1. Erdogan, Cagdas MD
  2. Koseoglu, Hasan Tankut MD
  3. Yuksel, Mahmut MD

Article Content

Extramammary Paget disease is a rare condition that affects apocrine gland-rich skin areas such as the axilla and anogenital region (Armitage, Jass, Richman, Thomson, & Phillips, 1989; Minicozzi, 2010). In this case, a patient with perianal Paget disease was treated in our clinic.


Patient Case

In 2009, a 59-year-old male patient presented with perianal irritation and a minor amount of rectal bleeding. Anal inspection revealed erythema and lichenified skin lesions in a 4 x 2-cm area at the 6 o'clock position. The rectal examination revealed no abnormalities. The abdomen examination revealed no abnormal findings. The inguinal examination revealed no lymphadenopathy. Except for Grade 1 internal hemorrhoids, there were no abnormal findings during colonoscopy.


The rectal biopsy result for the patient was normal. The abdominal computed tomographic (CT) scan revealed no abnormal findings. The patient's perianal skin biopsy, however, revealed atypical cells with large hyperchromatic nuclei, prominent nucleoli, and medium-wide cytoplasm, which are scattered one by one in the superficial epidermis, forming irregular groups in places, and are compatible with Paget disease. A wide local excision was performed to eliminate the discovered lesion.


In the patient's follow-up, there was no recurrence for a long period. Recurrence was found when he appeared again in June 2021 with the same complaint of itching (Figure 1). Then the patient underwent another full body examination, colonoscopy, and abdominal CT scan for malignancies, which were normal. After that, the patient was operated on once more. The patient has since been followed in remission.

Figure 1 - Click to enlarge in new windowFIGURE 1. Recurrence of perianal Paget disease.


Perianal Paget disease is an extremely unusual condition in which malignant Paget cells appear in the epidermis of the anorectal area. Perianal Paget disease accounts for 6.5% of all Paget disorders. In their investigation, Helwig and Graham (1963) evaluated 40 patients with anogenital Paget disease and discovered seven extracutaneous malignancies and four rectal adenocarcinomas. As a result, patients with anogenital Paget disease should undergo physical examination, colonoscopy, and imaging modalities to check for cancer. The degree of disease and whether or not lymph nodes are involved should be considered while developing a treatment plan. Treatment options include surgery, radiation therapy, and topical chemotherapeutic medicines.



Perianal Paget disease is a very rare condition. A thorough clinical assessment is critical with perianal Paget disease to rule out malignancy.




Armitage N. C., Jass J. R., Richman P. I., Thomson J. P., Phillips R. K. (1989). Paget's disease of the anus: A clinicopathological study. The British Journal of Surgery, 76(1), 60-63. doi:10.1002/bjs.1800760119 [Context Link]


Helwig E. B., Graham J. H. (1963). Anogenital (extramammary) Paget's disease. A clinicopathological study. Cancer, 16, 387-403. doi:10.1002/1097-0142(196303)16:3<=387::aid-cncr2820160314>;2-0 [Context Link]


Minicozzi A., Borzellino G., Momo R., Steccanella F., Pitoni F., de Manzoni G. (2010). Perianal Paget's disease: Presentation of six cases and literature review. International Journal of Colorectal Disease, 25(1), 1-7. doi:10.1007/s00384-009-0797-9 [Context Link]