Exercise-induced urticarial vasculitis as a paraneoplastic manifestation of cystic teratoma

F. Di Stefano, S. Siriruttanapruk and M. Di Gioacchino

Department of Medicine and Science of Ageing, University ‘G. D’Annunzio’, Via dei Vestini, 66100 Chieti, Italy

Correspondence to: F. Di Stefano, Internal Medicine, Presidio Ospedaliero ‘G. Bernabeo’, C. da S. Liberata, 66026 Ortona (Chieti), Italy. E-mail: distefa.fabio{at}tiscali.it

SIR, Urticarial vasculitis (UV), which is characterized clinically by urticarial lesions that last for more than 24 h and resolve with purpura or hyperpigmentation, and histologically by leucocytoclastic vasculitis [1], has been described as a rare association with visceral and haematological malignancy [27]. UV has also been described in a few patients as developing reproducibly after exercise [8].

We report a patient who developed UV after exercise, who was incidentally diagnosed as having a cystic teratoma, and who did not suffer from the exercise-induced UV after the tumour had been surgically resected.

A 42-yr-old man presented with a 1-month history of recurring erythematous wheals over the lower extremities which appeared a few hours after physical exercise (on every occasion after his weekly jogging). Each lesion persisted for more than 24 h but disappeared after less than 3 days, leaving the area with slight pigmentation. He was examined during the eruption and blood was taken for investigation. He had a normal haemoglobin concentration and total and differential white blood cell counts. The erythrocyte sedimentation rate was 29 mm/h (normally <20 mm/h). C-reactive protein concentration, plasma protein electrophoresis, immunoglobulins, complement components, liver function tests, urea and creatinine, urinalysis, plasma sodium, potassium, glucose and thyroid function were within normal ranges. Screening for hepatitis B and C virus infection was negative. Antinuclear antibodies and cryoglobulins were not detected. Skin biopsy of an urticarial lesion demonstrated leucocytoclastic vasculitis. After complete resolution (within 3 days and without any therapy), challenge by physical exercise was performed to determine whether exercise could reproduce the urticarial lesions. Exercise challenge was performed on a cycle ergometer for a total of 6 min at room temperature. The workload was increased in a stepwise manner until 85% of the subject's predicted value of maximal heart rate was reached. Within 5–6 h after exercise challenge, urticarial lesions developed on the patient's legs and started to disappear at 72 h, leaving slight pigmentation. Skin biopsy was carried out at 24 h, and histology was again indicative of leucocytoclastic vasculitis. The investigations were completed with abdominal ultrasound, which was normal, and a chest X-ray, which showed a tiny widening of the superior mediastinum. Therefore, the patient underwent a chest computed tomography scan, which showed a cystic mass in the anterior superior mediastinum. A chest MRI scan revealed that this mass was very well demarcated and had the imaging features of a cystic teratoma (Fig. 1). The mass was surgically resected, and histology of the specimen confirmed the diagnosis of cystic teratoma. Six months after the operation, the patient restarted his weekly jogging and told us that he did not suffer the urticarial lesions after physical exercise. He repeated the exercise challenge, which did not provoke any cutaneous lesions.



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FIG. 1. Chest MRI of a 42-yr-old man with exercise-induced UV. The scan shows a well-demarcated cystic mass in the anterior superior mediastinum, suggesting a diagnosis of cystic teratoma.

 
We believe this is the first report of UV as a paraneoplastic manifestation which is triggered by physical exercise. The exact pathogenesis of the association of UV with malignancy is unclear. It is thought that tumour-associated immune complexes may give rise to complement fixation in the vessel wall and subsequent development of an inflammatory process [6]. In exercise-induced UV it has been shown that mast cells are the first to be involved, with the activation and subsequent release of proinflammatory mediators, which precede the influx of eosinophils with their release of granule proteins and the influx of neutrophils with their release of proteolytic enzymes [8]. We hypothesize that deposits of immune complexes and complement fixation in the vessel wall sometimes need an additional event in order to elicit the initial influx of neutrophils, which are the final effector cells responsible for the vascular damage.

The patient gave informed consent to the investigations that led to the diagnosis reported here, and written permission was given for this case to be reported.

The authors have declared no conflicts of interest.

References

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  7. Wilson D, McCluggage WG, Wright GD. Urticarial vasculitis: a paraneoplastic presentation of B-cell non Hodgkin's lymphoma. Rheumatology 2002;41:476–7[Free Full Text]
  8. Kano Y, Orihara M, Shiohara T. Cellular and molecular dynamics in exercise-induced urticarial vasculitis lesions. Arch Dermatol 1998;134:62–7[Abstract/Free Full Text]
Accepted 11 March 2003





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