Laboratory Data

2 biopsies were taken from the vulva and the groin.

Sections of both skin biopsies show similar features. Hyperkeratotic, acanthotic epidermis within which are seen nests and individual vacuolated cells with pleomorphic nuclei. These cells are seen at all levels of the epidermis and stain positively for mucicarmine. The subepidermal zone contains a dense lymphocytic infiltrates.

Interpretation: skin biopsies of vulva and groin consistent with extramammary Paget's disease

Extramammary Paget's Disease

She was treated with oral itraconazole 100mg bd for 2 weeks which showed marked improvement of the symptoms and redness. However, at the end of 2 weeks, there were persistent erythema, diffuse swelling and superficial erosions on the left vulva. Biopsies of the skin were subsequently done which confirmed extramammary paget's disease.

She would require further evaluation for any underlying malignancies especially the gynecological and uro-genital system.

How would you manage her extramammary paget's disease?

Hatta N, Yamada M, Hirano T, Fujimoto A, Morita R. Extramammary Paget's disease: treatment, prognostic factors and outcome in 76 patients. Br J Dermatol. 2008 Feb;158(2):313-8.

Division of Dermatology, Toyama Prefectural Central Hospital, Nishinagae, Toyama 930-8550, Japan.

BACKGROUND: Extramammary Paget's disease (EMPD) is a rare cutaneous carcinoma usually presenting as a genital erythematous lesion in the elderly. Although most EMPD tumours are in situ, invasive EMPD has a poor prognosis. OBJECTIVE: To evaluate the clinical and pathological features of EMPD and determine prognostic factors for survival. METHODS: The medical records of 76 patients with EMPD were retrospectively reviewed. RESULTS: Of the 66 patients who underwent curative surgical excision, five (8%) developed local recurrence, but surgical margin (<or=2 cm or >2 cm) was not correlated with local recurrence. Thirteen of the 76 patients (17%) developed systemic metastases and 10 of these died of disease. On univariate analysis, the presence of nodules in the primary tumour, clinical lymph node swelling, elevated serum carcinoembryonic antigen (CEA) levels, tumour invasion level and lymph node metastasis were significant prognostic factors. On multivariate analysis, invasion level and elevated serum CEA were the only factors that were significantly associated with reduced survival. CONCLUSIONS: Invasion level and lymph node metastasis are important prognostic factors in EMPD. In patients with in situ tumour, local tumour control is the major aim of treatment; however, wide surgical margins are not associated with a lower risk of local recurrence.

Lloyd J, Evans DJ, Flanagan AM. Extension of extramammary Paget disease of the vulva to the cervix. J Clin Pathol. 1999 Jul;52(7):538-40.

Department of Histopathology, St Mary's Hospital NHS Trust, London, UK.

Extramammary Paget disease of the vulva was found in association with vulval adenocarcinoma in an elderly woman who also had a uterine prolapse. The characteristic histological appearances of extramammary Paget disease were masked by striking reactive changes in the squamous epithelium. Primary excision of both the intraepithelial and invasive disease appeared complete. However, a subsequent hysterectomy with repair of the prolapse revealed extramammary Paget disease in the upper vaginal mucosa and cervix, a finding which is very rarely described. Pathogenesis and diagnosis of extramammary Paget disease is discussed, with differential diagnosis and reference to immunohistochemical methods.

Khaled el-Hoshy MD, Troy, Michigan, USA on May 1, 2008

Special stains (minimum CEA) should be done. Mohs surgery, preceded by 2 weeks of 5-FU gives excellent cure rates. Malignancy in genito-urinary/lower GIT should be evaluated as suggested by presenting MD.

Joel Bamford MD, SMDC & UMD Medical School, Duluth, MN, USA on May 1, 2008

Excellent photos and current history. I presented a poster about a man with more extensive disease. All of his flat lesions resolved with Aldara 5 x daily over two months. This left him with several nodules which did not clear. He then developed allergic response to Aldara and stopped it. Subsequent radiation was of paliative help but not curative.

Firas Altamimi MD, Basra, Iraq on May 2, 2008

Nice case. EMPD has a high rate of metastases with very poor prognosis. Sentinal lymph node examination of this patient should be considerd to exclude any metastases. Surgical removal by Moh's surgery is treatment of choice. Imiquimod cream 5% apply daily can be used.

Abbas Naji Alshammari MD, Consultant Dermatologist, Doha, Qatar on May 2, 2008

Nice presentation and photos. To start with, treat the overlying candidal infection both orally and topically, preferably with mild to moderate topical steroid to calm down the irritation and inflammation . This makes easier to see the real extent of the disease. Then try to investigate the deeper origin and extent of the tumour. After that can adjust the proper way of treatment accordingly. Although Mohs surgery has its value it has certain indications and limitations. Update us with any new results or data please.

Hamish Dunwoodie MD, Dermatologist, Moncton, NB, Canada on May 3, 2008

I don't see any photomicrographs of the specimen from this patient. It is important to be 100% sure of dx since one will commit this patient to major intervention. It might be good to repeat the biopsy since the Candida has been cleared up.

David Elpern MD, Dermatologist, Williamstown, MA, USA on May 3, 2008

This is a great case for discussion. I suspect that if this woman has internal involvement with EMPD it will be difficult to eradicate. Hers is the kind of problem that is best handled at a tertiary centre (and you may not have that kind of service in Malaysia). Even S'pore may not have the depth since skin cancer is so uncommon in your area. I suppose I would repeat the biopsy and have it confirmed by Jag or Andy and then use Aldara but keep in mind that it will be difficult on the patient. I would also try to find an expert in USA, Europe or Australia to weigh in on this. Good luck and keep us posted.

Ian McColl MD, Dermatologist, Gold Coast, Australia on May 3, 2008

It all boils down to where the tumour is coming from, vagina, rectum, urethra etc. Little point in Mohs surgery for the outside tumour without dealing with the tumour of origin. That said you can get primary extramammary Pagets. In that case Mohs or PDT directed Mohs using aminolaevulinic acid and red light to help localise the tumour extent can be used but this will be a big defect. Radiotherapy can certainly be palliative but she needs scopes up all her local orifices. If the tumour is CK7pos and CK20 neg it may just be the primary type derived from local apocrine or eccrine stem cells and has a better prognosis after local resection.

I have used Imiquimod and PDT therapy with some success in both males and females but never achieved a cure. These therapies are better for local recurrence after surgery or radiotherapy.