[Vulvar syringoma: a rare cause of vulvar pruritus].

Although vulvar syringomas are rare, they are probably underdiagnosed because most are asymptomatic lesions. However, they can give rise to vulvar pruritus that is unresponsive to conventional treatments and, if severe, may considerably affect quality of life. Consequently, such lesions should be considered in the differential diagnosis of pruritus and papular lesions of the vulva. 1,2 We describe a 78-year-old woman with a 9-year history of urinary incontinence who consulted for intermittent, nonseasonal vulvar pruritus that had started 4 years earlier. She was treated with oral antihistamines and topical corticoids, but did not improve. The physical examination showed multiple papules of 3 to 5 mm that were flesh-colored and of lichenoid appearance. Some of these papules were eroded on the surface and coalesced to form plaques on both of the labia majora (Figure). No such lesions were observed at other sites. Contact allergy testing based on the standard Spanish panels was positive at 96 hours for the perfume blend and the black rubber blend. Biopsy of a papule showed a proliferation composed of nests and ducts of epithelial cells embedded in a stroma of collagen bundles. The ducts were lined with 2 layers of cuboid cells and some showed tadpole-like extensions. These findings were consistent with eccrine syringoma. The lesions were treated by carbon dioxide laser, with clear improvement in both the symptoms and the lesions. After 11 months of follow-up, the patient has presented few episodes of pruritus, all less severe than before and controlled with topical corticoids. Syringoma is a benign tumor of eccrine origin usually located in the periocular region; however, there are other, more unusual sites, such as the vulva. Simultaneous involvement of the vulvar region and extragenital areas has been described. 1 It mainly affects women during puberty and middle age, 1-3 and rarely manifests in the later years as in our case. Syringomas are usually asymptomatic, but can cause pruritus in the vulva. On occasions, pruritus may become more severe during menstruation, pregnancy, and summertime. 1,5 A review of the published cases of vulvar syringomas indicates that pruritus develops over a number of years before the definitive diagnosis, 2,4-8 severely impacting on quality of life. Persistence of the lesions may occasionally cause venereophobia and carcinophobia. Three clinical forms of presentation of vulvar syringomas have been described. Most commonly, they appear symmetrically on the labia majora as multiple flesh-colored or brownish papules. The other presentations …