Syringoma of the vulva

Abstract

Introduction: Syringomas are benign eccrine sweat duct tumors. They are typically found in young females predominantly over cheeks and lower eyelids. Rare sites for this tumour include the vulva, palm, soles and penis. Objective: To report a case of syringomas of the vulva which is a rare site for this tumour. Case report: We report a 52-year-old woman presenting with a 30-year history of multiple asymptomatic flesh-coloured non-tender papular lesions of the labia majora. Dermoscopy showed multiple glittering yellow whitish structures in clusters over fading pink background with cobble-stone appearance. Histopathological examination led to the diagnosis of vulvar syringomas. The lesions were treated with intralesional radiofrequency cautery. Conclusions:The vulva is a rare location of syringoma. Dermoscopy may be helpful in establishing early diagnosis and avoiding excessive surgical interventions.

Solitary Vulvar Syringoma With Deep Extension; Potential for Misdiagnosis as the Microcystic Adnexal Carcinoma (MAC)

“…Syringoma is treated mainly through nonsurgical modalities, and MAC requires complete excision. [3][4][5]7,14 Interestingly, they both have high recurrence rates-likely due to the diffuse dermal nature of both lesions. However, the local recurrence of MAC is significant (reported up to 50% with positive margins), with 40%-60% of patients experiencing one or more local recurrences anywhere from 6 months to 30 years after standard wide local excision.…”

“…2,5,7,10 Friedman and Butler proposed four variants of syringoma based on the clinical features that include localized, familial, generalized, and the form associated with Down syndrome. 2,7,8 Syringomas usually present with a bilateral, symmetrical distribution of multiple, small (1-5mm), smooth, skincolored, and dome-shaped papules. 1,4,5,18 The papules are classically painless but can also have associated pain and pruritus.…”

“…Adnexal tumors of the vulva comprise a large group of benign and malignant neoplasms that exhibit morphologic differentiation toward hair follicles, sebaceous glands, apocrine glands, eccrine glands, and nails. [1][2][3][4][5]7,8 Sweat gland tumors are rare, with a calculated incidence of less than 0.005% among all surgical pathologic specimens. 15 A subset of this group is syringoma, a benign adnexal tumor derived from intraepidermal luminal cells of eccrine sweat ducts.…”

“…3 It affects approximately 1% of the population. 7,16 The cause of syringoma is unknown. Hormonal influence and familial association are reported as predisposing factors for syringoma.…”

“…They most frequently involve the periorbital location. [1][2][3][4][5][6][7] On the other hand, MAC is a malignant adnexal tumor that typically presents as solitary, yellow, or flesh-colored, indurated plaque or cystic nodule. 1,2,[12][13][14] It predominantly occurs in the upper lip.…”