eMedicine Specialties > Dermatology > Reactive & Inflammatory Dermatoses
Lichen Sclerosus et Atrophicus: Treatment & Medication
Updated: Jan 29, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
Asymptomatic extragenital lichen sclerosus usually requires no treatment as control of pruritus other than resolution of the lesion, which is a more realistic goal of therapy. Also, these lesions rarely itch.
Genital lichen sclerosus may respond to potent topical corticosteroids although the patient should be warned that the clinical appearance does not always reverse, even if symptoms are relieved. It is widely reported that prepubertal lichen sclerosus in girls may resolve spontaneously although some of these patients may go on to suffer from various types of vulvodynia in adulthood.
Periodically, a report will suggest that areas of vulvar lichen sclerosus be surgically excised or ablated with a laser as a prophylactic measure. Most authors dispute this concept and do not recommend mutilating gynecologic surgery for what, in most patients, is a benign disorder. It is true, however, that circumcision may resolve male genital lichen sclerosus, although the use of potent topical steroids may obviate the need for surgery in such cases.
The calcineurin inhibitors (ie, tacrolimus, pimecrolimus) have been found to help some patients, especially with genital lichen sclerosus, but they do not work as fast or as effectively as potent topical corticosteroids. They may have a role in maintenance therapy, although several case reports have described that they were not effective.
Surgical Care
Circumcision may benefit male lichen sclerosus and the phimosis that may accompany it. Vulvar surgery is not recommended unless an associated malignancy is present. Extragenital lesions may be excised but some caution should be taken as lichen sclerosus has arisen in old surgical scars.
A variety of destructive procedures have been reported to be of benefit, although follow-up studies often do not show the same efficacy as original pilot reports. Not just tissue-vaporizing carbon dioxide lasers, but also nonablative lasers such as the pulsed dye and Er:YAG lasers, have been reported to benefit persons with lichen sclerosus. Cryotherapy of affected genital lesions is also reported to reduce the area involved after one or a series of treatments.10 Narrow-band UVB,11 psoralen plus UVA (PUVA), and photodynamic therapy12 using a photosensitizer with laser light activation are also reported to be anecdotally beneficial by various authors.
Consultations
- Dermatologist
- Gynecologist - If dysplasia or malignancy are identified or suspected on biopsy. Ulcerated areas should be examined repeatedly and frequently.
- Urologist - If lichen sclerosus is complicated by symptomatic phimosis and circumcision is required
- Pediatrician and/or social services personnel - If coexistent child abuse is suspected
Diet
One study suggests patients with lichen sclerosus might have a higher rate of positive patch tests to spices and flavorings.13 Older literature suggests that diets that lower the acidity in the urine may benefit female genital lichen sclerosus. These latter studies were not reproducible, and no dietary recommendations currently are proposed.
Activity
Lichen sclerosus–associated dyspareunia or painful erections may limit sexual activity. No specific activity limits or exercises are recommended. An author in the 1930s suggested that tight underwear and bicycle seats were the cause of lichen sclerosus in girls, but neither of these has been validated as the cause of lichen sclerosus in later studies.
Medication
Topical testosterone was mainstay of treatment for female genital lichen sclerosus for decades, although it was never shown useful in male genital or extragenital cases. More recent studies suggest it may not be any more efficacious than placebo. Systemic therapies, including anti-Borrelia antibiotics, potassium benzoate, penicillamine, and systemic steroids, have not proven reliably effective. Systemic retinoids have been useful in limited studies with isotretinoin, etretinate, and acitretin. Topical corticosteroids, especially in the superpotent class, have been found to be useful in genital lichen sclerosus in both sexes and in all age groups.14 These have been used long term (weeks to months) or daily, without significant adverse cutaneous effects, although patients should always be monitored regularly adverse effects.
Corticosteroids
Reducing inflammation helps reduce symptoms and, in some cases, helps resolve the lesion.15,16
Clobetasol (Temovate)
Class I superpotent topical steroid, Suppresses mitosis and increases synthesis of proteins that decrease inflammation and cause vasoconstriction. Other superpotent steroids would be expected to have the same results; however, most published studies showing benefit of such medications have used clobetasol.
Pulse dosing (2 consecutive d/wk) may be used long-term, even in genital cases. On off-days, a milder steroid or emollient alone may be used. No additional benefit in using potent and super-potent steroids >qd. Especially in genital applications, bid application of such a medication will more quickly lead to steroid adverse effects.
