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  Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 39  |  Issue : 2  |  Page : 136-138
 

Acquired angiokeratomas of the vulva


Department of Dermatology, Dr. D. Y. Patil Medical College and Hospital, Pune, Maharashtra, India

Date of Web Publication7-Dec-2018

Correspondence Address:
Dr. Kirti Deo
G-11, Gokhale Vrindavan, Chaphekar Chowk, Chinchwad, Pune - 411 033, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijstd.IJSTD_50_16

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   Abstract 

Angiokeratomas are benign cutaneous lesions consisting of numerous ectatic blood vessels present in the superficial dermis and usually involving the scrotum. Although usually asymptomatic, they may be complicated by sporadic or profuse bleeding. We report the development of multiple vulvar angiokeratomas in a 40-year-old female after undergoing a perineoplasty successfully treated with electrocautery.


Keywords: Angiokeratoma, perineoplasty, vulva


How to cite this article:
Deo K, Dash KN, Sharma YK, Wadhokar M, Gupta A. Acquired angiokeratomas of the vulva. Indian J Sex Transm Dis 2018;39:136-8

How to cite this URL:
Deo K, Dash KN, Sharma YK, Wadhokar M, Gupta A. Acquired angiokeratomas of the vulva. Indian J Sex Transm Dis [serial online] 2018 [cited 2019 Mar 23];39:136-8. Available from: http://www.ijstd.org/text.asp?2018/39/2/136/247087



   Introduction Top


Angiokeratomas, benign tumors characterized by hyperplasia and hyperkeratosis of the epidermis along with multiple dilated vessels in the superficial dermis, are clinically classified into disseminated (angiokeratoma corporis diffusum) and localized forms; the latter includes angiokeratoma of the scrotum, penis, and vulva (angiokeratoma of Fordyce), angiokeratoma of Mibelli, and circumscribed angiokeratoma.[1] While angiokeratoma of the scrotum is common, similar lesions over the vulva are relatively rare. These lesions, despite being benign and asymptomatic, cause significant anxiety and lead to poor quality of life due to their similarity with other benign or malignant lesions such as verruca vulgaris and pyogenic granulomas.[2],[3] Here, we report the development of multiple vulvar angiokeratomas in a 40-year-old female after undergoing a perineoplasty.


   Case Report Top


A 40-year-old female presented with multiple, raised, itchy, reddish lesions on the genitals since 14 years. After getting married, 16 years ago, the patient experienced severe dyspareunia due to a narrow introitus which led to nonconsummation of the marriage for 1½ years. The patient underwent perineoplasty followed by dilatation of the introitus three times within 2 years, which resulted in a successful pregnancy after 1 year. However, angiokeratomas started appearing during the 4th month of pregnancy and kept increasing in number and size for the next 4 years. The patient also noticed cyclic increase in the size of the lesions during each menstrual cycle.

Dermatological examination revealed multiple, well-defined, erythematous, hyperkeratotic papules on bilateral labia majora [Figure 1]. On histopathology, multiple dilated vascular lumina lined by a single layer of endothelial cells containing red blood cells and fibro-collagenous tissue were seen in the superficial dermis. The overlying epidermis was hyperkeratotic with irregular hyperplasia of the rete ridges engulfing the vascular spaces [Figure 2]. Spindle-shaped cells or solid collections of endothelial cells or granulomas were not evident. The clinical and histopathological features corroborated with the diagnosis of angiokeratoma. The angiokeratomas were removed by electrocautery and follow-up 6 months after removal revealed no recurrences.
Figure 1: Multiple angiokeratomas on labia majora

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Figure 2: Multiple dilated vascular lumina lined by a single layer of endothelial cells containing red blood cells: (a) (H and E, ×100) and (b) (H and E, ×400)

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   Discussion Top


Angiokeratomas of the vulva present as multiple, small (2–5 mm), unilateral, slowly evolving keratotic papules with color ranging from red-to-brown. Initially asymptomatic, the lesions later become itchy, painful, or cause bleeding and dyspareunia, developing usually between the age of 20 and 40 years.[2],[3] Increased local venous pressure and consequent papillary dermal capillary ectasia are thought to cause the disease. The vessels of the labia, surrounded by elastic tissue, smooth muscle, and venous structures are vulnerable to inflammation and obstruction, leading to an increase in the local venous pressure.[2],[3],[4] Loss of support of local vessels secondary to congenital deficiency of elastic tissue, venous malformations, varicocele, or thrombosis secondary to chronic inflammation is other associated factors. The similar embryological origin of labium majus and scrotum from the labioscrotal folds is attributed for the occurrence of lesions on the vulva as on the scrotum.[2],[3],[4] Pregnancy, increased parity, obesity, hemorrhoids, pelvic inflammatory disease, and prior hysterectomy are considered predisposing factors. Vulvar angiokeratomas have also been described following chronic infection with human papillomavirus.[4],[5] Our patient too developed the lesions during her pregnancy after undergoing a perineoplasty.

Clinically, angiokeratoma of Fordyce over the vulva should be differentiated from angiomas (cherry angiomas, pyogenic granuloma, and lymphangioma), verrucous lesions (seborrhoeic keratosis, condyloma acuminata, nodular prurigo, condyloma lata, and certain nevi), and malignancies (melanoma and vulvar intraepithelial neoplasia).[4],[5] Histopathological diagnosis is confirmatory and reveals hyperkeratosis, papillomatosis, and capillary ectasia of the papillary dermis, without significant endothelial proliferation.[1],[2] Multiple treatment options such as electrocautery, radiofrequency, cryotherapy, and ablative lasers such as CO2 and erbium-doped yttrium aluminium garnet exist.[4],[5]

Angiokeratoma of the vulva, a benign condition, appears to be much more common than the literature suggests and should be differentiated histopathologically from malignant conditions and sexually transmitted viral infections. The patient should be counseled about the innocuous nature of these lesions and treated after investigating for any underlying predisposing diseases.

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.

Acknowledgment

We hereby thank Dr. Pradeep Mahajan for his valuable inputs in histopathology.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Calonje E. Vascular tumors: Tumors and tumor-like conditions of blood vessels and lymphatics. In: Elder DE, Elenitsas R, Johnson BL Jr., Murphy GF, Xiaowei X, editors. Lever's Histopathology of the Skin. 10th ed. Philadelphia, (PA): Wolter Kluwer, Lippincott Williams & Wilkins; 2009. p. 1007-56.  Back to cited text no. 1
    
2.
Blair C. Angiokeratoma of the vulva. Br J Dermatol 1970;83:409-11.  Back to cited text no. 2
    
3.
Cohen PR, Young AW Jr., Tovell HM. Angiokeratoma of the vulva: Diagnosis and review of the literature. Obstet Gynecol Surv 1989;44:339-46.  Back to cited text no. 3
    
4.
Fogagnolo L, Cintra ML, Velho PE. Angiokeratoma of the vulva. An Bras Dermatol 2011;86:333-5.  Back to cited text no. 4
    
5.
Kudur MH, Hulmani M. Giant angiokeratoma of fordyce over the vulva in a middle-aged woman: Case report and review of literature. Indian J Dermatol 2013;58:242.  Back to cited text no. 5
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