In this review, we focus on rare benign and malignant diseases. It is
shown that benign vulvar conditions require thorough examination of ot
her parts of the body, because a relationship with systemic diseases,
like cicatricial pemphigoid, pemphigus vulgaris, toxic epidermal necro
lysis, infections with parasites and Behcet's syndrome, may be present
. Co-operation with medical specialists is warranted. One in every 10
malignancies of the vulva is not a squamous cell carcinoma. Apart from
melanoma, several other tumors can occur on the vulva. A correct hist
ological diagnosis is of paramount importance because treatment strate
gies depend on it.