Symptomatic Dermatographism Cholinergic and Aquagenic Urticaria

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					                                                               Symptomatic Dermatographism, Cholinergic, and Aquagenic Urticaria in a 10-year-old boy.
                                                                                                                                      Mitzi Go, MD and Thomas Leath, MD
                                                                    Department of Pediatrics, Section of Pediatric Pulmonology; The Children’s Hospital at Scott & White and Texas A&M Health Science Center College of Medicine, Temple, TX

                    ABSTRACT                                                                     CASe                                                                       diSCuSSion                                                                          diSCuSSion (ConT)                                                                                                                                                                ConCluSion
                                                                                                                                                                                                                                                                                                                                     With all 3 forms of physical urticaria, the pathogenesis is still
Urticaria or hives is a common disorder affecting a             We are presenting a case of a 10-year old Caucasian male with a 3-4             Urticaria, or hives, is a common disorder that affects up to 25% of the           Cholinergic urticaria is the term given to hives that are precipitated by an increase in core      poorly understood, although several mechanisms have been                Specific physical stimuli are the cause of approximately 20% of all urticarias.
quarter of the population at one time or another. We            year history of hives. The hives were described as first appearing to           population.1 The lesions are intensely pruritic, circumscribed, raised,           body temperature. 3 It was first described by Duke in 1924, in a patient who developed hives       proposed.3,4,8,10,12,15,16.                                             Although most frequently occurring in individuals between their 2nd and
report the case of a 10 year old male with a 3-4 year           be small, red, intensely pruritic, non-painful “bumps” appearing on             erythematous plaques, often with central pallor, that may enlarge and             after exercising, experiencing strong emotions, and applying heat to the skin.6 However,                                                                                   4th decade of life, there have been a reported cases as young as 3 years
history of having small, erythematous, intensely                his body and extremities while he was taking warm showers, enlarging            coalesce with other lesions,                                                      it was Grant, et al, in 1936, who proposed that the hives were caused by the cholinergic           Involvement of histamine and an IgE-mediated reaction have              of age, with onset of symptoms noted since birth. Dermatographism and
                                                                                                                                                                             Figure 1
pruritic, non-painful “bumps” appearing on his skin             and coalescing into hives over time. He has also noted this similar rash                                                                                          nervous system.7 It is believed to account for about 5% of all cases of chronic urticaria3,        been suggested for dermatographism but no allergen has been             cholinergic urticaria are fairly common, but aquagenic urticaria remains
after taking warm showers, after sweating (both with            appearing when he exercises outside and becomes sweaty, or even when                                                                                              and has it’s onset during the 2nd or 3rd decade of life. Classically, it presents as numerous      identified.3                                                            rare, with only a few case reports in literature since described by Shelley
and without exertion), after swimming in cool water, or         he sweats without significant activity (like getting into a hot tub). He also                                                                                     punctuate wheals (1-3mm) surrounded by large flares, typically beginning on the trunk and                                                                                  and Rawnsley in 1964. With all these forms of physical urticaria, the
even with just getting wet from a garden hose. These            frequently develops hives when swimming in a pool of cool water, or                                                                                               neck, spreading distally to involve the face and extremities, although lesions may begin           With cholinergic urticaria, it is generally believed that there is an   underlying pathogenesis still remains unclear, subsequently making it
bumps would coalesce into hives over time, but no               when splashed with water from a garden hose. The longest hive-free time                                                                                           anywhere in the body. (Figure 3 and 4)                                                             abnormal cutaneous response from the action of acetylcholine            difficult to establish guidelines for diagnosis and management. From all
other systemic symptoms have been noted. Review of              since the onset of these symptoms has been 3 days. Several medications                                                                                                                                                                                               on mast cells.3 Other investigators have demonstrated a more            reports, a very thorough history and physical exam is the most important
                                                                                                                                                                                                                                   Figure 3                                      Figure 4
other commonly known allergens that the patient was             have been tried previously, including diphenhydramine, cetirizine, and                                                                                                                                                                                               typical antigen-antibody reaction, with some patients having a          tool to establish a diagnosis. Additional laboratory testing usually are
exposed to did not reveal a similar reaction. Therapy           montelukast sodium, affording only slight relief of symptoms and no                                                                                                                                                                                                  type I allergy to their own sweat.3                                     of no additional benefit, frequently only ordered to reassure the patient
with diphenhydramine, cetirizine and montelukast                significant long-term control.                                                                                                                                                                                                                                                                                                               and doctor that no cause had been missed. For our patient, the presence
