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Indian Journal of Dermatology, Venereology and Leprology, Vol. 74, No. 2, March-April, 2008, pp. 185-186

Rama Rao GRaghu, Rani RRadha, Amareswar A, Krishnam Raju PV, Kumari PRaja, Kumar YHari Kishan

Surya Skin Care and Research Center, Visakhapatnam
Correspondence Address:Surya Skin Care and Research Center, #15-1-2, Gopal Sadan, Naoroji Road, Maharanipeta, Visakhapatnam - 530 002, Andhra Pradesh, graghuramarao@hotmail.com

A 26 year-old married woman presented with a two-year history of asymptomatic, multiple papular lesions on the vulva. There was no history of premarital or extramarital exposure. On clinical examination, there were multiple, small, firm, skin-colored papules on the vulva [Figure - 1]. The lesions were nontender and there was no lymphadenopathy. Systemic examination including per vaginal and per rectum examinations, revealed no abnormality. Routine investigations were normal. Test for HBsAg, HIV I and II, VDRL were non-reactive. A 3 mm punch biopsy was done for the lesion. Histopathology showed a normal epidermis and the dermis showed a number of small cysts and ducts lined by two rows of cells. Some of the cysts were filled with keratin material. Walls of some of the ducts had epithelial extensions [Figure - 2].

Syringoma is a benign tumor of eccrine sweat ducts characterized by single or multiple, small, firm, skincolored or yellowish papules. These papules have rounded or flat-topped surfaces and may or may not be pruritic. The most common site is the periorbital region (lower eyelid); other areas are cheeks, axillae, abdomen and genitals.[1]

Vulvar syringomas are rare with only a few cases reported in literature.[2-6] They occur in young women after puberty.[2] The most common presentation is that of multiple flesh-colored or brownish papules on the labia majora. These lesions are clinically difficult to differentiate from epidermal cysts, steatocystoma multiplex, condyloma, Fox-Fordyce disease, cherry hemangioma, angiokeratoma, soft fibroma and lymphangioma circumscriptum.[7] They are one of the causes for vulvar pruritus. Some patients have exacerbation of pruritus and increase in the size of lesions during the summer or during menstruation. A diagnosis of vulvar syringomas should be considered if a patient with lichen simplex chronicus of labia major responds poorly to oral antihistamine and topical steroids.[2] Similarly, in men, syringomas can occur albeit rarely on the penis.[6] A skin biopsy is necessary to confirm the diagnosis.

No effective treatment modality has been documented. Treatment options include excision, cryotherapy, electrosurgery and carbon dioxide laser treatment. Topical steroids and oral antihistamines were not effective in controlling pruritus in our patient. Topical atropine and topical tretinoin may be tried.[8] Carbon dioxide laser is an effective therapeutic modality in patients with intractable pruritus.[2]

Diagnosis : Syringoma of the Vulva

Discussion

Syringoma is a benign tumor of eccrine sweat ducts characterized by single or multiple, small, firm, skin-colored or yellowish papules. These papules have rounded or flat-topped surfaces and may or may not be pruritic. The most common site is the periorbital region (lower eyelid); other areas are cheeks, axillae, abdomen and genitals.^[1]

Vulvar syringomas are rare with only a few cases reported in literature.^{[2],[3],[4],[5],[6]} They occur in young women after puberty.^[2] The most common presentation is that of multiple flesh-colored or brownish papules on the labia majora. These lesions are clinically difficult to differentiate from epidermal cysts, steatocystoma multiplex, condyloma, Fox-Fordyce disease, cherry hemangioma, angiokeratoma, soft fibroma and lymphangioma circumscriptum.^[7] They are one of the causes for vulvar pruritus. Some patients have exacerbation of pruritus and increase in the size of lesions during the summer or during menstruation. A diagnosis of vulvar syringomas should be considered if a patient with lichen simplex chronicus of labia major responds poorly to oral antihistamine and topical steroids.^[2] Similarly, in men, syringomas can occur albeit rarely on the penis.^[6] A skin biopsy is necessary to confirm the diagnosis.

No effective treatment modality has been documented. Treatment options include excision, cryotherapy, electrosurgery and carbon dioxide laser treatment. Topical steroids and oral antihistamines were not effective in controlling pruritus in our patient. Topical atropine and topical tretinoin may be tried.^[8] Carbon dioxide laser is an effective therapeutic modality in patients with intractable pruritus.^[2]

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