John Weisnagel, M.D.
An 18 year-old young lady consulted recently with the following history: Late last summer, she began having localized swelling with pruritus after contact with heat, e.g. touching a hot plate, or a cup of coffee, swollen areas on her face if being over a boiling pot of water, or opening a hot oven, reactions that would last 15-20 min. No other symptoms associated. Drinking a hot liquid would make her mouth and throat swell with difficulty swallowing, again for about the same duration. Leaning on the roof of a car that was out in the sun caused a large white swelling on her forearm. Being in the sun was unbeareable because of similar white swollen patches on the exposed areas. Once, on taking a shower, she noticed a fine rash on her legs that she hasn’t seen since. Reactine was prescribed once a day for a month which seemed to reduce the duration of the reactions slightly. No similar reactions otherwise, whether on exercise, or after sweating.
Past hx : nég. except as a baby when drops of milk would fall on her skin, urticarial lesions appeared which were diagnosed as an allergy to milk.
Family hx : mother had eczema, a sister has solar urticaria and a brother pollen allergy.
On a follow-up visit, she reports a similar reaction after having held a cup of hot water (taken from the tap) for about 2 minutes (see photos she had taken, where white swellings are seen on the inside of her fingers and on the palmar surface.) As suggested, cooling the swollen areas with cold water or ice did not help, it rather increased the pruritus prolonging the duration of the reaction another 30 min.
While having lunch at school, she touched a plate that was hot => same reaction. Last summer she had no problem drinking hot liquids and being in the sun. Her reactions began suddenly, for no apparent reason. Stress was not involved according to her and her mother, as she was preparing going to college.
Conclusion : Localized angioedema following contact with heat. It is not cholinergic urticaria which presents with typical urticarial papules, usually 2-3 mm in diameter, with red patches, the affected area being warm with not only pruritus but a burning sensation.
There is no know cause. Stress is mentioned as a possible factor.
Follow-up : She hasn’t had any other reactions after drinking lukeward liquids, and she avoids touching anything hot. She’s taking Atarax 10 mg. at bedtime regularly. More later.
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A web search revealed only one reference to this type of physical allergy:
Mediator release in local heat urticaria by Atkins PC and Zweiman B in J. Allergy Clin. Immunology 1981 Oct;68(4) :286-9. The authors describe the 16th reported case of local heat urticaria in a 59 yr-old woman with erythema and angioedema upon contact with hot water or outdoor heat exposure. Immersing her hand in 39-40 degrees C heated water resulted in an erythematous, angioedematous response sharply demarcated by the line of immersion and was associated with immediate increases in histamine concentration (18 ti 135 ng/ml) and high molecular weight neutrophil chemotactic activity (two to five times prechallenge levels) in venous blood draining the challenge site. Conclusion: the authors suggest that the local heat urticarial response in this woman ws a form of physical urticaria associated with release of mast cell-derived mediators, akin to cold and cholinergic urticaira.
In Urticaria and Angioedema edited by M. W. Greaves, and Allen P. Kaplan in the chapter Physical and Cholinergic Urticarias by Anne Kobza Black, in the section called ‘localized heat urticaria’ the author writes : Warmth applied to the skin induces whealing restricted to the warmed area. This is one of the rarest forms of physical urticaria, with fewer than 50 cases described. It usually affects female adults but it has been described in a child. The pathomechanism is variable, with reported histamine release in some and complement activation in some cases, but not in others.
I e-mailed Harris Steinman, M.D., webmaster of AllAllergy.net, what he thought about it. His answer :
« I checked your query with a colleague, George du Toit, M. D. whose speciality is urticaria. He responded with "this sounds like physical urticaria with triggers of one or more of temp, pressure and u/v. Can be resistant to H1RA's “
An e-mail sent to ‘Ask the expert’ at the AAAA&I. Reply from Philip Lieberman, M.D., Medical Editor of the Allergy and Asthma Disease Management Center (AADMC):
Thank you for your recent inquiry.
