Localized heat urticaria Anwell Chang, MD,a and John A. Zic, MDb Cleveland, Ohio, and Nashville, Tennessee Localized heat urticaria is one of the rarest of the physical urticarias, characterized by well-defined urticarial lesions sharply confined to sites of heat exposure. We describe a case of localized heat urticaria in a 40-yearold woman. Because of the rarity of this disorder, much remains to be elucidated. The clinical features, pathogenesis and therapy are reviewed. (J Am Acad Dermatol 1999;41:354-6.)
F
ewer than 50 cases of localized heat urticaria have been reported in the world literature since the first description in 1924.1 The pathogenesis of this disorder remains obscure, and previous studies have yielded conflicting results. Treatment options are limited.
CASE REPORT A 40-year-old white woman had a 4- to 5-year history of hives as a result of heat exposure. The patient first noticed sensitivity to heat after a warm bath, localized to the immersed areas. She observed similar reactions on her face shortly after exposure to heat from an oven, and on sun-exposed areas after sun exposure. The patient denied systemic symptoms. Physical exertion or sweating did not induce urticaria. A warm container of water (~41°C) was placed against the volar aspect of her forearms for 25 seconds. Within 15 minutes after removal, well-defined plaques of urticaria developed (Figure). These lesions were erythematous and edematous, with prominent hair follicle openings. Dermatographism was absent.
DISCUSSION Only 47 patients with localized heat urticaria have been described in the world literature. Nine of these patients are not included in this discussion because their reports could not be obtained2-8 or incomplete data was provided.9 Localized heat urticaria predominantly affects women (32 of 38 cases). There has been only 1 report of a familial form affecting 3 generations of a family (9 cases), which appears to have This supplement is made possible through an educational grant from Ortho Dermatological to the American Academy of Dermatology. From the Department of Dermatology,a Cleveland Clinic Foundation, and the Department of Dermatology,b Vanderbilt University School of Medicine. Reprint requests: John A. Zic, MD, 1301 22nd Ave S, 3900 TVC, Nashville,TN 37232-5227. E-Mail:
[email protected] Copyright © 1999 by the American Academy of Dermatology, Inc. 0190-9622/99/$8.00 + 0 16/4/98283
354
been transmitted in an autosomal dominant manner.10 The remainder of the cases were acquired cases. The age of onset ranges between 17 to 70 years of age in acquired cases, and is in late childhood in familial cases. The lesions of localized heat urticaria are welldefined and limited to the area of contact with heat. Lesions may be indurated11,12 and are typically pruritic. In addition, burning sensations have been described.10,11,13,14 Whealing typically develops within 2 to 15 minutes of heat exposure, and resolves after 1 to 3 hours in acquired cases of localized heat urticaria. Familial cases have delayed urticarial responses, with whealing occurring 1 to 2 hours after heat contact and persisting for as long as 10 hours.10 Threshold temperatures for whealing range from 39°C to 56°C (with the exception of 1 patient who had a threshold temperature of 80°C), and may vary with anatomic location.10,15 The initial experience of whealing may occur suddenly after an episode of extensive sun exposure16,17 or bathing in warm water18-20 as in our patient. Common triggers for localized heat urticaria include warm baths, radiant heat, sunlight, oven steam, hot utensils, and hairdryers.10-12,14,16-26 In addition, hot foods and drinks have been reported to cause swelling, pruritus, or irritation of the palate and throat12,14,19,20 or lips18 and even difficulty breathing.22 About 50% of patients experience systemic symptoms such as syncope, fatigue, headaches, wheezing, dyspnea, nausea, vomiting, abdominal cramps, diarrhea, flushing, or fever.10,13-15,17-20,27,28 These symptoms are particularly apt to occur when large areas of skin are involved.24 An increased incidence of atopy has also been reported in patients with localized heat urticaria.14,19 Ragweed allergy,18 allergic rhinitis,13,15,17,18,23 and eczema14,15 have been reported to occur in association with localized heat urticaria. Localized heat urticaria is also associated with an increased incidence of other physical urticarias.17,28
J AM A C A D D ERMATOL V OLUME 41, NUMBER 2, PART 2
Chang and Zic 355
Figure. Localized plaque of urticaria induced by application of warm specimen container filled with water (~41°C).
