extramammary paget 's disease ( empd ) is a rare cutaneous , intraepithelial adenocarcinoma involving primarily the epidermis but occasionally extending into the underlying dermis which accounts for < 1% of carcinomas in vulva and majority of the patients are postmenopausal females .
it presents with a long - standing history of pruritic , erythematous , scaly , or velvety patches .
the most frequent site is the vulva , but perineal , scrotal , perianal , and penile skin are also common areas .
the cancer cells in the neoplasm usually stay  in situ  and only rarely invade into the dermis to be metastatic through the lymphatic system .
a 55-year - old postmenopausal female presented with history of itchy and gradually progressive reddish lesion on genitals for 3 years .
cutaneous examination revealed a well - defined , moist erythematous plaque of size 15 cm  10 cm with multiple erosions involving bilateral labia majora and clitoris [ figure 1 ] . on full body examination , no other lesion or lymphadenopathy was observed . on investigations ,
a well - defined erythematous plaque the clinical diagnosis of empd was confirmed by a 3 mm punch biopsy which showed cells within the basal layer of epidermis having nuclear enlargement with atypia , prominent nucleoli , and well - defined ample cytoplasm .
in addition , a focus of squamous cell compression and moderate inflammatory cell infiltrate was seen in the upper dermis [ figure 2 ] .
our patient was advised to undergo excisional surgery , but she refused and was lost to follow - up .
in 1874 , sir james paget first described mammary paget 's disease and in 1901 , dubreiuhl reported the first case of vulvar empd .
vulvar paget 's is a rare disease and typically presents as a red , velvety , pruritic skin rash of the vulva region which closely mimics a multitude of other , more common conditions . as a result
paget 's disease can be found in both men and women ; however , it is most commonly seen in postmenopausal caucasian females .
typically , involved sites are the vulvar , perianal , scrotal and penile regions ; rare sites include the thighs , buttocks , axilla , eyelids and external ear canal .
the lesions can be white to red , scaling or macerated , can appear infiltrated , eroded or look like an ulcerated plaque .
due to disease 's relatively low incidence and since many cases are unreported , its true incidence remains unknown .
the clinical differential diagnosis for empd includes psoriasis , contact dermatitis , fungal infections , lichen sclerosus , intraepithelial neoplasia and melanoma .
the nonspecific clinical findings often lead to misdiagnosis and an average of 1 year can pass before a biopsy is taken and definitive diagnosis is made .
it is characterized microscopically by the presence of specific tumor cells called paget 's cells .
paget cells are large cells with pale clear cytoplasm , large round hyperchromatic nuclei which tend to form clusters or solid nests .
paget 's cells have intracellular mucopolysaccharides , with empd having a greater amount of mucin as compared to mpd . as a result ,
cells frequently show positive staining for periodic acid - schiff and diastase resistance , mucicarmine , alcian blue at ph 2.5 , and colloidal iron .
surgery remains the treatment of choice for empd with recurrence rate of up to 44% with wide local excision .
other modalities include mohs micrographic surgical excision , radiotherapy , topical agents including 5-fluorouracil , bleomycin , imiquimod and photodynamic therapy .
this case is being reported for its rarity and to stress on the fact that an early biopsy should be advised to avoid misdiagnosing empd .
 extramammary paget 's disease is a rare cutaneous , intraepithelial adenocarcinoma involving primarily the epidermis but occasionally extending into the underlying dermis . 
 the condition typically presents as a red , velvety , pruritic skin rash of the vulva region which closely mimics a multitude of other , more common conditions . as a result , 
 vulvar paget 's disease is frequently misdiagnosed , leading to an often lengthy lag time ( an average of about 23 years ) between the onset of symptoms and diagnosis . 
INTRODUCTION
CASE REPORT
DISCUSSION
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