sodium in the past have failed to establish long-term                                                                                           and typically disappear over a few hours without leaving residual marks on                                                                                                           With aquagenic urticaria, some authors postulated that water            of significant dermatographism on exam during the initial clinical visit
control. On physical examination, the patient developed         The patient denies wheezing, angioedema, nausea, vomiting, diarrhea             the skin (unless there is damage from scratching). It can further be classified                                                                                                      interacted with sebum to form a substance capable of acting as a        made it difficult to perform other provocative challenges to confirm
significant local and distant hives when stroked with a         or lightheadedness with the rash. No residual bruising has been noted,          as acute or chronic, depending on the duration of the outbreaks. Chronic                                                                                                             direct mast cell degranulation, resulting in histamine release.4,10     cholinergic and aquagenic urticaria. However, the history provided by the
tongue blade, preventing further provocative testing.           ruling out vasculitis from the differential. The patient has been eating all    urticaria is defined by the presence of hives on most days of the week, for a                                                                                                        Sibbald et al concluded that enhancing the ability of water to          family strongly supported the diagnoses. Although avoidance of known
With the history and physical examination strongly              the common food allergens (i.e. eggs, nuts, seafood, dairy products, etc.)      duration of longer than six weeks.2 This form accounts for about 30 percent                                                                                                          penetrate the stratum corneum layer of the skin (with removal           triggers remain the most sensible and practical step toward symptom
suspicious for symptomatic dermatographism,                     with no noted temporal association with the hives. There was never any          of all cases of urticaria3. It can occur through a variety of mechanisms,                                                                                                            of this layer in any way or pretreatment with organic solvents)         control, this would be difficult with aquagenic urticaria since water is one
cholinergic, and aquagenic urticaria, the patient was           history of arthralgias, joint stiffness or joint swelling. He did, however,     including allergic, cytotoxic, autoimmune, complement-mediated and                                                                                                                   increases the wheal-provoking effects of water in these patients.15     of the most ubiquitous substances on earth, and - much to our patient’s
started on fexofenadine and ImmunoCAP                  RAST     develop hives with intake of amoxicillin at age 2 years. There is no family     idiopathic mechanisms, which are waiting to be defined.                                                                                                                              Others believe that water is just an “innocent bystander,” acting as    disappointment - is a necessity for good hygiene. Even avoidance of
studies were sent for allergy testing. Follow-up after 2        history of known food allergy, atopy or chronic urticaria. Both parents                                                                                                                                                                                              a carrier of an unknown antigen, while some think that activation       non-specific triggers - like the use of NSAIDs, ingestion of alcohol,
months showed significant improvement of symptoms               smoke (outside), and they have an outside dog, carpet, curtains and             Physical urticarias make up a subset of chronic urticaria, accounting                                                                                                                of the cholinergic pathways play a role.12,15,16                        peanuts, and wearing tight clothing is a reasonable precaution. In our
with milder, and less frequent flare-ups responsive to          stuffed animals. They deny having mold or water damage in the home.             for about 20-30% of cases.3 Patients have urticaria that is induced by a                                                                                                                                                                                     patient’s case, a positive response to fexofenadine and diphenhydramine
diphenhydramine. Repeat stroking with the tongue                                                                                                wide variety of environmental stimuli, such as exercise, temperature              This is usually accompanied by a tingling, itching, or burning sensation, prior to appearance      Treatment involves identification and avoidance of known                emphasize the role of antihistamines as a cornerstone for therapy and
blade only produced a local wheal. Dermatographism,             On exam, the patient’s skin did not have any rash or lesions, but developed     changes, cold, heat, pressure, sunlight, vibration, and water. There are          of the lesions. Reported triggers include exercise, hot baths or showers, strong emotional         triggers (for symptomatic and cholinergic urticaria), and the use       symptom control.
cholinergic, and aquagenic urticarias are types of              both local and distant hives within 15 minutes after stroking both              different types of physicial urticarias, including those that our patient have:   feelings, and ingestion of spicy or hot foods, all of which lead to increased sweating,            of antihistamines. H1 antihistamines (particularly first generation
physical urticarias that fall under the classification          forearms with a tongue blade. Skin testing was not performed because            dermatographism, cholinergic urticaria, and aquagenic urticaria.                  which is postulated to have bearing on the pathogenesis of this condition. Provocation             drugs) have been shown to be effective in dermatographism and
of chronic urticaria. Although dermatographism and              of the significant dermographia demonstrated. From the history and                                                                                                testing aimed to raise the patient’s core body temperature have been used to confirm               cholinergic urticaria. Several investigators have reported that
cholinergic urticaria are fairly common, aquagenic              exam, it was suspected that the patient has symptomatic dermographia,           Dermatographism is the most common of the physical urticarias and is often        the diagnosis of cholinergic urticaria. The more common methods are: 1) the exercise               addition of an H2 antihistamine is beneficial for better long-term                                          RefeRenCeS
urticaria is still relatively rare, with only a few reported    cholinergic urticaria, and a more rare diagnosis of aquagenic urticaria.        an incidental finding in the evaluation of other skin conditions. Simple (or      challenge (preferably performed in a controlled setting); and 2) the intradermal injection of      control. With aquagenic urticaria, pretreatment with antihistamines     1.  Zuberbier, T. Urticaria. Allergy 2003; 58:1224.
