• Chronic lymphocyte mediated skin disease, first described in 1887 (J Urol 2007;178:2268)
• Persistent/progressive inflammatory dermatosis of unknown etiology with predilection for vulva; may be perianal
• Usually age 40 years+ (more common after menopause)
• Rare in children, may involute spontaneously at puberty
• May be familial; associated with autoimmune diseases
• Very itchy and scratchy skin
• Not a premalignant lesion, but associated with well differentiated squamous cell carcinoma; in one study, 9% developed VIN, 21% developed invasive squamous cell carcinoma mean 4 years later (Hum Pathol 1998;29:932)

• Recognition and treatment of early stages may cause longstanding remission
• Topical steroids (first line) followed by oral or topical retinoids (second line)
• Lifetime surveillance because of increased risk of squamous cell carcinoma (Drugs Aging 2009;26:803)
• Biopsy recommended to rule out squamous cell carcinoma

"Cigarette paper" appearance

Clitoris becomes buried under clitoral hood

• Atrophic skin resembles cigarette paper (crinkly atrophy); often with loss of labia
• Also white / red ill-defined patches
• No vaginal involvement

• Severe hyperkeratosis; thin epidermis, loss of rete pegs, basal cell degeneration, homogenized band of dense fibrosis at papillary dermis, upper dermal edema, chronic inflammation around vessels (particularly eosinophils), often band like
• In early stages, findings are subtle and often more prominent in adnexal structures than in interfollicular skin; adnexal structures show acanthosis, luminal hyperkeratosis and hypergranulosis with/without dystrophic hair and basement membrane thickening
• Early dermal changes are homogenized collagen and wide ectatic capillaries in dermal papillae immediately beneath basement membrane
• Lymphocytic infiltrate can be sparse or dense, lichenoid or interstitial with epidermal lymphocyte exocytosis and lymphocytic / lymphohistiocytic vasculitis
• Proposed minimal histologic criteria: vacuolar interface reaction pattern in conjunction with dermal sclerosis (homogenized and hyalinized eosinophilic collagen bundles) of any thickness intervening between inflammatory infiltrate and epithelium or vessel walls (Mod Pathol 1998;11:844)

Various images

• Allergic contact dermatitis

• Am Fam Physician 2008;77:321, Dermatol Clin 2010;28:707

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