This action mediates wheal and flare reactions, bronchial constriction, mucus secretion, smooth muscle contraction, edema, hypotension, central nervous system (CNS) depression, and cardiac arrhythmias.

Hydroxyzine (Vistaril)

Clinical Context:  Hydroxyzine is a sedative antihistamine that also has an anxiolytic effect. It antagonizes H1 receptors in the periphery. It may suppress histamine activity in the subcortical region of the CNS.

Diphenhydramine (Benadryl, Diphenhist, Allerdryl)

Clinical Context:  Diphenhydramine is given for symptomatic relief of symptoms caused by release of histamine in allergic reactions.

Class Summary

First-generation antihistamines compete with histamine at the tissue-receptor level, preventing it from carrying out its mediator functions in urticaria.

Antihistamines act through competitive inhibition of histamine at the H1 receptor, the H2 receptor, or both. This action mediates wheal and flare reactions, bronchial constriction, mucus secretion, smooth muscle contraction, edema, hypotension, central nervous system (CNS) depression, and cardiac arrhythmias.

Omalizumab (Xolair)

Clinical Context:  Omalizumab, a recombinant humanized monoclonal antibody against immunoglobulin E (IgE), has been successfully used in patients with physical urticaria, including symptomatic dermographism. It is administered subcutaneously at an initial dosage of 150 mg/mo, but the dosage can be increased to 300 mg/mo. Some patients have reported complete symptom resolution within days after the first injection.

Class Summary

Omalizumab (Xolair) is a recombinant DNA-derived humanized immunoglobulin G monoclonal antibody that binds selectively to human immunoglobulin E on the surface of mast cells and basophils. The drug reduces mediator release, which promotes an allergic response.

Author

Simone Laube, MD, MRCP, Consulting Staff, Department of Dermatology, Borders General Hospital, Melrose and Western General Hospital, Edinburgh

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Ackerman Academy of Dermatopathology, New York

Disclosure: Nothing to disclose.

Additional Contributors

Paul Krusinski, MD Director of Dermatology, Fletcher Allen Health Care; Professor, Department of Internal Medicine, University of Vermont College of Medicine

Paul Krusinski, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Shyam Verma, MBBS, DVD, FAAD Adjunct Clinical Assistant Professor, Department of Dermatology, University of Virginia, State University of New York at Stonybrook, Penn State University

Shyam Verma, MBBS, DVD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MDis a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Elsbeth Young, MD, FRCP Consulting Staff, Department of Dermatology, Amersham Hospital and Wycombe General Hospital, UK

Disclosure: Nothing to disclose.

References

  1. Grimm V, Mempel M, Ring J, Abeck D. Congenital symptomatic dermographism as the first symptom of mastocytosis. Br J Dermatol. Nov 2000;143(5):1109. [View Abstract]
  2. Taskapan O, Harmanyeri Y. Evaluation of patients with symptomatic dermographism. J Eur Acad Dermatol Venereol. Jan 2006;20(1):58-62. [View Abstract]
  3. Wallengren J, Isaksson A. Urticarial Dermographism: Clinical features and response to psychosocial stress. Acta Derm Venereol. 2007;87:493-8. [View Abstract]
  4. Wu JJ, Huang DB, Murase JE, Weinstein GD. Dermographism secondary to trauma from a coral reef. J Eur Acad Dermatol Venereol. Nov 2006;20:1337-8. [View Abstract]
  5. Rahim KF, Dawe RS. Dermatomyositis presenting with symptomatic dermographism and raised troponin T: a case report. Journal of Medical Case Reports. July 2009;3:7319. [View Abstract]
  6. Dinc A, Karaayvaz M, Caliskaner AZ, Pay S, Erdem H, Turan M. Dermographism and atopy in patients with Behcet's disease. J Investig Allergol Clin Immunol. Nov-Dec 2000;10(6):368-71. [View Abstract]
  7. Martorell A, Sanz J, Ortiz M, Julve N, Cerda JC, Ferriols E. Prevalence of dermographism in children. J Investig Allergol Clin Immunol. May-Jun 2000;10(3):166-9. [View Abstract]
  8. van der Valk PG, Moret G, Kiemeney LA. The natural history of chronic urticaria and angioedema in patients visiting a tertiary referral centre. Br J Dermatol. Jan 2002;146(1):110-3. [View Abstract]
  9. Lambiris A, Greaves MW. Dyspareunia and vulvodynia: unrecognised manifestations of symptomatic dermographism. Lancet. Jan 4 1997;349(9044):28. [View Abstract]
  10. Matthews CN, Warin RP. Cold urticaria and cold precipitated dermographism. Br J Dermatol. Jan 1970;82:91. [View Abstract]
  11. Jedele KB, Michels VV. Familial dermographism. Am J Med Genet. May 1 1991;39(2):201-3. [View Abstract]
  12. Sharpe GR, Shuster S. In dermographic urticaria H2 receptor antagonists have a small but therapeutically irrelevant additional effect compared with H1 antagonists alone. Br J Dermatol. Nov 1993;129(5):575-9. [View Abstract]
  13. Metz M, Altrichter S, Ardelean E, at al. Anti-Immunoglobulin E Treatment of Patients with Recalcitrant Physical Urticaria. Int Arch Allergy Immunol. 2011;154:177-180. [View Abstract]
  14. Vieira Dos Santos R, Locks Bidese B, Rabello de Souza J, Maurer M. Effects of Omalizumab in a patient with three types of chronic urticaria. Br J Dermatol. Sep 14 2013;[View Abstract]
  15. Borzova E, Rutherford A, Konstantinou GN, Leslie KS, Grattan CE. Narrowband ultraviolet B phototherapy is beneficial in antihistamine-resistant symptomatic dermographism: A pilot study. J Am Acad Dermatol. Sept 2008;59:752-7. [View Abstract]
  16. Grattan CEH, Kobza Black A. Urticaria and mastocytosis. In: Burns DA, Breathnach SM, Cox N, Griffiths C, eds. Rook's Textbook of Dermatology. Vol 3. 7th ed. London, England: Blackwell Science; 2004:47.1-47.37.

dermographism or skin writing on forearm

Appearance of the word "Dermatographia" on a human arm 15 minutes after the letters have been traced with a blunt object on the skin. A type of urticaria or "hives", also known as dermatographic urticaria, dermatographism, or "skin writing".