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Paper text:
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 .
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 .
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 .
the patient was married 20 years back and there was no history of obstetric complications during delivery .
multiple 0.5 to 1 cm bluish papules and nodules were seen on either side of the vulva [ figure 1 ] .
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 ] .
angiokeratoma of fordyce  multiple , reddish blue soft papules and nodules are seen on either side of the vulva 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 angiokeratoma of fordyce ( h and e , 40 view )  multiple vessels with rbcs are seen in the dermis
the pathogenesis of angiokeratoma of fordyce is unknown ; the speculated pathogenesis is localized venous hypertension .   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 ) .
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 .
early lesions of angiokeratoma are soft , red and easily compressible ; later , they become blue , keratotic and non - compressible . solitary type of presentation is more common . in a study by imperial et al .
, 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 .
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 .
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 .
angiokeratoma of fordyce can also be seen is seen in females over vulva and can present in giant form .
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 .