Lichen Planus
by Dr. Hope Haefner
Clinical Appearance | Diagnosis | Treatments
Vaginal Lichen Planus (LP)
LP of the Gums
Erosive LP
Lichen planus is a dermatologic condition involving the glabrous skin, hair-bearing skin and scalp, nails, and mucous membranes of the oral cavity, and vulva. Hair loss as well as a history of papular lesions on skin surface (ankle, dorsal surface of the hands, and flexor surfaces of the wrists and forearms) may be found. A symptom such as pruritus may be present. The age range is approximately 30-60 years of age.
Lichen planus is a disease of unknown etiology. It most likely is an autoimmune disorder of cellular immunity. Additionally, the histologic features of lichen planus closely resemble those of another condition of immune origin, graft versus host disease.
Clinical Appearance
Appearance ranges from white, reticulate papules/plaques, to uniform, white epithelium, to non-specific erosions (usually with surrounding white epithelium). Vaginal erythema is present which often results in bleeding. Late scarring with loss of labia minora and clitoral hood occurs. Erosions of the buccal mucosa, gingivae, and/or tongue are usually present. Examination of the mouth may reveal a reticulated gray, lacy pattern of Wickham's striae. The mouth lesions may precede or follow by months or years the vulvovaginal lesions. The gingival tissue may be diffusely swollen with interdental accentuation or actually eroded with red, scalloped borders at the gingival margins of the teeth. Keratinized skin involvement is present only rarely in erosive disease. In the absence of sexual activity or regular vaginal dilation, adhesions begin to form in the upper part of the vagina. In long-standing cases, the vagina may be totally obliterated. Contact bleeding and dyspareunia are often noted.
Diagnosis
The diagnosis of erosive lichen planus is often made by finding characteristic skin lesions in mouth or skin biopsy. Within hair-bearing and keratinized epithelium, histopathology reveals a chronic inflammatory cell infiltrate, which consists predominantly of lymphocytes without plasma cells. The inflammation is lichenoid in that it involves the superficial dermis immediately beneath the epithelium, and extends into the basalar and parabasalar epithelium. Liquefaction necrosis is present within the basal epithelial cells; colloid bodies are present secondary to degeneration of keratinocytes. The interface at epidermal-dermal junction is obscured by the inflammatory infiltrate. The epithelium may have prominent acanthosis with a prominent granular layer and hyperkeratosis. In older lesions, acanthosis may be absent and the epithelium thinned with loss of the rete ridges.
When lichen planus involves the nonkeratinized epithelium of the vestibule, the interface inflammatory infiltrate is present, but the inflammatory cell population may contain plasma cells within the preeminently lymphocytic infiltrate. Hyperkeratosis and a prominent granular layer usually are not present. Bullae and ulceration are evident in severe cases. In the presence of erosive disease, a biopsy may be totally devoid of epithelium.
At times, immunofluorescent studies may be required to rule out other conditions in the differential diagnosis such as pemphigus or pemphigoid.
Direct immunofluorescence testing is superior for diagnosing pemphigus and pemphigoid. Immunofluorescence is slightly inferior to histologic evaluation for diagnosing lichen planus. Optimal criteria are IgG and C3 intercellular substance staining for pemphigus, linear C3 basement membrane zone deposits for pemphigoid, and shaggy fibrinogen basement membrane zone staining plus IgM cytoids for lichen planus.
Treatments
STEROIDS
Lichen planus is a difficult condition to treat. Steroids are frequently used as a first line of therapy.
Topical steroids–A high potency topical steroid ointment is used to treat lichen planus.
Potency Ranking of Some Commonly Used Topical Corticosteroids
Group I is the superpotent category; potency descends with each group, to group VII, which is least potent (II, III, potent steroids; IV, V, midstrength steroids; VI, VII, mild steroids). There is no significant difference between agents within groups II through VII; the compounds are simply arranged alphabetically. However, within group I, clobetasol ointment is the most potent topical steroid.
Commonly Used Topical Corticosteroids Potency Ranking
Intravaginal steroids have been used for lichen planus as a first line of treatment if vaginal involvement is present.
What are the various treatments for Lichen Planus?
Papular lichen planus tends to respond to topical corticosteroids. Triamcinolone acetonide 0.1% ointment is used for mild disease and clobetasol propionate 0.05% ointment is used for severe disease.
For erosive disease the following table contains many medications that have been tried for lichen planus treatment.
It is important to note that many of these medications are formulated for off–label use.
|
Agent
|
Discussion
|
|
Anti-inflammatory antibiotics are used long term
|
This treatment works best for early erosive lichen planus
Doxycycline or clindamycin used long-term. Consider adding weekly fluconazole to prevent yeast infection.
|
|
Steroids are often used for lichen planus
|
Vaginal LP
Anusol HC vaginal suppositories are used in the following manner:
1/2 of a Anusol HC suppository per vagina twice daily for 2 months, then daily for 2 months, then maintenance treatment at 1 to 3 times per week. However, many patients do not experience significant long-term response to intravaginal steroids. The vaginal vault tends to continue to scar. To keep the vault open and prevent adhesions, it often will be necessary to use vaginal dilators. The dilator may be lubricated with a hydrocortisone cream.