Cold urticaria9,19,22,28,29 or solar urticaria30 may coexist with localized heat urticaria. In 1 patient, it was postulated that strenuous exercise led to an elevation of skin temperature above the threshold temperature for whealing.15 Dermatographism was present in some cases of localized heat urticaria,10,18,23,28 but could not be demonstrated in our patient. The diagnosis of localized heat urticaria is suggested by history and confirmed by local heat challenge. Localized heat urticaria must be distinguished from other physical urticarias, including cholinergic, solar, and aquagenic urticarias. Unlike cholinergic urticaria, it is not induced by sweating, exertion, or emotion, and cannot be experimentally reproduced by injecting cholinomimetics,25 or can the whealing be inhibited by atropine.15 Localized heat urticaria is entirely temperature-dependent, and independent of light wavelengths, in contrast to solar urticaria.22,30 The pathogenesis of localized heat urticaria remains to be determined. Few data are available, much of which are conflicting. Microscopic examination of skin biopsy specimens of patients with localized heat urticaria has shown variable amounts of perivascular infiltration with lymphocytes,10,26 but has not shown increased numbers of mast cells.28 Mast cell degranulation has not been observed at the time of whealing,11,12,14,24 although in one study, degranulation was observed 24 hours after heat challenge.12 Histamine has been implicated in many physical urticarias including dermatographism, light, cold, vibratory, and cholinergic urticaria,13 and likely plays a role in localized heat urticaria. Some patients developed exaggerated whealing responses to intradermal injections of histamine,16 but this finding was not confirmed in other patients.18,21 Elevated histamine levels have been documented in skin per-
fusates,21 as well as plasma after localized heatinduced urticaria.12-15,17 Other studies, however, have contradicted these findings.11,26 Increased concentrations of histamine were also found in suction blisters after heat challenge, suggesting local release of histamine in the skin.28 Increased levels of neutrophil chemotactic and prostaglandin D2 factor were found in plasma after local heat challenge in some studies14,17 but other studies did not find similar changes.26 Activation of the alternate complement pathway has been suggested to contribute to localized heat urticaria. Serum levels of total hemolytic complement, C3 and factor B were reported to decrease after heat challenge in some studies11,24; however, this decrease was not observed in other studies.12-15,17,27 Prior infiltration of skin with local anesthetics abolished the urticarial response in some studies,18 but not in others.15,16,22 Topical capsaicin impairs chemosensitive nerve endings. In one study, it prevented whealing in patients with acquired cold and heat urticaria, suggesting that in these patients there may be an abnormal reaction of chemosensitive fibers to sensory stimuli,29 but another study found no effect on whealing.15 Treatment options for localized heat urticaria are limited and have been variably effective. Combinations of systemic H1 and H2 blockers have successfully prevented urtication in some,12 but not all patients.11,17,19,23,25,27 Systemic doxepin, which has a high affinity for both H1 and H2 receptors, was demonstrated to be effective in some patients.28 Desensitization has also been shown to be effective in treating localized heat urticaria15,17,19,20,26; however, when used as monotherapy, it may not be completely effective19,26 and in some patients, tolerance may not be achieved.16,24 Indomethacin has been
356 Chang and Zic
