|Year : 2020 | Volume
| Issue : 2 | Page : 140-142
Vulvar syringoma – A rare distribution of a common entity: Report of two cases
B Sathish Pai1, Kanthilatha Pai2, Varsha M Shetty1, Anuradha Jindal1, Sandhiya Ramesh1
1 Department of Dermatology, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, India
2 Department of Pathology, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, India
|Date of Submission||20-May-2019|
|Date of Acceptance||20-Aug-2019|
|Date of Web Publication||10-Nov-2020|
Dr. Varsha M Shetty
Department of Dermatology, Kasturba Medical College, Manipal Academy of Higher Education, Manipal - 576 104, Karnataka
Source of Support: None, Conflict of Interest: None
Syringomas are common benign appendageal tumors arising from eccrine sweat glands. They are commonly encountered in women, appearing as yellowish to skin-colored papules, 1–5 mm in diameter. They are typically distributed around the lower eyelids and malar areas; however, vulvar syringoma occurs rarely. Vulvar syringoma needs to be differentiated from papular eruptions occurring on the vulva. Histopathological examination may be needed to distinguish them from other disorders as clinical examination alone may fail to do so. Herein, we report two cases of vulvar syringomas.
Keywords: Papular lesions on the vulva, syringoma, vulva, vulvar syringoma
|How to cite this article:|
Pai B S, Pai K, Shetty VM, Jindal A, Ramesh S. Vulvar syringoma – A rare distribution of a common entity: Report of two cases. J Dermatol Dermatol Surg 2020;24:140-2
|How to cite this URL:|
Pai B S, Pai K, Shetty VM, Jindal A, Ramesh S. Vulvar syringoma – A rare distribution of a common entity: Report of two cases. J Dermatol Dermatol Surg [serial online] 2020 [cited 2020 Dec 4];24:140-2. Available from: https://www.jddsjournal.org/text.asp?2020/24/2/140/300388
| Introduction|| |
A syringoma is a benign adnexal tumor arising from eccrine sweat glands, typically found on the face, specifically the periorbital area. Vulvar syringomas are not very common, and occurrence without extragenital involvement is a rarer event. We report two cases of vulvar syringoma in middle-aged women. Vulvar syringomas need to be differentiated from papular eruptions occurring on the vulva. Histopathological examination may be needed to differentiate them from one another.
| Case Report|| |
Patient 1 is a 38-year-old woman who presented with multiple asymptomatic skin-colored papules on her labia majora for 10 months [Figure 1]. Patient 2 is a 31-year-old woman who presented with itchy flesh-colored papular lesions on her labia majora present for 10 years [Figure 2].
|Figure 1: Patient 1 showing multiple skin colored papules on the labia majora|
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Neither patient had similar appearing lesions elsewhere on their body. Patient 1 denied any previous treatment; conversely, patient 2 had tried topical antifungals with no relief.
Histopathology of the lesion from both the patients showed multiple cystically dilated glands lined by two-layered epithelium with luminal secretions and compressed tadpole-shaped tumor islands surrounded by fibrocollagenous tissue in the dermis. A diagnosis of vulvar syringoma was made [Figure 3].
|Figure 3: Epidermis overlying small ducts lined by two-layered epithelium, some of which showing tadpole- and comma-shaped configuration, H and E, ×200|
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| Discussion|| |
Syringomas are benign appendageal tumors derived from the intraepidermal portion of eccrine sweat ducts. They are commonly located on the face, neck, and trunk. They were first reported by Kaposi and Biesiadeki in 1872., They are frequently seen in middle-aged women as multiple skin-colored to yellowish asymptomatic papules measuring 1–5 mm in diameter. Syringomas of the genitalia are rare but may usually occur as a part of a more generalized distribution. Isolated genital occurrence is considered to be rare. In addition, vulvar syringomas may be associated with pruritus more so during summer and menses.
Morphologically, vulvar syringomas may present in three different forms [Table 1]., The most common is multiple flesh-colored to brownish papules which need to be distinguished from epidermal cysts, Fox–Fordyce disease, condylomas, and angiokeratomas. This was the variant observed in our patients. Discrete whitish cystic variant may mimic milia, and vulvar syringomas presenting as lichenified plaques may be confused for lichen simplex chronicus. In 18 patients with vulvar syringoma, one-third had extragenital involvement and 72% had associated pruritus.
Syringomas may be influenced by hormones as they increase in size during pregnancy, in the premenstrual period and with the use of oral contraceptives. However, there has been considerable variability in the staining of estrogen and progesterone receptors with some showing positive staining and some negative on immunohistochemistry.
There are limited treatment options for vulvar syringoma. In asymptomatic patients, the treatment is aimed at cosmetic improvement. Various topical agents such as corticosteroids, tretinoin, and tranilast have been tried with variable results. Alternatives include electrocautery, cryotherapy, carbon dioxide laser, and surgical excision.
Vulvar syringoma is an underreported entity, likely to be missed or misdiagnosed and is in the differential of papular vulvar eruptions. Vulvar syringomas are difficult to distinguish from other conditions on clinical examination alone. Hence, histopathological examination may be needed to rule out other disorders.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Akoglu G, Ibiloglu I, Durmazlar N. Vulvar nonclear cell syringoma associated with pruritus and diabetes mellitus. Case Rep Dermatol Med 2013;2013:418794.
Mendiratta V, Harjai B, Gupta T. Vulvar syringomas in an Indian female. Indian J Dermatol 2007;52:158-9. [Full text]
Nibhoria S, Tiwana KK, Yadav A. Vulvar syringoma: A rare case report. J Clin Diagn Res 2014;8:FD06.
Huang YH, Chuang YH, Kuo TT, Yang LC, Hong HS. Vulvar syringoma: A clinicopathologic and immunohistologic study of 18 patients and results of treatment. J Am Acad Dermatol 2003;48:735-9.
Yorganci A, Kale A, Dunder I, Ensari A, Sertcelik A. Vulvar syringoma showing progesterone receptor positivity. BJOG 2000;107:292-4.
Heller DS. Benign papular lesions of the vulva. J Low Genit Tract Dis 2012;16:296-305.
Amitha AA, Rao AC, Rao R. Pruritic papules on the vulva. Indian J Sex Transm Dis AIDS 2015;36:118-9.
[Figure 1], [Figure 2], [Figure 3]