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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 5  |  Issue : 1  |  Page : 123-125

Syringoma: A rare presentation at an unusual site


1 Department of Dermatology, Venereology and Leprosy, Sri Siddhartha Institute of Medical Sciences, Bengaluru Rural, Karnataka, India
2 Department of Dermatology, Venereology and Leprosy, ESIC Medical College and PGIMSR, Bengaluru, Karnataka, India

Date of Submission23-Dec-2019
Date of Decision10-May-2020
Date of Acceptance03-Jun-2020
Date of Web Publication19-Feb-2021

Correspondence Address:
Anitha Bhakthavatsalam
Department of Dermatology, Venereology and Leprosy, Sri Siddhartha Institute of Medical Sciences, T Begur, Bengaluru Rural, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/CDR.CDR_52_19

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  Abstract 


Syringomas are benign adnexal tumors arising from the lower intraepidermal and upper dermal eccrine sweat ducts, manifesting as asymptomatic, multiple tiny firm skin-colored papules, commonly over the lower eyelid and malar area of the face. A case of syringoma in an adult female patient presenting with asymptomatic lesions occurring exclusively on the vulva with an unusual presentation as a polypoidal growth is reported. Vulvar syringomas may not be recognized if they are asymptomatic or they may be misdiagnosed. Examination of the vulvar region should not be missed when syringoma is found outside the genital area. Syringomas should be considered in the differential diagnosis of vulvar pruritus, vulvar pain syndrome, and papulonodular lesions or polypoidal mass over the vulva.

Keywords: Polypoidal growth, syringoma, vulvar


How to cite this article:
Bhakthavatsalam A, Shivanna R. Syringoma: A rare presentation at an unusual site. Clin Dermatol Rev 2021;5:123-5

How to cite this URL:
Bhakthavatsalam A, Shivanna R. Syringoma: A rare presentation at an unusual site. Clin Dermatol Rev [serial online] 2021 [cited 2021 Feb 28];5:123-5. Available from: https://www.cdriadvlkn.org/text.asp?2021/5/1/123/309769




  Introduction Top


Syringomas are benign adnexal tumors arising from the lower intraepidermal and upper dermal eccrine sweat ducts. They manifest as asymptomatic, multiple tiny firm skin-colored papules, commonly over the lower eyelid and malar area of the face. In some patients, syringomas can occur on the genitalia causing itching and discomfort. We report a case of vulvar syringomas with an unusual presentation as polypoidal growth.


  Case Report Top


A 25-year-old married woman presented to the skin outpatient department with the complaints of multiple lesions on the external genitalia of 3 years duration. They were asymptomatic but would cause itching and also increase in size during menstruation. There was no history of any discharge from these lesions. They were confined only to external genitalia and not found anywhere else on the body. On further enquiring, there were no such lesions present in the spouse. She also denied any family history. The patient had no history of multiple sexual partners. On examination, multiple firm skin-colored polypoidal growth of size varying from 2 mm to 5 mm was seen distributed on the prepuce and labia minora. Furthermore, multiple tiny firm skin-colored papules were seen on the labia majora and mons pubis [Figure 1].
Figure 1: Polypoidal growth seen on the prepuce and labia minora and multiple papules seen on the labia majora and mons pubis

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A differential diagnosis of condylomata accuminata, syringoma, Fox-Fordyce's disease, and epidermal cyst was considered, and one of the lesions was excised and subjected to histopathological examination. Biopsy report revealed numerous small tubular structures lined by two rows of flat epithelial cells embedded in a dense stroma. Few of them had comma-shaped tail like extension of epithelial cells resembling tadpoles [Figure 2].
Figure 2: Small tubular structures lined by two rows of flat epithelial cells and few of them showing comma.shaped tail-like extension of epithelial cells (H&Ex40)

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With these clinical and histopathological features, a diagnosis of vulvar syringomas was made. The patient was given an insight about the dermatological condition and was advised to undergo electrocautery or carbon dioxide laser treatment.


  Discussion Top


Syringoma was first described in 1872 by Kaposi and Biesiadeki as Lymphangioma tuberosum multiplex. They are benign appendageal tumors (adenoma) originating from the lower intraepidermal and upper dermal portion of the eccrine sweat ducts. They are more common in females affecting predominantly adolescent age group but can manifest between the first and sixth decades of life. Syringomas appear as multiple, small firm skin colored to yellowish papules. The common sites of occurrence are lower eyelid and malar area of the face but can also be present in the axillae, neck, chest, abdomen, upper arms, and external genitalia. They are usually asymptomatic, but lesions occurring over vulva may be pruritic at times. They are generally bilateral and symmetrical. Syringomas are usually benign tumors, but a case with malignant changes was reported by Glatt et al. in 1984.[1] Syringomas show increased incidence in patients with Down's syndrome.[2] Furthermore, this disease has shown familial occurrence with autosomal dominant trait, but hereditary transmission has not been clarified.[3]

