Plasma Cell Vulvitis: A Case Report and Review of Literature

Abstract

Plasma cell vulvitis (PCV) is a rare disease affecting anogenital skin. It is also known by multiple names including Zoon's vulvitis and vulvitis circumscripta plasmacellularis. It is an inflammatory condition that can cause severe local discomfort and psychosocial distress. The diagnosis is challenging and often remains unrecognized. It usually presents as a glistening, red, well-demarcated plaque or patch. The pathophysiology is unknown. Treatment recommendation is mostly based on case reports and case series and this condition is often recalcitrant to topical and systemic treatment.

In a Pinch: An Unusual Case of Zoon’s Vaginitis, a Plasma Cell Disorder

“…Regarding recommended therapy, experts have varying opinions and success rates. Biopsy is emphasized as a necessity for diagnosis, with the most common treatment using varying regimens of topical corticosteroids (TCS) and tacrolimus [ 12 - 14 , 17 ].…”

P16-Positive Vulvovaginitis: Answer

“…Plasma cell vulvitis (PCV) is a rare, inflammatory disorder of the vulva, characterized by pruritic or asymptomatic, bright-red, glistening, and flat mucosal plaques, with a "lacquer paint" appearance. 1,2 Small petechial hemorrhages may be visible, 1 and there are often no signs of discharge or crusting. 1,3 PCV can also be desquamative and hemorrhagic, as presented in this case.…”

“…18 DVIN is also commonly associated with lichen sclerosus (LS). 2 Another common disease of the vulva that may be confused with PCV is lichen sclerosus (LS). 2 Clinically, LS presents as clearly demarcated, hypopigmented to erythematous plaques that most often begin around the preclitoral hood.…”

“…2 Another common disease of the vulva that may be confused with PCV is lichen sclerosus (LS). 2 Clinically, LS presents as clearly demarcated, hypopigmented to erythematous plaques that most often begin around the preclitoral hood. 19 Telangiectasias, fissures, or ulcerations may be present.…”

“…Despite PCV's unique clinical features, it is often misdiagnosed. Potential explanations include histologic samples missing some classic PCV elements, the tendency of PCV to coexist with lichen sclerosis and other diseases, 2 and PCV's rarity and availability bias. 1 In this case, overexpression of p16 may have also contributed to the initial misdiagnosis.…”