Multiple syringomas of the vulva are an unusal finding. We report a 42-year-old woman with a 2 year history of asymptomatic yellow papules on the vulva that were proven to be syringomas by histopathologic examination.
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| Figure 1 |
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A 42-year-old woman presented with asymptomatic, multiple yellowish papules on the vulva of 2 years duration. 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. There was no history of high-risk sexual behavior. The rest of the cutaneous and systemic examination, including her vaginal and her rectal examinations, revealed no abnormality. Test for HBsAg, HIV I and II, and VDRL were non-reactive. There was no history of similar complaints in her husband.
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| Figure 2 |
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Skin biopsy taken from a papule showed normal epidermis with proliferation of eccrine ductal structures in the dermis; some of them resembled tadpoles. There were no horn cysts or milia and the stroma was mildly fibrotic. The ducts were lined by two layers of cuboidal cells and the ductal lumina were filled with an amorphous material (Figure 2).
Larger lesions were treated with radiofrequency ablation. However, we lost the patient to follow up after one treatment.
Syringoma is a benign adnexal tumor derived from the eccrine sweat ducts [1]. The most common presentation is characterized by multiple, small, firm, flesh colored papules, most commonly located on the face (periorbital area) and neck. The papules have rounded or flat-topped surfaces and may be pruritic. Other areas involved are cheeks, axillae, abdomen, and genitals [2].
Involvement of the vulva is relatively rare and usually occurs in young women after puberty. The most common presentation is that of multiple skin colored or brownish and yellowish papules on the labia majora that may be associated with intense pruritus or distress [3]. Friedman and Butler classified syringomas based on the clinical features and associations into four principal clinical variants. These are a localized form, a familial form, a form associated with Down's syndrome, and a generalized form that encompasses eruptive and multiple syringomas [1].
Clinically, lesions over the vulva may need to be differentiated from epidermoid cysts, steatocystoma multiplex, early condyloma, Fox-Fordyce disease, lymphangioma circumscriptum, angiokeratoma, and molluscum contagiosum [2, 3].
Histologically, syringomas show, normal epidermis with numerous dermal tumors composed of multiple small ducts of the eccrine type, usually lined by two layers of cuboidal epithelium. Some of the ducts possess a small comma like tail of epithelial cells giving the appearance of a tadpole. Solid nests and strands of cells with a basaloid appearance may be present as seen in our case. Surrounding stroma may be fibrotic [1, 4].
The exact pathogenesis of syringoma is not known and the inflammatory process seen around the eccrine ducts may be secondary to neoplastic proliferation of ducts.
It has also been hypothesized that the growth of syringoma is under hormonal influence, but a study by Yu-Huei Huang et al found no expression of estrogen or progesterone receptors in their 15 patients [4, 5].
Treatment of syringoma is mainly cosmetic and many treatment modalities are used. These include dermabrasion, electrodesiccation with curettage, scissor excision, carbon dioxide (CO2) laser, argon laser, 585-nm and 595-nm pulsed dye laser, erbium-YAG laser, and the combination of TCA and CO2 laser, all of which have been tried with some success. Oral and topical retinoids may also be useful. Topical steroids and oral antihistamines may not be effective in controlling pruritus [2, 4, 5].
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