Urticaria pigmentosa presenting as cardiac emergency
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Department of Dermatology, Sexually Transmitted Diseases and Clinical Immunology, Faculty of Medical Sciences, University of Warmia and Mazury in Olsztyn, Poland
Agnieszka Owczarczyk-Saczonek   

Department of Dermatology, Sexually Transmitted Diseases and Clinical Immunology, Faculty of Medical Sciences, University of Warmia and Mazury in Olsztyn, al. Wojska Polskiego 30, 10-229 [4TD$DIF][5TD$DIF]Olsztyn, Poland. Tel.: +48 89 678 66 70; fax: +48 89 678 66 75.
Submission date: 2016-07-05
Acceptance date: 2016-11-04
Online publication date: 2017-02-22
Publication date: 2019-12-15
Pol. Ann. Med. 2017;24(1):43–47
Urticaria pigmentosa is a common type of mastocytosis. It is a disease that needs to be treated by doctors of many specialties.

We present a case report based discussion concerning the approach to a patient with this disease including treatment, prophylaxis of degranulation and anesthesia in affected patients.

Case study:
A 57-year-old male patient was admitted for diagnosis of maculopapular lesions that first occurred about 10 years before. He presented massive maculopapular lesion covering his trunk and legs with positive Darie's sign. The punch biopsy revealed mastocytosis, the tryptase level was slightly elevated – 22 mg/L (N < 20 mg/L) and the trepanobiopsy revealed no involvement of the disease in bone marrow. We started PUVA therapy with improvement.

Results and discussion:
Patient was not diagnosed before although he had many serious symptoms of the disease which were ignored by both, doctors and the patient (fainting, the cardiorespiratory arrest and the seizures during the anesthesia, recurring diarrhea). After last incident, when the skin lesion was already covering almost whole trunk and legs he was sent to the Department of Dermatology for further diagnosis that was described in beginning.

Mastocytosis is a disease that is not easy to diagnose, classify and treat for doctors. But it is crucial that patients knowthe disease well and are informed on how to cope with the symptoms.

None declared.
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