                                                                                                                                                                                                                                                                                                                                                                                                             2.  Kaplan, A. Urticaria and angioedema. In: Middleton’s Allergy: Prinicples and Practice, 6th
cases in medical literature. Pathogenesis for all 3                                                                                             asymptomatic) dermatographism is the most common variant, occurring in            methacholine to produce local hives. However, these tests are not specific enough to rule          has been reported to completely control symptoms in some                    Ed.Adkinson NF, Yunginger JW, Busse WW, et al (Eds), Mosby, St Louis, MO 2003. p. 1537.
types of physical urticarias are still largely uncertain,                                                                                       approximately 2-5% of the general population, but the symptomatic forms           out other causes of urticaria due to frequent overlap of triggers.3                                cases, while others report significantly attenuated symptoms with       3. Dice JP. Physical urticaria. Immunol Allergy Clin N Am. 24(2004) 225-246.
                                                                                                                                                                                                                                                                                                                                                                                                             4. Shelley WB, Shelley ED. Follicular dermographism. Cutis 1983; 32(3):244-5, 254, 260.
but diagnosis is usually established with a very                                                                                                are much less common and no prevalence data have been established.3                                                                                                                  medication. Barrier methods have been shown to be effective in          5. Warin RP. Factitious urticaria: red dermographism Br J Dermatol 1981; 104(3):285-8.
                                                                                                                                                                                                                                                                                                                                                                                                             6. Duke WW. Urticaria caused specifically by the action of physical agents. JAMA 1924;83:3-9.
thorough history and physical examination, confirmed                                                                                            Our patient has the symptomatic form, which is characterized by lesions           Aquagenic urticaria is a rare condition, with fewer than 50 cases reported in medical              one study.15 Some authors used UVB light treatments, and PUVA           7. Grant RT, Pearson RSB, Comeau WJ. Observations on urticaria provoked by emotion, by
by provocative challenges for a specific stimuli.                                                                                               appearing in less than 5 minutes and lasting at least 30 minutes (as opposed      literature.3 It was first described by Shelley and Rawnsley in 1964,8 and arises from direct       therapy in isolated cases with improvement.3,13 However, because            exercise, and by warming the body. Clin Sci 1936;2:253-72.
                                                                                                                                                                                                                                                                                                                                                                                                             8. Shelley WB, Rawnsley HM. Aquagenic urticaria contact sensitivity reaction to water. JAMA 1964;
Antihistamines remain a cornerstone in therapy, but                                                                                             to “asymptomatic” lesions, appearing within 6-7 minutes, and fading 15-           skin contact with water. The lesions are characteristically indistinguishable from the wheals      of the rarity of this diagnosis, no guidelines have been established        1898:895-8.

avoidance of both specific and non-specific triggers
                                                                                         MAnAgeMenT                                                                                                                               of cholinergic urticaria (small, punctuate, and perifollicular), but appear rapidly after direct                                                                           9. Luong K, Nguyen LTH. Aquagenic urticaria: report of a case and review of the literature. Ann Allegy
                                                                                                                                                30 minutes later). In addition to classic wheals, variants of symptomatic                                                                                                            for pharmacotherapy at this time.                                           Asthma Immunol 1998; 80:483-5.
remain essential in long-term symptom control.                                                                                                  dermatographism have been described in which the reactions are                    contact with any source of water. Wheal formation is not influenced by temperature or pH                                                                                   10. Chalamidas SL, Charles CR. Aquagenic urticaria. Arch Dermatol 1971; 104:541-6.
                                                                ImmunoCAP RAST studies were sent for allergy testing during this                                                                                                  of the water, appears within 20-30 minutes, and generally fade 30-60 minutes after water
                                                                                                                                                                                                                                                                                                                                                                                                             11. Davis RS, Remigio LK, Schoket AL, Bock SA. Evaluation of a patient with both aquagenic and
                                                                                                                                                follicular or inflamed and swollen.4,5 Purposeful stroking of the skin is                                                                                                                                                                                        cholinergic urticaria. J Allergy Clin Immunol 1981;68(6):479-83.