According to the history you present, your patient has a condition known as "localized heat urticaria." The cause of this condition is unknown. In addition, the cases have been too rare for there to be any standardized treatment based on double-blind, placebo-controlled studies. The etiology of the problem remains undetermined; however, the pathogenesis is reasonably well understood.
In patients with this condition, there is mast cell degranulation with release of histamine and prostaglandin D2, and there has been some evidence for activation of the complement system as well. Studies have been limited to case reports.
For your convenience, I have copied below a few references:
1. Journal of the European Academy of Dermatology and Venereology 2008 (March); 22(3):384-386.
2. British Journal of Dermatology 1998 (February); 138(2):326-328.
3. ACTA Derm Venereol 1991; 71(5):434-436.
4. J Allergy Clin Immunol 1978 (April); 61(4):273-278.
The last reference is one in which I was a coauthor.
As noted, there is no standardized therapy for this disorder. However, based on my personal experience, the symptoms are difficult to control. We have had the best luck with a combination of H1 and H2 antagonists. Quite often doses higher than those standardly employed are needed. This disorder is considered a form of physical urticaria. However, the lesions are not generalized, but rather localized to areas of heat exposure. The lesions are actually more angioedema than urticarial in that there is edema in the subcutaneous tissue and dermis that produces swelling. The characteristic wheal of urticarial lesions does not occur. The diagnosis can be confirmed by applying localized heat to the forearm. This can be done, for example, with a cup containing warm to hot water. However, caution is advised since if heat is applied too long, or to too high a degree, blistering can occur at the site.
In sum, I believe that your patient has localized heat urticaria. This is a misnomer, as mentioned, since the lesions are more angioedematous in nature. The cause is unknown, but the pathophysiology clearly involves mediator release from mast cells and perhaps complement. It is a difficult management problem. Of course, heat should be avoided, and antihistamines are sometimes of help. This includes both an H1 and an H2 antagonist, and often requires higher than "normal" doses.
The Internet contains a number of references to this disorder, and I have listed a few for you above.
Thank you again for your inquiry and I hope this information has been helpful to you.
Sincerely,
Phil Lieberman, M.D.
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The following work-up was done in April, 09 and results were all normal: CBC, urinalysis, rhumatoid factor, C reactive protein, creatinine, liver function tests: alkaline GGT, ALT,alkaline phosphatase, total proteins, protein electrophoresis, ANA, C3, C4, protoporphyrins, TSH, anti-thyroid antibodies, HBS Ag, VDRL.
Nov. 10th, 2009
Since august, her condition seems to have improved: her reactions have diminished and of shorter duration. No more white areas, just erythema, pruritus and slight sensitivity when cutaneaous contact with heat. She could stand being in the sun for short periods during the summer months. She was on Atarax 10 mg daily which she takes only on occasion. She drank a warm cup of coffee today and had pruritus on the tongue and a slightly swollen palate, nothing else, for about ten minutes. She feels uncomfortable in a hot closed room, but has no other symptoms.
Jan. 30th, 2010
Her reactions remain the same, erythema on hands when she touches hot items, with slight itching or burning of short duration. She seems to tolerate more heat than previously. She is not on any medication, but takes Atarax 10 mg. on days she knows she'll be exposed to more heat.
Recently, a PPD test was done as a prophylactic measure with work scheduled in hospital settings, and the reaction was >20mm. Family and personal history is negative re tuberculosis and she did not have BCG vaccination. After being seen by a pneumologist, she's on Isoniazid for the next 6 months. Is there a link?
Nov. 14th, 2010
She has stopped taking Atarax or other antihistamines. Consuming hot beverages does not seem to bother her anymore and contact with heat causes only slight erythema without pruritus or pain. The problem seems to have dissapeared, almost.
Jan. 14th, 2012
A phone call made today revealed that she's completely tolerant of heat, not having any of the symptoms she once had with skin contact with heat or by drinking hot beverages. She is not taking any antihistamines as she once did.