Indian Journal of Dermatology
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CASE REPORT
Year : 2007  |  Volume : 52  |  Issue : 3  |  Page : 158-159
Vulvar syringomas in an Indian female


Department of Dermatology and STDs, Lady Hardinge Medical College and Associated Hospitals, New Delhi, India

Correspondence Address:
Tanvi Gupta
Department of Dermatology and STDs, Lady Hardinge Medical College and Associated Hospitals, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.35353

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   Abstract 

Syringomas are benign appendageal tumors of the eccrine sweat glands which are more common in women over the extragenital sites like the periorbital, cervical and axillary areas and the chest. Vulvar syringomas although not rare, are reported uncommonly perhaps owing to a missed diagnosis. Vulvar Syringomas are remarkable for pruritus. A case of multiple vulvar syringomas is reported in an Indian female.


Keywords: Syringoma, uncommon, vulvar


How to cite this article:
Mendiratta V, Harjai B, Gupta T. Vulvar syringomas in an Indian female. Indian J Dermatol 2007;52:158-9

How to cite this URL:
Mendiratta V, Harjai B, Gupta T. Vulvar syringomas in an Indian female. Indian J Dermatol [serial online] 2007 [cited 2019 Dec 12];52:158-9. Available from: http://www.e-ijd.org/text.asp?2007/52/3/158/35353



   Introduction Top


Syringomas are benign adnexal tumors that occur more commonly in the middle-aged females over the periorbital location. [1] They often appear at adolescence, but can develop later also. The clinical presentation is in the form of multiple or solitary, localized or generalized, small, skin-colored to yellowish papules up to 1-5mm in diameter. The chief areas that are affected are the chest, face, the neck and extremities. [2],[3]

In general, these tumors are asymptomatic and permanent, but they can undergo cyclic changes in their size and symptoms during the premenstrual period, pregnancy and with the use of oral contraceptives. [4],[5]

Syringomas affecting the genital area are rare and may affect the genital areas as part of their generalized occurrence. [6] Syringomas localized only to the vulva are very rare. [7],[8],[9] Vulvar syringomas may be symptomatic with a complaint of itching. Asymptomatic lesions are usually not detected by the clinicians due to their histopathological characteristics.

Host of treatment modalities have been employed in the past for the treatment of vulvar syringomas that range from topical atropine [10] and topical tretinoin [11] to excision, cryotherapy, electrosurgery and CO 2 laser. [10]

Although syringomas are benign and require treatment only for cosmetic reasons, they may cause considerable discomfort due to pruritus and anxiety that may lead on to sexual maladjustments and venerophobia.

We report this case of multiple vulvar syringomas in a 32-year-old Indian female; this was localized at labia majora, which were extremely pruritic.


   Case Report Top


A 32-year-old married woman presented with a 3-year history of pruritic vulvar skin lesions. She did not complain of any exacerbation of the pruritus, particularly during menstruation or in the summer. There was no history of similar lesions elsewhere or in any other family member. She had been treated with topical steroids and topical antimycotic preparations in the past without any relief.

On examination, there were multiple, discrete, asymptomatic, firm, dark-brown-colored papules measuring upto 5mm in diameter present on the labia majora [Figure - 1]. Per speculum examination and the bacteriological swabs from both the vulva and the vagina were normal. Systemic examination was also normal.

A clinical differential diagnosis of Fox-Fordyce's disease, milia and epidermal cyst were considered and one of the lesions was excised for histopathological examination. Histopathology revealed numerous tubular structures with central lumina and a thin epithelial lining along with the presence of comma-shaped appendages embedded in a fibrous stroma in the papillary and reticular dermis; all these observations were consistent with the clinical diagnosis of syringoma. Immunohistochemistry for estrogen and progesterone receptors could not be performed due to its nonavailability. The patient was advised to undergo CO 2 -laser-assisted removal of the rest of the lesions.


   Discussion Top


Extragenital syringomas are common place unlike the vulvar syringomas. Both these types may coexist. A large series describes the clinicopathologic and immunohistologic features in 18 patients; [10] a total of six patients also had coexisting periorbital syringomas years before the occurrence of vulval lesions.

Our patient had only vulval lesions similar to the earlier cases. [7],[8],[9] The lesions were moderately pruritic similar to most of the other cases; however, there was no exacerbation of pruritus or increasing size of lesions during the summer or menstruation in contrast to the other seven patients in the series of cases of vulvar syringomas. [10] The exacerbation in summer could possibly arise from increased sweating, and topical atropine could effectively control the pruritus and reduce the size of the lesions. [10] Morphologically, three clinical appearances of the lesions have been described with multiple flesh-colored or brownish papules as the most common presentation and same was observed in our case.

We cannot comment on the estrogen or progesterone receptor sensitivity of the lesions in our case. Previous studies have shown the variable expressions of progesterone and estrogen receptors with the positive staining of progesterone receptor marker in eight out of nine extragenital syringoma cases. [11]

In conclusion, we summarize that vulvar syringomas are underreported as they may not be recognized over the genitalia in case they are asymptomatic. Moreover, the possibility of vulvar syringomas must be considered in all papulonodular lesions over the vulva.

 
   References Top

1.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. Lippencott- Raven: Philadelphia; 1997. p. 778-9.  Back to cited text no. 1      
2.Tagami H, Jnove F, Yamada M, Egami K. Syringomas of the forehead. Int J Dermatol 1983;22:113-4.  Back to cited text no. 2      
3.van den Broek H, Lundquist CD. Syringomas of the upper extremities with onset in the sixth decade. J Am Head Dermatol 1982;6:534-6.  Back to cited text no. 3      
4.Gottz RW. Syringoma (syringocystadenoma). In : Dermis J, Dobson RL, McGuire J, editors. Clinical dermatology. Harper and Row: Hagerstown, Maryland; 1976. p. 22-4.  Back to cited text no. 4      
5.Turan C, Vgur M, Kutlvay L, Kukner S, Dabakoglu T, Aydogdu T, et al . Vulvar syringoma exacerbation during pregnancy. Eur J Obstet Gynecol Reprod Biol 1996;64:141-2.  Back to cited text no. 5      
6.Scherbenske JM, Lupton GP, James WD, Kiruk DB. Vulvar syringomas occurring in a 9 year old child. J Am Acad Dermatol 1988;19:575-7.  Back to cited text no. 6      
7.Zhu WY. Vulvar syringoma associated with epidermal cyst. Int J Dermatol 1989;28:142-3.  Back to cited text no. 7  [PUBMED]    
8.Yorganci A, Kale A, Dunder I, Ensari A, Sertcelik A. Vulvar syringoma showing progesterone receptor positivity. BJOG 2000;107:292-4.   Back to cited text no. 8  [PUBMED]  [FULLTEXT]  
9.Gerdsen R, Wenzel J, Verlich M, Bieber T, Petrow W. Periodic genital pruritus caused by syringoma of the vulva. Acta Obstet Gynecol Scand 2002;81:369-70.  Back to cited text no. 9      
10.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.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]  
11.Wallace MI, Smaller BR. Progesterone receptor positivity supports hormonal control of syringomas. J Cutan Pathol 1995;22:442-5.  Back to cited text no. 11      


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    Abstract
    Introduction
    Case Report
    Discussion
    References
    Article Figures

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