                                                                initial visit, and the patient was started on fexofenadine 60 mg BID, and                                                                                         source is removed from the skin. Systemic symptoms are rare but have been reported.9 A                                                                                     12. Czarnetzki BM, Breetholt KH, Traupe H. Evidence that water acts as a carrier for an epidermal antigen
                                                                                                                                                the most common way to elicit symptoms and this is how diagnosis is                                                                                                                                                                                              in auagenic urticaria. J Am Acad Dermatol 1986; 15(4):623-7.
                                                                ranitidine 150 mg QHS. Results of the allergy tests showed very low level                                                                                         refractory period lasting several hours have been demonstrated after an attack, and some                                                                                   13. Parker RK, Crowe MJ, Gui JD. Aquagenic urticaria. Cutis 1992;50:283-4.
                                                                positive reactions to mix of trees, grasses, house dust, weeds and foods        established. (Figure 2)                                                                                                                                                                                                                                      14. Mathelier-fuusade P, Aissaoui M, Chabane MH, Mounedji N, Leynadier F. Association of cold urticaria
                                                                                                                                                                          Figure 2                                                report that repeated, short, purposeful exposures to water can lead to exhaustion of the                                                                                       and aquagenic urticaria. Allergy 1997;52:678-9.
                                                                including wheat, soy and peanuts that are believed to be not clinically                                                                                           wheal response.10-11 Women seem to have a slightly higher incidence in men, and in most                                                                                    15. Sibbald RG, Kobza Black A, Eady RAJ, James M, Greaves MW. Aquagenic urticaria: evidence of
                                                                                                                                                                                                                                                                                                                                                                                                                 cholinergic and histaminergic basis. Br J Dermatol 1981;105:297-302.
                                                                significant. On follow-up visit 2 months later, the patient reports that the                                                                                      cases, age of onset is at or slightly after puberty.3 Several case reports exist of patients                                                                               16. Tkach JR. Aquagenic urticaria. Cutis 1981;28:454, 463.
                                                                hives and pruritus seem to have improved, but he was still experiencing                                                                                           with aquagenic urticaria and coexisting dermatographism, or cholinergic urticaria, but
                                                                                                                                                                                                                                                                                                                                                                                                             17. Frances AM, Fiorenza G, Frances RJ. Aquagenic urticaria: report of a case. Allergy Asthma Proc 2004.
                                                                breakthrough hives for which he takes diphenhydramine. During this                                                                                                only one study mentioned a patient with all 3.3,9,11-13 There has also been a case report                                                                                  18. Kozel MMA, Moein MCA, Mekkes JR, Meinardi MMHM, Bossuyt PMM, Bos JD. Evaluation of a clinical
                                                                                                                                                                                                                                                                                                                                                                                                                 guideline for the diagnoses of physical and chronic urticaria and angioedema. Acta Derm Venerol
                                                                follow-up visit, tongue blade stroking yielded only very localized erythema                                                                                       of it associated with cold urticaria, another form of physical urticaria.14 Diagnostically,                                                                                    2002; 82; 270-4.
                                                                but no wheal and flare response. fexofenadine was then increased to                                                                                               it is important to rule out other physical urticarias given the overlap in potential inciting
                                                                                                                                                                                                                                                                                                                                                                                                             19. Kozel MMA, Mekkes JR, Bossuyt PMM, Bos JD. Natural course of physical and chronic urticaria and
                                                                                                                                                                                                                                                                                                                                                                                                                 angioedema in 220 patients.
                                                                180 mg once a day, and diphenhydramine will continue to be used for                                                                                               triggers. The standard test is to apply a water compress of 35°C to the upper body for 30                                                                                  20. Kozel MMA, Bossuyt PMM, Mekkes JR, Bos JD. Laboratory tests and identified diagnoses in patients
                                                                                                                                                                                                                                                                                                                                                                                                                 with physical and chronic urticaria and angioedema: A systematic review.
                                                                breakthrough symptoms since this is not sedating for him. Follow-up                                                                                               minutes.3 The set temperature avoids confusion with cold-induced or local-heat urticaria.                                                                                  21. Habif, TP. 2004. Clinical dermatology:A color guide to diagnosis and therapy. pp. 142-147. Mosby,
                                                                                                                                                                                                                                                                                                                                                                                                                 Inc,Philadelphia, USA.
                                                                was scheduled after 3 months.                                                                                                                                     Performing direct bath and shower challenges have been attempted as well.                                                                                                  22.
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