Based on morphological features and associations, four principle classification of syringoma has been proposed by Friedman and Butler: Localized disease occurring as solitary or multiple lesions, generalized disease occurring as multifocal pattern, association with Down's syndrome and familial cases of syringoma.[4]

Vulvar syringomas are not rare but are underreported because they either go unrecognized when asymptomatic or are misdiagnosed. The first patients of vulvar syringomas were reported by Carneiro et al. in 1971.[5] Vulvar syringomas have been described in association with extragenital lesions. Hence, examination of the vulvar region should not be missed when syringoma is found outside the genital area. Similarly, lower eyelid and malar area of the face should be examined without fail when a suspected syringoma is found in the vulva. Syringomas localized only to vulva are very rare, as in our patient.[6],[7]

Although syringomas are asymptomatic, vulvar syringomas cause itching and discomfort which exacerbates during summer, probably due to increased sweating, and during menstruation. They also increase in size during pregnancy, premenstrual period, during the use of oral contraceptive pills and usually occur in women during puberty. Based on these observations, it is considered that syringomas are hormonally responsive.[6],[7] Immunohistochemistry study of syringoma has shown progesterone positivity.[6] The main concerns associated with vulvar syringomas are pruritus and venerophobia.[8]

The uniqueness of our case report is syringoma occurring exclusively on the vulva without any extragenital lesions as a polypoidal growth. The polypoidal presentation of syringoma is extremely rare with very few cases being reported.[9],[10]

The various diseases which should be considered as the differential diagnosis for vulvar syringomas include steatocystoma multiplex, Fox-Fordyce disease, epidermal cyst, lymphangioma circumscriptum, angiokeratomas, condylomata accuminata, and fibroepithelal polyp. Biopsy confirms the diagnosis. Histopathological features of syringoma are normal epidermis with upper and middermis showing small cystic ducts containing colloid material and solid epithelial strands surrounded by fibrous stroma. Small ducts are lined by two rows of flat epithelial cells. Some ducts have comma-like tails of epithelial cells appearing like a tadpole.

Syringomas have to be treated either for cosmetic reasons when they occur in visible areas or when they are symptomatic. Medical modalities of treatment include topical atropine, topical tretinoin, and oral tranilast.[10],[11] More satisfactory results are achieved by electrodessication, partial removal by excision, cryotherapy with nitrogen oxide, and carbon dioxide laser treatment. Although pruritus may resolve, tumor recurrence and scar formation following treatment are common.[12]


  Conclusion Top


Syringoma presenting as polypoidal growth, localized only to vulva without extragenital lesions is rare. Vulvar syringomas are underreported because they may not be recognized over the genitalia if they are asymptomatic or they may be misdiagnosed. Syringomas should be considered in the differential diagnosis of vulvar pruritus, vulvar pain syndrome, and papulonodular lesions or polypoidal mass over the vulva. Inspection of genitalia should not be missed in all the patients with syringomas for the sake of completion of clinical examination.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Glatt HJ, Proia AD, Tsoy EA, Fetter BF, Klintworth GK, Neuhaus R, et al. Malignant syringoma of the eyelid. Ophthalmology 1984;91:987-90.  Back to cited text no. 1
    
2.
Schepis C, Siragusa M, Palazzo R, Ragusa RM, Massi G, Fabrizi G. Palpebral syringomas and Down's syndrome. Dermatology 1994;189:248-50.  Back to cited text no. 2
    
3.
Draznin M. Hereditary syringomas: A case report. Dermatol Online J 2004;10:19.  Back to cited text no. 3
    
4.
Friedman SJ, Butler DF. Syringomas presenting as milia. J Am AcadDermatol 1987;16:310-14.  Back to cited text no. 4
    
5.
Carneiro SJ, Gardner HL, Knox JM. Syringoma of the vulva. Arch Dermatol 1971;103:494-6.  Back to cited text no. 5
    
6.
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. 6
    
7.
Gerdsen R, Wenzel J, Uerlich 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. 7
    
8.
Agrawal S, Kulshrestha R, Rijal A, Sidhu S. Localized vulvar syringoma causing vulval pruritus and venerophobia. Australas J Dermatol 2004;45:236-7.  Back to cited text no. 8
    
9.
Kim NR, Cho HY, Kim KH, Ro JY, Lee SP. Vulvar syringomas presenting with a polypoidal mass or pruritus: Report of two cases. J Womens Med 2010;3:32-4.  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
    
11.
Iwao F, Onozuka T, Kawashima T. Vulval syringoma successfully treated with tranilast. Br J Dermatol 2005;153:1228-30.  Back to cited text no. 11
    
12.
Miranda JJ, Shahabi S, Salih S, Bahtiyar OM. Vulvar syringoma, report of a case and review of the literature. Yale J Biol Med 2002;75:207-10.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2]



 

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