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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 37  |  Issue : 1  |  Page : 15-17

Vulvar syringomas in a human immunodeficiency virus-infected patient: a rare presentation


Department of Dermatology, Venereology and Leprosy, Narayana Medical College and Hospital, Nellore, Andhra Pradesh, India

Date of Submission14-Jun-2016
Date of Acceptance17-Nov-2016
Date of Web Publication2-Jun-2017

Correspondence Address:
V M Avisa Rao
Department of Dermatology, Venereology and Leprosy, Narayana Medical College and Hospital, Nellore, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1110-6530.207485

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  Abstract 

Syringomas are appendageal tumors of intraepidermal eccrine sweat ducts found more commonly in women over the malar and periorbital regions. Here, we report on a 30-year-old HIV-positive woman on antiretroviral therapy for the past 3 years presenting with pruritic papules on the vulva. The diagnosis of vulvar syringoma was made on the basis of the characteristic double-lined ductal structures and solid epithelial cords found during a histopathological examination.

Keywords: appendageal tumor, HIV infection, vulvar syringomas


How to cite this article:
Avisa Rao V M, Kumar HN, Natraj C H, Rajamma U. Vulvar syringomas in a human immunodeficiency virus-infected patient: a rare presentation. Egypt J Dermatol Venerol 2017;37:15-7

How to cite this URL:
Avisa Rao V M, Kumar HN, Natraj C H, Rajamma U. Vulvar syringomas in a human immunodeficiency virus-infected patient: a rare presentation. Egypt J Dermatol Venerol [serial online] 2017 [cited 2017 Jun 27];37:15-7. Available from: http://www.ejdv.eg.net/text.asp?2017/37/1/15/207485


  Introduction Top


Syringomas are benign neoplasms of eccrine lineage [1] that occur more commonly in the middle-aged women over the periorbital location [2]. They may also appear on the scalp, forehead, neck, anterior chest, axillae, upper abdomen, and extremities. Clinically syringomas appear as small, multiple, and firm skin-colored to yellowish papules, 1–3 mm in diameter, distributed symmetrically over the lower eyelids and malar areas. Syringomas localized to the genitalia occur rarely. Vulvar syringomas may be symptomatic with a complaint of itching [3]. We report this case of multiple vulvar syringomas in a 30-year-old Indian woman with HIV infection; this has not been reported in the literature to date.


  Case report Top


A 30-year-old HIV-positive woman on antiretroviral therapy for the past 3 years presented with a 3-month history of pruritic papules on the vulva. Her husband died of HIV encephalopathy 3 years ago. There was no history suggestive of extramarital sexual exposure, menstrual flare, and diabetes mellitus and tuberculosis, or contact with chemicals.

Cutaneous examination indicated multiple, discrete, brown-colored papules, measuring up to 4 mm in diameter, distributed symmetrically over the labia majora ([Figure 1]). The rest of the mucocutaneous examination, including per vaginal, per speculum, and per rectal examination, was normal. Systemic examination was normal. A clinical differential diagnosis of bowenoid papulosis, Fox–Fordyce disease, and condyloma acuminatum was considered.
Figure 1: Multiple brown-colored papules over the labia majora.

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Routine blood investigations were within normal limits. Her CD4 count was 400 cells/mm3. Tests for venereal disease research laboratory, hepatitis B surface antigen, and hepatitis C virus were nonreactive. Immunohistochemical stains for receptors of estrogen and progesterone could not be performed because of nonavailability. Punch biopsy of papule over the right labium majus was performed.

Histopathology indicated an epithelial neoplasm consisting of solid epithelial islands and ductal structures lined by two or three layers of cuboidal cells surrounded by hyalinized stroma. The cells within solid cords showed ductal differentiation ([Figure 2] and [Figure 3]). These histopathological findings were suggestive of syringoma. Histopathological differentials such as desmoplastic trichoepithelioma and microcystic adnexal carcinoma were ruled out as there were no horn cysts and infiltration of fat tissue with cellular atypia.
Figure 2: Epithelial ducts and solid cords resembling sweat ducts. H&E, ×10.

