Vulvitis circumscripta plasmacellularis. A clinicopathologic entity?

Four cases of vulvitis circumscripta plasmacellularis (plasma cell vulvitis) are presented. One case was associated with cutaneous lupus erythematosus and another with a history of desquamative vaginitis. Two patients were postmenopausal, and two were premenopausal. The presenting symptoms were pruritus, tenderness, superficial dyspareunia and vulvar dysuria. The lesions were situated in the introitus in three patients and on the lateral aspect of the labium minus in the fourth and appeared as well-circumscribed, glistening, erythematous patches with a faint orange hue. Histologically, epidermal edema and inflammation, a dense upper dermal band of chronic inflammatory cells, including many plasma cells, dilated capillaries, extravasated red blood cells and hemosiderin deposition, were seen. There was a variable response to local steroid therapy, but one of the postmenopausal patients responded to local estrogen alone. The term vulvitis circumscripta plasmacellularis is useful to describe an idiopathic form of erosive vulvitis with a characteristic clinical and histologic appearance.