reported to ameliorate symptoms.14,26 Chloroquine prevented symptoms in 1 patient,30 but had no effect on another.27 Our patient was treated with loratidine, 10 mg per day, which resulted in tolerable symptoms. Long-term follow-up has been limited, thus the natural history of this disorder is not well-defined. Some patients have manifested symptoms for more than 10 years.14 A gradual decline in severity of symptoms was reported in 1 patient at a 3-year follow-up.13 A 7-year follow-up of 8 patients found that 2 patients went into remission.15 Patients treated with desensitization have been reported to have remained symptom-free at 615 or 12 months followup.19 Because of the rarity of this condition, more long-term follow-up is needed. And given the conflicting data in the literature, future studies need to focus on the pathogenesis of this disorder. REFERENCES 1. Duke WW. Urticaria caused specifically by the action of physical agents. JAMA 1924;83:3-9. 2. Lehner E, Rajka E. Warmeurticaria. Arch Dermatol Syph 1929; 158:402. 3. Hopkins JG, Kesten BM, Hazel OG. Urticaria provoked by heat or psychic stimuli. Arch Dermatol Syph 1938;38:679-91. 4. Melczer N, Wlassics T. Uber die Warmeuberempfindlichkeit. Arch Dermatol Syph Berlin 1938;176:157-66. 5. Jelic S, Vozovic M, Bojic P. Localized heat urticaria. Srpski Arhiv Za Celokupno Lekarstvo 1978;106:203-11. 6. Bedello PG, Goitre M, Cane D, Alovisi V. Apropos of a case of heat contact urticaria. G Ital Dermatol Venereol 1984;119:433-4. 7. Gonzalez JR, Palacio R. Heat urticaria clinically simulating solar urticaria. Bol Asoc Med P R 1984;76:188-91. 8. Eichelberg D. Desensitization (“hardening”) in heat urticaria. Z Hautkrankheiten 1988;63:385-6. 9. Neittaanmaki H. Cold urticaria. Clinical findings in 220 patients. J Am Acad Dermatol 1985;13:636-44. 10. Michaelsson G, Ros AM. Familial localized heat urticaria of delayed type. Acta Derm Venereol 1971;51:279-83. 11. Daman L, Lieberman P, Ganier M, Hashimoto K. Localized heat urticaria. J Allergy Clin Immunol 1978;61:273-8. 12. Irwin RB, Lieberman P, Friedman MM, Kaliner M, Kaplan R, Bale G, et al. Mediator release in local heat urticaria: protection with combined H1 and H2 antagonists. J Allergy Clin Immunol 1985; 76:35-9.
J AM A C A D D ERMATOL A UGUST 1999
13. Grant JA, Findlay SR, Thueson DO, Fine DP, Krueger GG. Local heat urticaria/angioedema: evidence for histamine release without complement activation. J Allergy Clin Immunol 1981; 67:75-7. 14. Koro O, Dover JS, Francis DM, Kobza Black A, Kelly RW, Barr RM, et al. Release of prostaglandin D2 and histamine in a case of localized heat urticaria, and effect of treatments. Br J Dermatol 1986;115:721-8. 15. Tatnall FM, Gaylarde PM, Sarkany I. Localised heat urticaria and its management. Clin Exp Dermatol 1984;9:367-74. 16. Moragas JMd, Gimenez-Camarasa JM, Noguera J. Localized heat urticaria. Arch Dermatol 1973;108:684-6. 17. Atkins PC, Zweiman B. Mediator release in local heat urticaria. J Allergy Clin Immunol 1981;68:286-9. 18. Delorme P. Localized heat urticaria. J Allergy 1969;43:284-91. 19. Higgins EM, Friedmann PS. Clinical report and investigation of a patient with localized heat urticaria. Acta Derm Venereol 1991;71:434-6. 20. Leigh IM, Ramsay CA. Localized heat urticaria treated by inducing tolerance to heat. Br J Dermatol 1975;92:191-4. 21. Greaves MW, Sneddon IB, Smith AK, Stanworth DR. Heat urticaria. Br J Dermatol 1974;90:289-92. 22. Tennenbaum JI, Lowney E. Localized heat and cold urticaria. Rare phenomena occurring in the same individual. J Allergy Clin Immunol 1973;51:57-9. 23. Wise RD, Malkinson FD, Luskin A, Gewurz AT, Zeitz HJ. Localized heat urticaria. Arch Dermatol 1978;114:1079-80. 24. Johansson EA, Reunala T, Koskimies S, Lagerstedt A, Kauppinen K, Timonen K. Localized heat urticaria associated with a decrease in serum complement factor B (C3 proactivator). Br J Dermatol 1984;110:227-31. 25. Freeman PA, Watt GC. Localized heat urticaria (heat contact urticaria). Australas J Dermatol 1988;29:43-6. 26. Chung HS, Lee KH, Ro JY. Heat contact urticaria—a case report. Yonsei Med J 1996;37:230-5. 27. Starr JC, Brasher GW, Hammerschmidt DE. Localized heat urticaria. Ann Allergy 1980;44:164-5. 28. Neittaanmaki H, Fraki JE. Combination of localized heat urticaria and cold urticaria. Release of histamine in suction blisters and successful treatment of heat urticaria with doxepin. Clin Exp Dermatol 1988;13:87-91. 29. Toth-Kasa I, Jancso G, Obal F Jr, Husz S, Simon N. Involvement of sensory nerve endings in cold and heat urticaria. J Invest Dermatol 1983;80:34-6. 30. Willis I, Epstein JH. Solar- vs heat-induced urticaria. Arch Dermatol 1974;110:389-92.
