Khalifa E. Sharquie, Wesal K. Al-Janabi.
Background: Paget’s disease is a rare cancer whether mammary or extra-mammary but must be considered in any persistent eczematous eruption of nipple &/ areola or elsewhere like genitalia which does not responds to topical treatment. Although it is slowly growing tumor but diagnosis and therapy should be carried out before deep invasiveness might occur.
Objective: To report cases of Paget’s disease in order to pay attention to the increasing frequency of this rare disease among population especially among females.
Patients and Methods: This study was conducted at the Dermatology Center, Medical City, Baghdad, Iraq, during the period from May 2007 to Oct. 2019. All the cases of biopsy proven Paget’s disease were evaluated for the demographic data like age, gender, family history. In addition, clinical features such as site, underlying palpable breast mass, regional lymphadenopathy, examination of contralateral breast and any associated systemic symptoms. Relevant work up to find any associated underlying malignancy was done. A number of patients who refused surgery were treated by topical podophyllin 25% solution at weekly sessions for minimum six weeks with follow up period of one year.
Results: Twenty-One patients were included in this study, 19 females and 2 males (9.5:1). The mean age was 40.2 ± 5.3 (range 28-53) years. The duration of lesions ranged between 2 months -1 year. Sixteen (76.19%) cases were mammary Paget's disease (MPD), while 5(23.8%) cases were extramammary (EMPD). The frequency of MPD in relation to EMPD was 3.2:1. Regarding MPD, the right breast was involved in 12 cases, while only 2 cases affecting the left breast. Two cases (12.5%) had bilateral MPD (1 nipples only and the other nipples and areolas). Ten (62.5%) cases affected the nipple and areola together (one case bilateral), whereas in 6 (37.5%) cases the nipple (one case bilateral) was only diseased. The lesions of nipple and/areola presented as erythematous, scaly, crusted, some with oozing, erosions and even ulcerations. The nipple looked atrophied to nearly eroded in 3 (14.82) cases. Pigmented MPD was seen in 4 (25%) patients. For the five patients with EMPD, 2 female patients had natal cleft lesions (1 with erythematous patch and the other with hypopigmented patch) and one patient with left vulvar lesion. Perianal erythematous moist eroded plaque was seen in one male patient while another male patient had penile erythematous plaque. All the lesions of both MPD and EMPD were well defined. The associated symptoms were mainly itching, burning and discomfort No underlying palpable breast mass (MPD) or associated underlying internal Or malignancy (EMPD) and no regional lymphadenopathy nor distant metastasis were detected. Topical podophyllin was applied in 7 cases, six on breast and one vulvar who refused surgery and it had good palliative action preventing progressing their lesion.
Conclusion: We think that Paget's disease is increasing among Iraqi females when compared with previous years. Paget’s disease must be kept in mind when facing persistent eczematous involvement of the nipple and/ areola, or elsewhere not responding to a local treatment as urgent intervention is highly recommended. Topical therapy (like using topical podophyllin) is an option for isolated disease and as an adjuvant in patients who refused radical treatment.View pdf