Description of the clinical case:

A 79-year-old man who consulted for an episode of facial angioedema without other symptoms. Upon physical examination, he presented erythema on the face and neck, peri-palpebral angioedema and erythematous macules on the back of the hands. A biopsy of a skin lesion compatible with Gottron papule was performed. A computed tomography urography was performed to rule out systemic disease, observing grade III left ureterohydronephrosis, possibly due to ureteral neoplasia. Extension study negative. Laparoscopic left nephroureterectomy and endovesical instillation of Mitomycin C were performed, with anatomopathological results of multifocal high grade papillary urothelial carcinoma. The patient presented a favorable postoperative evolution with improvement of dermal lesions, but 7 months later he developed multiple metastases and died.

Relevance:

The association of dermatomyositis with neoplasms is well known, but few cases have been described with urological malignancy. Ureteral cancer with amyopathic dermatomyositis is extremely rare.

Clinical implications:

It is important to perform cancer screening in patients with dermatomyositis in order to treat them and improve their prognosis.

Conclusion:

Dermatomyositis is a rare paraneoplastic syndrome that is very infrequently associated with ureteral cancer. Cancer screening in these patients is very important to improve the prognosis. The surgical treatment of this tumor led to the disappearance of the skin changes.

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