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Figure 3: Multiple ducts lined by two to three layers of cuboidal cells. H&E, ×40.

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The patient was prescribed topical tretinoin 0.025% cream application at night and tablet levocetirizine 5 mg at night. On review, after 4 weeks, there was no improvement in the lesions. Electrosurgery or cryotherapy of the lesions was advised, but the patient refused to undergo the treatment and she was lost for further follow-up.


  Discussion Top


Syringoma is a benign adenexal tumor derived from eccrine sweat ducts. Its name is derived from the Greek word syrinx, which means pipe or tube [4],[5]. Syringoma was first described in 1872 by Kaposi and Biesiadeki as lymphangioma tuberosum multiplex. The first patient with vulvar syringoma was reported by Carneiro and colleagues in 1971 [4],[5].

Vulvar syringomas alone are rare and may present as multiple brown or skin-colored, small 1–5 mm-sized papules on the labia majora and may be associated with pruritus. Yorganci et al. [6] reported progesterone receptor positivity of vulvar syringoma in one case.

Biopsy with a microscopic examination is the key in establishing the diagnosis and to rule out diseases such as epidermal cysts, steatocystoma multiplex, condyloma acuminata, Fox–Fordyce disease, cherry hemangiomas, angiokeratomas, soft fibromas, lymphangioma circumscriptum [3], irritant or allergic contact dermatitis, lichen simplex chronicus [4],[5], and bowenoid papulosis.

Histologically embedded in a fibrous stroma are numerous small ducts, the walls of which are usually lined by two rows of epithelial cells. Some of the ducts have small, comma-like tails of epithelial cells, giving them an appearance resembling tadpoles [2].

Treatment modalities of syringoma are mainly cosmetic and these include electrodessication with curettage, excision, CO2 laser, argon laser, 585 and 595 nm pulsed dye laser, Erbium YAG laser, and the combination of TCA and CO2 laser and cryotherapy, all of which have been attempted with some success. Oral and topical retinoids may also be useful [7].

Most of the patients in earlier series were immunocompetent. A single report of vulval lesions with diabetes mellitus has been reported [8]. Our patient was a known retroviral positive who was on antiretroviral therapy.

Vulvar syringomas in HIV-infected patient have not been reported in the literature to date

Acknowledgements

This study was supported by Dr Uday Khopkar.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Hashimoto K, Lever WF. Histogenesis of skin appendageal tumors. Arch Dermatol 1969; 100:356–369.  Back to cited text no. 1
[PUBMED]    
2.
Elder D, Elenitsas R, Ragsdale BD. Tumors of the epidermal appendages. In: Elder D, Jaworsky C, Johnson B Jr, editors. Lever’s histopathology of the skin [chapter 30]. Philadelphia: Lippencott-Raven; 1997. 778–779.  Back to cited text no. 2
    
3.
Young AW Jr, Herman EW, Tovell HM. Syringoma of the vulva: incidence, diagnosis and cause of pruritus. Obstet Gynaecol 1980; 55:515–518.  Back to cited text no. 3
    
4.
Miranda JJ, Shahabi S, Salih S, Bahtiyar OM. Vulvar syringoma, report of a case and review of the literature. Yale J Biol Med 2002; 75:207–210.  Back to cited text no. 4
    
5.
Gerdsen R, Wenzel J, Verlich M, Bieber T, Petrow W. Periodic genital pruritis caused by syringoma of the vulva. Acta Obstet Gynecol Scand 2002; 81:369–370.  Back to cited text no. 5
    
6.
Yorganci A, Kale A, Dunder I, Ensari A, Sertcelik A. Vulvar syringoma showing progesterone receptor positivity. Br J Obstet Gynaecol 2000; 107:292–294.  Back to cited text no. 6
    
7.
Gutte RM, Chikhalkar S, Khopkar U. Unknown multiple asymptomatic skin coloured to yellowish papules over vulva. Dermatol Online J 2011; 17:7.  Back to cited text no. 7
    
8.
Akoglu G, Ibrahim I, Nezih D. Vulvar nonclear cell syringoma associated with pruritus and diabetes mellitus. Case Rep Dermatol Med 2013; 2013:418794  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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