PMC6290115_01
EMPD before irradiation. The patient presented with an exceptionally large plaque measuring 27 x 16 cm with a 12-cm region of central maceration and surrounding hyperpigmentation. The lesion originated in the vulva and extended superiorly into the abdomen and laterally onto the bilateral thighs. The lesion did not cross over onto the poster perineum.
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In other areas, there was hyperpigmentation (Fig 1).
Carcinoembryonic antigen staining of EMPD before irradiation. Carcinoembryonic antigen marks positive in all the epidermal pagetoid cells. Pagetoid cells in the stratum corneum are also evident.
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The pagetoid cells expressed cytokeratin 7, carcinoembryonic antigen, and epithelial membrane antigen but did not mark with Mart1 (Fig 2).
Electron beam plan. External electron beam radiation plan for extensive EMPD shows isodose lines of superficially deposited radiation. Ninety-five percent of the prescribed total dose of 6000 cGy in 200 cGy fractions was delivered to the area bounded by the magenta lines.
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The patient received 6000 cGy in 30 fractions, delivered as a single en face electron cone field to the lower abdomen and perineum, using 6 MeV electrons and a 1/2-cm bolus (Fig 3).
Resolution of EMPD after irradiation. After electron beam irradiation, the skin over the treatment area is mildly hyperpigmented, areas of desquamation and bleeding have resolved, and the skin is re-epithelized and smooth without ulcerations.
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After 12 months of follow-up, the patient had an excellent response to radiation therapy (Fig 4).