Therapeutic alternatives in Behçet's syndrome.

Behçet's Syndrome (BD) is a chronic, relapsing, recurrent systemic vasculitis with an unknown cause. The disease affects all organs of the body concurrently or consecutively. Its various clinical manifestations result from ubiquitous small-vessel vasculitis, which is the underlying pathology. An Italian study has reported an increased association of the extended haplotype B51-DR5-DQw3. Without a known etiology BD syndrome has no uniformly acceptable therapy. Our study addresses therapeutic alternatives for the treatment of BD, with the systemic use of interferon alpha-2a., which has antiviral. immunomodulatory, antiproliferative, and antitumoral properties. Ten patients diagnosed with BD were referred from September 2002 to September 2005 to the Department of Gynaecology, Obstetrics and Reproduction of the Second University of Naples. The International Study Group (ISG) Criteria for Behçet's Disease (27) was applied. Patients were treated with oral prednisone; sulfasalazine; clobetasol; and interferon alpha-2a. Every month all patients had a complete blood count, platelet count, and liver function test. Biopsies of genital ulcerations identified small vessel vasculitis with mononuclear cell and lynphocytic infiltrates. HLA-B27 and B5 were positive in three subjects. The pathergy test was positive in all patients. Today the therapy is still ongoing, and none of the patients in therapy with our protocol present clinical symptoms of BD or intolerance. Laboratory findings are in a normal range and none have had neurological failure. Our findings may be attributable to less severe disease in a patients, to our smaller number of patients, or to other unknown factors. Nonetheless, these findings remain to be confirmed in a larger number of patients.

A rare case of female pelvic mass: angioleiomyoma of the broad ligament.

Angioleiomyoma is a benign mesenchymal neoplasm composed of smooth muscle cells and thick-walled vessels. It is usually found in the skin of the lower extremities. Angioleiomyoma is a very rare tumor among the ever-expanding repertoire of growth variants described in benign uterine leiomyoma. More rare is a solitary tumor of the broad ligament. Thus angioleiomyoma of the broad ligament is an extremely rare benign tumor of the female pelvis. In this report a 52-year-old woman with a one-year history of abdominal pain was admitted to our hospital. Gynaecological and ultrasonography exams showed a large mass with increased vascularization in the right adnexal region. The patient underwent total hysterectomy and bilateral salpingo-oophorectomy. The site of the benign mass was the left broad ligament of the uterus. On pathologic examination of the specimen, the tumor was diagnosed as angioleiomyoma. We present a case of angioleiomyoma of the broad ligament because of its extreme rarity and the large size of the tumor.