Full Recovery of Recurrent Extramammary Paget’s Disease of the Vulva (REMPD-V) After Imiquimod Treatment (PDF)


Extramammary Paget’s disease is an uncommon cutaneous adenocarcinoma, characterized by glandular differentiation and an insidious course.The standard treatment, although possible loss of tissue function and disease recurrence are seen. The EMPD-V recurrence rates are high despite aggressive surgical intervention1,5.The diagnosis is made histopathologically, and management is based on wide surgical excision.The histopath- ological features are similar in mammary and extramammary Paget’s disease. The Paget’s cells are present singly or in small clusters and characteristically stain with hematoxylin and eo- sin6. Identi cation of new therapeutic strategies less mutilating/ aggressive than reexcision, x-ray therapy, or chemotherapy is warranted.

We report a case where complete clinical and histological resolution of non-invasive EMP-V of the vulvar was achieved with minimal adverse effects after six weeks of imiquimodap- plication6-9.Imiquimod is an immunomodulator that stimulates the production of a range of cytokines including IL-1, IL-6, IL-8, and IL-12, and especially interferon IFN-a and TNF1,3,10. Imiquimod is generally well tolerated, without any major side effects or tissue damage. Imiquimod may be a useful alternative or adjuvant in the treatment of EMPD. 


Hear we present a case one patient with rEMPD-V, who re- sponded to topical imiquimod therapy. A 72 year old female with a history of hypertension and diabetes underwent margins free vulvectomy due to EMPD-V (1995). She abandoned follow-up and returned 8 years later with a benign erythematous papule lesion near to the surgical scar. Upon reliving of symptoms with topic corticoid the patient did not show up for a programmed vulvoscopy.

The patient reappeared two years later with a greater lesion (Figure 1a). On examination, bilateral well-de ned hypo-pigmented leukoplakia-like lesion was observed on the vulvar and gluteus area. The biopsy specimen was positive for rEMPD-V and the dif- ferential diagnosis was con rmed by histopathology (HE – Figure 1b) and immunohistochemistry (Table 1). The immunohistochemis- try was positive for CK 7 (Figure 1c), EMA (Figure 1d) and CEA (Figure 1e).

Due to chronic underline diseases a surgery was not advised to the 72 years old patient. Therapeutic options were discussed with the patient and she agreed to undergo and patient consent form signed to treatment with imiquimod 5% cream for 6 weeks.

The imiquimod treatment was started on 10.08.2005. One week after, she attended the emergency room with headache and in u- enza-like symptoms. She received medical attention and also a gynecological evaluation (Figure 2). Due to pain, burning and ul- ceration at the target site she returned on 24.08.2005 (Figure 3) and the topical application was discontinued for two weeks. On the next visit, she presented signi cant improvement with only a mild hyperemia (Figure 4) and the medication was restarted. After the complete treatment schedule, the patient evaluation and biopsy were negative (Figures 5a-d). The follow-up con rmed successful disease approach (Figure 6). The patient has remained clinically free of EMPD-V for approximately 1 year.