At times a stronger steroid may be required for vulvar LP.
Topical- Clobetasol propionate (Temovate®) 0.05% ointment.
Intralesional- triamcinolone acetonide 5-10 mg/ml.
Oral- Oral prednisone may be required until healing has occurred. As the skin heals, topical corticosteroids may be added as the prednisone is tapered.
IM steroids (place in thigh or buttock). Used for moderate or severe disease. Dose 1 mg/kg (up to 80 mg total) can be used. If repeat is necessary, it can be repeated monthy x 3 total doses.
For Oral LP- Apply Clobetasol propionate (Temovate®) gel 0.05% to affected area up to qid
Apply on a cotton ball in mouth for 5 min.
Some providers use dental molds to hold in medications in patients with gingival LP
|
|
Tacrolimus and
Pimecrolimus
|
Tacrolimus (Protopic) 0.1% ointment bid to qid.
Apply on a cotton ball in mouth for 5 min
Vaginal medication (made by compounding pharmacy)
tacrolimus vaginal suppositories
Insert one suppository per vagina (2 mg tacrolimus per 2 gram supp) qhs
Disp 50
Or - 0.1% vaginal cream (compounded in a vaginal cream / Replens like base) 2-5 gms =
2 - 5 mg/dose for 2 weeks then Mon-Wed-Fri for 2 weeks and slowly decrease Disp 100 grams
Vulvar medication Apply to skin bid Tacrolimus 0.1% ointment Available in 30 or 60 gram tubes
Calcineurin inhibitors (steroid sparing)
pimecrolimus (Elidel) 1% cream bid for mild LP
topical tacrolimus (Protopic) 0.03%, 0.1% oint
Note – can burn especially on raw areas
Long term safety unknown
|
Less Frequently Used Medications
|
|
Hydroxychloroquine (Plaquenil)
|
Occasionally used. Dose is 200 mg po bid.
|
|
Retinoids
|
Accutane (isotretinoin) or Etretinate (Tegison) have been used to treat oral lichen planus; however, discontinuation of the medication results in recurrence of the oral lesions. Long-term use of retinoids may result in liver dysfunction and there is no documented successful use of retinoids for vulvovaginal lichen planus. Liver function tests, cholesterol, triglycerides and complete blood cell counts should be monitored since laboratory changes are associated with the use of oral isotretinoin. Patients should be counseled concerning teratogenicity and need for optimal contraception. Topical retinoids (Retin A) are generally too irritating for this vulvar condition.
|
|
Cyclosporine
|
Used topically and systemically. Topical cyclosporine provides a safe and often effective but very expensive alternative for mucous membrane disease. Pelisse et al. described the use of the oral or injectable form of the medication in 100 mg amounts directly to the affected skin four times a day initially. If several mucous membranes were affected for example, 100 mg was applied to the vulva, 100 mg inserted into the vagina, and 100 mg held in the mouth for as long as tolerated before spitting. As disease is controlled, the frequency of application can be tapered. Systemically it is dosed at 4-5 mg/kg/day for 3 months (used in severe disease). Occasionally, in patients with debilitating and painful disease not adequately treated by therapies discussed above, oral cyclosporine may be used. This medication should be used only by health care providers experienced in its use.
|
|
Cyclophosphamide
|
Systemic antimetabolite
|
|
Azathioprine
|
Systemic antimetabolite
|
|
Etanercept (Enbrel)
|
This is used SQ (50 mg sq 2x/week until symptoms improve, then 25 mg sq 2x/week)
|
|
Mycophenolate mofetil (CellCept)
|
Oral use 250mg -1g bid
|
|
Methotrexate
|
Oral or subcutaneous injection weekly. Methotrexate 5-10mg/week PO or SC with folate 1 mg/d
|
| Misoprostol |
This agent has been used on oral as well as vulvovaginal lesions. Do not use this medication in pregnancy.
This agent is not used as commonly as the other agents listed.
|
Table developed in conjunction with Lynne Margesson, MD
With any of the treatments described, the lichen planus tends to recur after the treatment is discontinued.
MISCELLANEOUS
Surgery
At times the vagina is scarred and surgery is required. It is best this is performed after active disease is controlled, otherwise scarring rapidly reoccurs. Following surgery, patients must use vaginal dilators daily to maintain adequate opening of the introitus and vagina. Close follow–up is required at all times.
Prognosis
At times this is a disease recalcitrant to corticosteroids and very difficult to control with other medications. There is little or no tendency for remission, and there is a small risk for the development of a squamous cell carcinoma in chronic lesions.
Follow-up
Vulvar lesions are often chronic and may undergo malignant change. Long-term follow-up of patients with lichen planus is necessary. Patient education and support are important at all times when treating people diagnosed with lichen planus.