Paget's disease of the vulva with underlying apocrine adenocarcinoma and local lymph node invasion.

A case of Paget's disease of the vulva with an underlying apocrine adenocarcinoma and local lymph node invasion is reported in a female aged 70. Subjective symptoms of pruritus, burning and pain were associated with typical, but unspecific clinical findings of an erythematous, indistinctly limited, weeping lesion of palm-size with small superficial erosions. Near the right labium majus a solid measuring 2.5 X 1.5 cm in diameter was palpable. The diagnosis was established by microscopic examination of biopsy material, showing intraepidermally the typical Paget's disease with a subjacent, highly differentiated apocrine adenocarcinoma of the sweat glands combined with inguinal lymph node invasion. Other epidermal malignancies were excluded by histochemical reactions. For therapy, telecobalt irradiation combined with high energy electron was applied.