Adult
Apply qd for 12 wk or less if symptoms are improved
Pediatric
Administer as in adults; genital use for 3 mo or longer is reported without adverse effects
None reported
Documented hypersensitivity; viral or fungal skin infections
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May suppress adrenal function in prolonged therapy; observe for evidence of steroid atrophy; consider biopsy to exclude malignancy in patients with genital lichen sclerosus who have good response yet have a single persistent lesion involved or ulcerated area
Hydrocortisone (LactiCare HC, Westcort, Dermacort, Cortaid, DermaGel)
An adrenocorticosteroid derivative suitable for application to skin or external mucous membranes. It has mineralocorticoid and glucocorticoid effects resulting in anti-inflammatory activity.
Adult
Apply sparingly to affected areas bid/qid
Pediatric
Apply as in adults
None reported
Documented hypersensitivity; viral, fungal, and bacterial skin infections
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Prolonged use, applying over large surface areas, application of potent steroids, and occlusive dressings may increase systemic absorption of corticosteroids and may cause Cushing's syndrome, reversible HPA axis suppression, hyperglycemia, and glycosuria
Retinoids
Mechanism of action for successful effects systemic retinoids have had in lichen sclerosus (usually studied in female genital cases) is not clear but may have to do with down-regulation of fibroblast function.
Acitretin (Soriatane)
Retinoic acid analog, like etretinate and isotretinoin. Etretinate is main metabolite and has demonstrated clinical effects close to those seen with etretinate. Mechanism of action is unknown. Has been reported to benefit both symptoms and induce resolution of lesion. Although not well studied, combining a medication in this class with a topical corticosteroid as described above may be useful in refractory cases.
Adult
25 or 50 mg/d PO given as single dose with main meal initial; 25-50 mg/d PO after initial response to treatment; terminate therapy when lesions have resolved sufficiently
Pediatric
Not established
Increases toxicity of methotrexate (avoid concomitant use); interferes with effects of microdosed progestin minipill; concomitant use with tetracycline product may lead to pseudotumor cerebri; coadministration with alcohol may enhance synthesis of etretinate, which has much longer half-life than acitretin (>120 d)
Documented hypersensitivity; pregnancy must not be planned for at least 3 y after cessation of drug, many recommend medication be avoided if patient is planning to ever be pregnant, especially if patient consumes alcohol, which encourages conversion to teratogenic metabolite that may persist indefinitely
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Do not use in severe obesity; women of childbearing age must be capable of complying with effective contraceptive measures; perform AST, ALT, and LDH tests prior to initiation of acitretin therapy at 1- to 2-wk intervals until stable and thereafter at intervals as clinically indicated
Isotretinoin (Accutane)
Oral agent that treats serious dermatologic conditions. Synthetic 13-cis isomer of the naturally occurring tretinoin (trans -retinoic acid). Both agents are structurally related to vitamin A.
Decreases sebaceous gland size and sebum production. May inhibit sebaceous gland differentiation and abnormal keratinization. Has been reported to benefit both symptoms and may help resolution of lesion itself. Although not well studied, combining a medication in this class with a topical corticosteroid as described above may be useful in refractory cases.
Adult
0.5-1 mg/kg/d PO; duration of therapy will likely be at least 4-6 mo
Pediatric
Administer as in adults
Toxicity may occur with vitamin A coadministration; pseudotumor cerebri or papilledema may occur when coadministered with tetracyclines; acitretin may reduce plasma levels of carbamazepine
Documented hypersensitivity; pregnancy
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
May decrease night vision; inflammatory bowel disease may occur; may be associated with development of hepatitis; occasional exaggerated healing response of acne lesions (excessive granulation with crusting) may occur; diabetes patients may experience problems in controlling blood sugar; avoid exposure to UV light or sunlight until tolerance achieved; discontinue treatment if rectal bleeding, abdominal pain, or severe diarrhea occur
Topical calcineurin inhibitors (topical immune modulators)
Many have concerns about the use of potent topical corticosteroids in occluded areas such as the genitals. They often do not work as well or as fast as the corticosteroids when used as monotherapy, but this class of medications may have a role either as maintenance medications after steroid-driven improvement or in conjunction with them in a pulse-steroid regimen (ie, superpotent corticosteroid on weekend days and a TIM during the week). Some believe this may delay or obviate the onset of tachyphylaxis and reduce the risk of steroid atrophy. This is an off-label use for both medications. Recently, some have raised concerns about the use of these medications long term, especially in young children and infants. The exact risks of long-term use, if any, remain to be defined, but the prescriber should be aware of the issues.
