Indian Journal of Allergy, Asthma and Immunology

: 2016  |  Volume : 30  |  Issue : 1  |  Page : 42--44

Rash before shock: A case of cholinergic urticaria associated with syncope

Anand Kumar Vaggu, Prachi Rakesh Srivastava 
 Department of Dermatology, Krishna Institute of Medical Sciences, Secunderabad, Hyderabad, Telangana, India

Correspondence Address:
Prachi Rakesh Srivastava
Krishna Institute of Medical Sciences, Ministers Road, Secunderabad, Hyderabad, Telangana


We report a case of cholinergic urticaria (CU) associated with systemic manifestations, in the form of syncope. A 21-year-old female patient, suffering for 14 years, triggers being strong emotional reactions, foul smell, and sunlight. She was diagnosed after a thorough history and extensive literature search as systemic symptoms are not usually suspected in the spectrum of CU. She was effectively managed with nonsedating antihistamines.

How to cite this article:
Vaggu AK, Srivastava PR. Rash before shock: A case of cholinergic urticaria associated with syncope.Indian J Allergy Asthma Immunol 2016;30:42-44

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Vaggu AK, Srivastava PR. Rash before shock: A case of cholinergic urticaria associated with syncope. Indian J Allergy Asthma Immunol [serial online] 2016 [cited 2023 Mar 23 ];30:42-44
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Full Text


Cholinergic urticaria (CU) is a rare form of physical urticaria presenting with pruritic, pinpoint wheals usually triggered by heat, exercise, and emotional stress, but other factors such as foul smells and spicy food can also bring on the attacks. Systemic manifestations in CU are further rare present in only 10% of the cases. It is diagnosed on the basis of patient history and examination; however, provocation tests are confirmatory. We report this case for the rarity of systemic involvement in CU.


A 21-year-old female presented to the emergency department with a history of fainting episode, following physical exercise in the form of skipping. It was associated with fecal incontinence. She was admitted to the Neurology Department for evaluation suspecting epilepsy. Since there was a history of development of rash prior to the episode of fainting, dermatological opinion was sought. On inquiry, there was a history of development of papular lesions along with itching and burning following exposure to sunlight, spicy food, peanut preparations, foul smell, emotional situations, and also exercise since 7 years of age. She reported a typical sequence of reactions starting with a feeling of warmth, followed by the development of papular lesions all over the body, headache which subsided on drinking water and was associated with chills and rigor, and then fainting with spontaneous recovery and subsidence of rash. She had three episodes of fainting associated with these episodes in the past 2 years with the most recent episode accompanied with fecal incontinence. Earlier two episodes were preceded by standing in the sun while waiting for the bus and present episode was precipitated by physical exercise.  There was neither family history of similar episodes nor personal or family history of atopy. The patient was investigated, and her neurological profile including magnetic resonance imaging of the brain and electroencephalogram and biochemical investigations were normal except serum IgE, which was grossly elevated that is 772 IU/L (1.31-165.5 IU/L).

Based on the above history, clinical picture, and investigations, after a thorough literature search, a diagnosis of CU with systemic (cardiovascular) involvement in the form of syncope was made. Electrocardiogram and two-dimensional echo performed were normal and cardiologist opinion was sought ruling out any other cardiovascular abnormality. Exercise-induced anaphylaxis was ruled out as patient had these episodes even in the absence of exercise on exposure to sunlight, spicy food, emotional situations, all of which induced sweating in the patient. The relatives did not agree for a provocation test. She was managed with oral desloratadine 5 mg at bedtime to which she responded very well and had been symptom-free ever since.


CU is a rare form of physical urticaria, first reported by Duke in 1924. [1] It is clinically characterized by highly pruritic pinpoint wheals which are syringeal coincident. [1],[2] In approximately 10% of the patients, the cutaneous features are associated with systemic symptoms as was the case in our patient. [3] The peak incidence is seen in the age group of 20-28 years. [4]

CU can be classified into three types: (1) CU with sweat hypersensitivity, (2) CU with associated hypohidrosis/anhidrosis, and (3) idiopathic CU.

Our patient was suffering from the first type of CU. A hypothesis that has been put forth for this type says that patients are hypersensitive to unknown substances in their sweat and develop wheals in response to sweat substance leaking from the syringeal ducts to the dermis possibly by obstruction of the ducts. [1] Acetylcholinesterase (AChE) is a processing enzyme of acetylcholine and disordered AChE might contribute to the pathogenesis. [2] The increased acetylcholine levels then cause degranulation of mast cells thus causing whealing which is syringeal dependent. [1] It is thus the result of rise in core body temperature, manifested beyond a rise of 1°C thereby promoting sweating. [1],[3],[4] It is diagnosed by either provocation test with exercise or autologous sweat testing. [1],[5]

CU with systemic manifestations is rare and often missed. The various systemic manifestations include upper airway obstructive symptoms such as throat constriction, dysphagia, dysphonia, inspiratory stridor, lower airway symptoms such as wheezing, cough, chest tightness and dyspnea, gastrointestinal involvements such as nausea, vomiting, crampy abdominal pain, diarrhea, and cardiovascular manifestations such as presyncope, syncope, and palpitations. [6]

A study by Davis et al. suggested that histamine released from circulating basophils is responsible for systemic symptoms whereas degranulation from cutaneous mast cells causes local pruritus and urticaria. [7]

Vadas et al. reported 78.9% upper airway involvement, 78.9% lower airway involvement, 57.9% gastrointestinal system, and 78.9% cardiovascular system involvement in their study of 19 patients having CU with systemic involvement. [6]

Our patient presented with only cardiovascular manifestations.

Because of the relative lack of familiarity with systemic involvement in this condition, there is often a delay between the onset of symptoms and diagnosis, thereby causing a major impact on the quality of life and many unnecessary investigations and treatments of this otherwise easy to manage the condition.

Management is primarily with nonsedating antihistamines. For nonresponsive cases, ketotifen, montelukast, propranolol, and danazol have been tried. Desensitization with autologous sweat and the IgE-inhibitor omalizumab have recently been tried. [1],[4],[8]


We thus try to bring to focus the systemic manifestations of CU which are generally not suspected in its spectrum. Prompt identification based on a history of specific triggers will obviate unnecessary investigations, and effective education will help to avoid triggers of this rare form of urticaria.

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Conflicts of interest

There are no conflicts of interest.


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