Giant angiokeratoma of Fordyce over the vulva in a middle-aged woman: case report and review of literature.

Angiokeratoma of Fordyce occurring over vulva is rare. Angiokeratoma of Fordyce commonly occurs in males over scrotum or penile shaft and presents as multiple verrucous reddish papules. They are usually asymptomatic and noticed accidentally. In the present article, we present and review the literature of giant angiokeratoma of Fordyce in middle-aged women due to its rarity.

What was known?

Angiokeratoma of Fordyce is commonly seen in males over scrotum.

Introduction

The angiokeratomas are benign vascular proliferations with hyperkeratosis at the surface.[1] The clinical variants of angiokeratoma can be divided into widespread form and localized forms. Angiokeratoma corporis diffusum (Fabry disease) is a widespread form associated with deficiency of Galactosidase A. The localized form includes solitary angiokeratoma, angiokeratoma circumscriptum neviforme, angiokeratoma of Mibelli and angiokeratoma of Fordyce.[2] Angiokeratomas are a group of telangiectasias with secondary epidermal hyperkeratosis. Angiokeratoma of Fordyce is the commonest type of angiokeratoma seen, common over the scrotum, can also be seen on the vulva and thighs. Angiokeratoma of the vulva (Fordyce) is common in older women and usually asymptomatic. We present a case of giant and multiple angiokeratoma of the vulva in a middle-aged woman because of its rarity and uncommon presentation.

Case Report

A 45-year-old female presented with multiple asymptomatic soft papules and nodules over the vulva of two years’ duration. The lesions started spontaneously on the right side of the vulva as small papules and gradually grew to attain the present size. Lesions were asymptomatic. There was history of occasional bleeding on touch from the lesions. The patient was married 20 years back and there was no history of obstetric complications during delivery. There was no history of trauma or surgery over the vulva. On examination multiple 0.5 to 1 cm bluish papules and nodules were seen on either side of the vulva [Figure 1]. On palpation, they were soft to firm, non-tender, pedunculated tumors. Per vaginal and colposcopic examination of the vagina was normal. Examination of the perianal area was normal. The tumors were excised by electrocautery under local anesthesia under aseptic precautions and sent for histopathological examination. Histopathology revealed hyperkeratosis, irregular acanthosis and papillomatosis of the epidermis and multiple thin-walled vessels containing red blood corpuscles (RBCs) in the papillary dermis, confirming the diagnosis of angiokeratoma of Fordyce [Figures 2 and 3].

Figure 1

Angiokeratoma of Fordyce – multiple, reddish blue soft papules and nodules are seen on either side of the vulva

Figure 2

Angiokeratoma of Fordyce (H and E, ×10 view) – epidermis is showing hyperkeratosis, irregular acanthosis and papillomatosis and multiple thin-walled vessels containing RBCs are seen in the papillary dermis

Figure 3

Angiokeratoma of Fordyce (H and E, ×40 view) – multiple vessels with RBCs are seen in the dermis

Discussion

Angiokeratoma of Fordyce was first described by John Addison Fordyce in 1896.[3] Angiokeratoma of Fordyce is the commonest type of all angiokeratomas. Exact data of incidence of angiokeratoma of Fordyce is lacking. The pathogenesis of angiokeratoma of Fordyce is unknown; the speculated pathogenesis is localized venous hypertension. [4] There are reports of angiokeratoma of Fordyce seen in association with varicocele or conditions with increased venous pressure (e.g., hernias, epididymal tumors, trauma, thrombophlebitis, tumors of urinary system).[35]

Angiokeratoma of Fordyce presents as asymptomatic, 2 to 5 mm, blue to red papules with scaly surface over the scrotum (commonest presentation), shaft of penis, labia majora, inner thighs, or lower abdomen.[5] They may bleed spontaneously, after scratching, on trauma or following sexual intercourse. Early lesions of angiokeratoma are soft, red and easily compressible; later, they become blue, keratotic and non-compressible.[6] Solitary type of presentation is more common.

In a study by Imperial et al.,[7] on vulval angiokeratomas, 54% patients had predisposing factors for the development of angiokeratomas such as pregnancy, vulval varicosity and hysterectomy while the rest had none. There are many reports of penile and vulval angiokeratomas following radiation treatment for genitourinary malignancy.[8] Vulval angiokeratomas have been described following chronic infection with human papilloma virus.[9]

The differential diagnoses of angiokeratoma on the vulva (Fordyce) are angiokeratoma corporis diffusum (Fabry disease), condyloma accuminata, cherry hemangioma, and granuloma pyogenicum. Patients of Fabry disease present with acute lancinating limb pains and angiokeratoma all over the body.[10]

Treatment of angiokeratomas depends on the site and size of the lesion and also availability of the surgical equipment like electrocautery, radiofrequency, cryotherapy or ablative Lasers. It can be removed by simple excision with closure if lesions are few. Cryotherapy, electrocautery and radiofrequency can be done for multiple lesions. Er: YAG[11] or CO2 Laser are effective in clearing angiokeratomas with less scarring.

What is new?

Angiokeratoma of Fordyce can also be seen is seen in females over vulva and can present in giant form.