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In 1889 Crocker published the first report of Extra mammary Paget's disease (EMPD). EMPD is characterized by a progressive erythematous marginated plaque resembling Paget's disease

 Clinically and histologically but occurring in anogenital region, axilla or most commonly the vulva. It is more common in women than in men and starts usually in the fifth decade or later.

 The commonest area is the vulva followed by the perianal area, the scrotum, penis and axilla. Other sites include the eyelids and the external ear canal. Clinically axillary lesions start with slight erythema and progress into sharply marginated erythematous plaques.

 Pruritus is commonly present.Paget's cells are distinctive large cells (10 to 20mm) with abundant pale vacuolated cytoplasm and a prominent nucleus. 

Positive staining for carcinoembryonic antigen has established the glandular origin while specific immunoreactants like antigross cystic fluid protein antibody and apocrine epithelial antibodies have helped to prove their relationship to apocrine and not eccrine sweat glands.

 Differential diagnosis include eczema, intertrigo and Bowen's disease and basal cell carcinoma. A skin biopsy with histochemistry studies is usually conclusive.

 The most appropriate treatment is surgical excision while radiotherapy has also been used effectively. However with high local recurrence rate regular follow up of patients with EMPD is a must.