Tacrolimus (Protopic)
Mechanism of action in atopic dermatitis is not known. Reduces itching and inflammation by suppressing release of cytokines from T cells. Also inhibits transcription for genes that encode IL-3, IL-4, IL-5, GM-CSF, and TNF-alpha, all of which are involved in the early stages of T-cell activation. Additionally, may inhibit release of preformed mediators from skin mast cells and basophils and may down-regulate expression of FCeRI on Langerhans cells. Indicated in dermatitis patients as young as 2 y. Drugs of this class are more expensive than topical corticosteroids. Available as an ointment in concentrations of 0.03% and 0.1%.
Adult
Apply thin layer to affected skin areas bid and rub in gently and completely; continue treatment for 1 wk after clearing of signs and symptoms
Pediatric
<2 years: Not established
2-15 years: Apply 0.03% ointment bid to affected area(s)
>15 years: Administer as adults
None reported
Documented hypersensitivity to tacrolimus or components of ointment
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Patients may experience a burning sensation during first few days of application; skin can become photosensitive, and patients should be cautioned about exposure to direct or artificial sunlight and to use sunscreen
Safety and efficacy in infected atopic dermatitis is not known
Application under occlusion, which may promote systemic exposure, has not been evaluated (do not use with occlusive dressings)
Absorption following topical applications is minimal (relative to systemic administration), but is excreted in human milk and, thus, a decision should be made whether to discontinue nursing or to discontinue drug, taking into account importance of drug to mother (potential for serious adverse reactions in nursing infants from tacrolimus should also be a concern)
Caution with conditions that suppress the immune system (eg, AIDS, cancer)
Possible risk of lymph node or skin cancer based on animal studies and a small number of patients; may increase risk of viral infections; other adverse effects include headache, sore throat, flulike symptoms, fever, and cough
Pimecrolimus (Elidel)
First nonsteroid cream approved in the United States for mild-to-moderate atopic dermatitis. Derived from ascomycin, a natural substance produced by Streptomyces hygroscopicus var. ascomyceticus. Selectively inhibits production and release of inflammatory cytokines from activated T cells by binding to cytosolic immunophilin receptor macrophilin-12. The resulting complex inhibits phosphatase calcineurin, thus blocking T-cell activation and cytokine release. Cutaneous atrophy not observed in clinical trials, a potential advantage over topical corticosteroids. Indicated only after other treatment options have failed
Adult
Apply topically to affected areas bid
Pediatric
<2 years: Not established
>2 years: Administer as in adults
None reported
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Potential exacerbation of existing infection at site of application; may cause burning and irritation; caution with conditions that suppress the immune system (eg, AIDS, cancer); possible risk of lymph node or skin cancer based on animal studies and a small number of patients; may increase risk of viral infections; other adverse effects include headache, sore throat, flulike symptoms, fever, and cough
More on Lichen Sclerosus et Atrophicus |
| Overview: Lichen Sclerosus et Atrophicus |
| Differential Diagnoses & Workup: Lichen Sclerosus et Atrophicus |
 Treatment & Medication: Lichen Sclerosus et Atrophicus |
| Follow-up: Lichen Sclerosus et Atrophicus |
| Multimedia: Lichen Sclerosus et Atrophicus |
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References
Chan I, Oyama N, Neill SM, Wojnarowska F, Black MM, McGrath JA. Characterization of IgG autoantibodies to extracellular matrix protein 1 in lichen sclerosus. Clin Exp Dermatol. Sep 2004;29(5):499-504. [Medline].
Regauer S, Liegl B, Reich O, Beham-Schmid C. Vasculitis in lichen sclerosus: an under recognized feature?. Histopathology. Sep 2004;45(3):237-44. [Medline].
Kowalewski C, Kozlowska A, Chan I, et al. Three-dimensional imaging reveals major changes in skin microvasculature in lipoid proteinosis and lichen sclerosus. J Dermatol Sci. Jun 2005;38(3):215-24. [Medline].
