Complete remission of two patients with recurrent and wide spread extramammary Paget disease obtained from 5-aminolevulinic acid-based photodynamic therapy and imiquimod combination treatment.

BACKGROUND: Extramammary Paget disease (EMPD) is an uncommon malignant neoplasm affecting apocrine gland-bearing skin which usually occurs in the anogenital area of patients older than 50 years. However, as EMPD in old men develops in penoscrotal area and sometimes asymptomatic, in situ or dermal invasion is usually widely spread before a confirmed diagnosis was made. Skin graft is needed after surgery. Both photodynamic therapy (PDT) and topical use of imiquimod reported effective for EMPD.

METHODS: Two patients with recurrent and wide spread at penoscrotal and groin area were diagnosed EMPD by biopsy and histopathological and immunohistochemical examination. We use 6 cycles of 20% 5-aminolevulinic acid (ALA) photodynamic therapy guided by 5-ALA induced porphyrin fluorescence. After PDT, patients were treated by topical use of imiquimod to prevent the recurrence.

RESULTS: After 6 cycles of PDT and imiquimod treatments, the patients' examination showed total remission. At the end of the 6 and 12 months, biopsy and pathology examination remain no signal of recurrence. No clinical recurrence after 24 and 36 months' follow up.

CONCLUSION: The combination treatment with 20% PDT and imiquimod acquired complete remission in treatment for recurrent and wide spread extramammary Paget disease in two patients.