Vulva Lichen planus

Lichen planus (LP)

Lichen planus is a distinctive inflammatory eruption of the skin and mucous membranes. Vaginal lichen planus is part of a wide spectrum of disease involving the skin, anal and oral mucosa, scalp, nails, eyes, esophagus, bladder, nose, and larynx.

Pathophysiology: Unknown, genetically predisposed immunologic disease possibly induced by exogenous irritants and antigens, resulting in targeting of the epidermis with immune destruction.

Symptoms vary. In the reticular (lacy) pattern there may be mild to severe pruritus. The erosive and ulcerative patterns have variable pain and burning that can be severe and intractable. With extensive erosions of the vagina there is copious malodorous discharge, dyspareunia, and apareunia. These patients eventually become very depressed or frankly hostile and angry. Their interpersonal relationships at home and their dealings with physicians can be very strained.

Note that any irritation can flare lichen planus - scratching, caustic chemicals, irritants, friction with intercourse, etc.

On physical exam in the classic papular form there may be small purple papules with a lacy or reticulated surface on the vulva and in the mouth. The lacy reticulated pattern may spread to the vaginal vestibule or out onto the labia minora. With erosive disease there may be a glazed erythema with exquisite tenderness to touch or frank almost ulcerated erosions, often with a lacy or slightly grayish edge. There are variations in the loss of architecture. Up to 70% of women with cutaneous LP have vaginal involvement. The vagina shows erosions, ulcers, synechiae, stenosis, shortening and rarely loss of the vagina.

The diagnosis is made looking at the vulvar area, but it is essential to look at the rest of the skin. Note the clinical pattern in the mouth, nails, scalp, and other body skin. Biopsy for both regular histopathology and immunofluorescence to differentiate from other white or bullous diseases. NB: Stop any topical steroid for 1-2 weeks before biopsy

Differential Diagnosis includes: Lichen sclerosus, Drug eruption, Cicatricial pemphigoid, Lichen simplex, Graft versus host disease.

Treatment is challenging, as no single agent is universally effective. Stop irritation and stop trauma and treat infection. Restore the barrier function with Sitz bath or tub bath 1-2 times a day. Reduce inflammation with topical superpotent corticosteroids, halobetasol or clobetasol 0.05% ointment 1-2 times a day. For the vagina, hydrocortisone acetate 25 ?100 mg suppository nightly. If very severe consider oral prednisone. For localized disease consider intralesional triamcinolone from 3.3 mg up to 10 mg per mL. Tacrolimus 0.03% and 0.l% ointment can be used topically but can sting and must be used only on areas already under control with topical steroids. Tacrolimus is used as a steroid sparer.

Controlling severe lichen planus requires aggressive management. Prednisone 1-1.5 mg per kg per day for 2 weeks, tapering to zero over 2-4 months or IM triamcinolone 1 mg per kg q3-4 w for three injections or add cyclosporine 4 mg per kg per day and continue until the patient is clear and wean them onto Plaquenil 200 mg bid and / or hydrocortisone acetate vaginally.

Other drugs to consider include doxycycline, etronidazole, acitretin, methotrexate and azathioprine. Prognosis: 38% complete resolution, 30% significant resolution, 32% ongoing problems.