Hunger RE, Bronnimann M, Kappeler A, Mueller C, Braathen LR, Yawalkar N. Detection of perforin and granzyme B mRNA expressing cells in lichen sclerosus. Exp Dermatol. May 2007;16(5):416-20. [Medline].
De Vito JR, Merogi AJ, Vo T, et al. Role of Borrelia burgdorferi in the pathogenesis of morphea/scleroderma and lichen sclerosus et atrophicus: a PCR study of thirty-five cases. J Cutan Pathol. Aug 1996;23(4):350-8. [Medline].
Eisendle K, Grabner T, Kutzner H, Zelger B. Possible role of Borrelia burgdorferi sensu lato infection in lichen sclerosus. Arch Dermatol. May 2008;144(5):591-8. [Medline].
Cooper SM, Ali I, Baldo M, Wojnarowska F. The association of lichen sclerosus and erosive lichen planus of the vulva with autoimmune disease: a case-control study. Arch Dermatol. Nov 2008;144(11):1432-5. [Medline].
Gunthert AR, Faber M, Knappe G, Hellriegel S, Emons G. Early onset vulvar Lichen Sclerosus in premenopausal women and oral contraceptives. Eur J Obstet Gynecol Reprod Biol. Mar 2008;137(1):56-60. [Medline].
Prowse DM, Ktori EN, Chandrasekaran D, Prapa A, Baithun S. Human papillomavirus-associated increase in p16INK4A expression in penile lichen sclerosus and squamous cell carcinoma. Br J Dermatol. Feb 2008;158(2):261-5. [Medline].
Stucker M, Grape J, Bechara FG, Hoffmann K, Altmeyer P. The outcome after cryosurgery and intralesional steroid injection in vulvar lichen sclerosus corresponds to preoperative histopathological findings. Dermatology. 2005;210(3):218-22. [Medline].
Kreuter A, Gambichler T. Narrowband UV-B phototherapy for extragenital lichen sclerosus. Arch Dermatol. Sep 2007;143(9):1213. [Medline].
Romero A, Hernandez-Nunez A, Cordoba-Guijarro S, Arias-Palomo D, Borbujo-Martinez J. Treatment of recalcitrant erosive vulvar lichen sclerosus with photodynamic therapy. J Am Acad Dermatol. Aug 2007;57(2 Suppl):S46-7. [Medline].
Vermaat H, Smienk F, Rustemeyer T, Bruynzeel DP, Kirtschig G. Anogenital allergic contact dermatitis, the role of spices and flavour allergy. Contact Dermatitis. Oct 2008;59(4):233-7. [Medline].
Fischer G, Rogers M. Treatment of childhood vulvar lichen sclerosus with potent topical corticosteroid. Pediatr Dermatol. May-Jun 1997;14(3):235-8. [Medline].
Bornstein J, Heifetz S, Kellner Y, Stolar Z, Abramovici H. Clobetasol dipropionate 0.05% versus testosterone propionate 2% topical application for severe vulvar lichen sclerosus. Am J Obstet Gynecol. Jan 1998;178(1 Pt 1):80-4. [Medline].
Lindhagen T. Topical clobetasol propionate compared with placebo in the treatment of unretractable foreskin. Eur J Surg. Dec 1996;162(12):969-72. [Medline].
Jones RW, Sadler L, Grant S, Whineray J, Exeter M, Rowan D. Clinically identifying women with vulvar lichen sclerosus at increased risk of squamous cell carcinoma: a case-control study. J Reprod Med. Oct 2004;49(10):808-11. [Medline].
Fung MA, LeBoit PE. Light microscopic criteria for the diagnosis of early vulvar lichen sclerosus: a comparison with lichen planus. Am J Surg Pathol. Apr 1998;22(4):473-8. [Medline].
Meffert JJ, Davis BM, Grimwood RE. Lichen sclerosus. J Am Acad Dermatol. Mar 1995;32(3):393-416; quiz 417-8. [Medline].
Pugliese JM, Morey AF, Peterson AC. Lichen sclerosus: review of the literature and current recommendations for management. J Urol. Dec 2007;178(6):2268-76. [Medline].
Thomas RH, Ridley CM, McGibbon DH, Black MM. Anogenital lichen sclerosus in women. J R Soc Med. Dec 1996;89(12):694-8. [Medline].
Further Reading
Keywords
lichen sclerosus, lichen sclerosus et atrophicus, kraurosis vulvae, balanitis xerotica obliterans
