Lichen sclerosus

Lichen sclerosus
ICD-10	L90.0
ICD-9	701.0
eMedicine	derm/234

Template:Search infobox Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Kiran Singh, M.D. [2]

Overview

Lichen sclerosus (LS) (also known as lichen sclerosus et atrophicus (LSA), white-spot disease) is an uncommon disease of unknown cause that results in white patches on the skin, which may cause scarring on and around genital skin.

Several risk factors have been proposed, including autoimmune disease, infections and genetic predisposition. The disease is be associated with thyroid disease.

Synonyms and short history

Lichen Sclerosis (LS), Lichen Sclerosus et Atrophicus (LSA), Balanitis xerotica obliterans (BXO), Csillag's disease, White Spot Disease, kraurosis vulvae, lichen albus and lichen sclerosus et atrophicans. These are all different names for the same disease. Typically it's called LSA or BXO when it affects men, LS when it affects women or in referring to the disease in general.

LS was first described in 1887 by Dr. Hallopeau. In 1989 the International Society for the Study of Vulvovaginal Disease (ISSVD) officially proclaimed the name ‘lichen sclerosus’. Since then this is the official medical name for this disease.

General

Women are more commonly affected than men, particularly around and after menopause, but girls may also get the disease. The condition most commonly occurs on the vulva and around the anus with ivory-white elevations that may be flat and glistening. There may be marked itching or the condition may be without any symptoms. There may also be thinning and shrinkage of the genital area that may make coitus painful.

In males, the disease may take the form of whitish thickening of the foreskin, which cannot be retracted easily. One study has revealed that 51 (98%) of 52 patients diagnosed with penile LS were uncircumcised. [3] In men, this genital involvement has traditionally been known as balanitis xerotica obliterans (BXO).[4]

On the non-genital skin, the disease may manifest as porcelain-white spots with small visible plugs inside the orifices of hair follicles or sweat glands on the surface. Thinning of the skin may also occur.

Diagnosis needs sometime biopsy because LSA may be difficult to differentiate from condyloma. Histologically there's hyperkeratosis, atrophic epidermis, sclerosis of dermis and lymphocyte activity in dermis.

The disease often goes undiagnosed for several years, as it is sometimes not recognised and misdiagnosed as thrush or other problems and not correctly diagnosed until the patient is referred to a specialist when the problem does not clear up.

The disease can last for a long time. Occasionally, spontaneous cure may ensue, particularly in young girls.

When it occurs on the vulva, it leads in 1-4 % of cases to vulvar carcinoma. For men it might be a major cause for tight foreskin, phimosis.

LS is usually treated with application of potent steroids, which may cause relief and prevent scarring. Other options are cryotherapy, and laser therapy. Occasionally, cancer may develop on the patches.[5] Periodic consultation is therefore necessary.

LS may cause sufferers to feel depressed due to their condition, due to the pain caused and feelings that they are 'not normal', 'can't have sex properly', 'the only one like this' and other self-esteem issues. It may be useful for them to contact other sufferers and speak to them, to see they are not alone and since they may understand what they are feeling better than non-sufferers, or speak a counsellor to work through any issues. Support groups exist for sufferers who may be able to help in this respect, one example is the National Lichen Sclerosus Support Group which is based in the UK, but does have an international membership. Their website also contains a lot of useful information on Lichen Sclerosis, its symptoms and its treatment.

Diagnosis

Physical Examination

Skin

Trunk

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

Genitalia

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

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  Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.

See also

• Lichen simplex
• Balanitis xerotica obliterans

References

1. S.M. Neill, F.M. Tatnall, N.H. Cox: Guidelines for the management of lichen sclerosus: British Journal of Dermatology 2002; 147: 640-649.

External links

Support groups

• National Lichen Sclerosus Support Group
• Lichen Sclerosus Support and Awareness Group

Medical information

• OMNI
• NIAMS - Questions and Answers About Lichen Sclerosus
• NIAMS - Fast Facts About Lichen Sclerosus
• eMedicine
• vulvarhealth.org
• dermnetnz.org
• mayoclinic.com

medical pictures (NOT FOR MINORS)

http://www.dermlectures.com/LecturesWMV.cfm?lectureID=88 http://dermis.multimedica.de/dermisroot/de/34088/diagnose.htm http://dermnetnz.org/immune/ls-imgs.html

Medical Literature

Yesudian PD, Sugunendran H, Bates CM, O’Mahony C. Lichen sclerosus. Int J STD AIDS 2005; 16:465-74

Tasker GL, Wojnarowska F. Lichen Sclerosus. Clin Exp Dermatol 2003; 28:128-33

Smith YR, Haefner HK, Vulvar lichen sclerosus: pathophysiology and treatment. Am J Clin Dermatol 2004;5:105-25

Powell JJ, Wojnarowska F., Lichen sclerosus. Lancet 1999;353:1777-83

Neill SM, Ridley CM, Management of anogenital lichen sclerosus. Clin Exp Dermatol 2001; 26:637-43

Marini A, Blecken S, Ruzicka T, Hengge UR. Lichen sclerosus. Hautartzt 2005;56:550-55

Cooper SM, Gau XH, Powel JJ, Wojnarowska F. Does treatment of vulvar lichen sclerosus influence its prognosis? Arch Dermatol 2004;140:702-06

Funaro D. Lichen Sclerosus: a review and practical approach. Dermatol Ther. 2004;17(1):28-37. Review.

Meffert JJ, Davis BM, Grimwood RE. Lichen sclerosus. J Am Acad Dermatol. 32, no 3. 1995: 393-416.

Smith YR, Haefner HK. Vulvar lichen sclerosus : pathophysiology and treatment. Am J Clin Dermatol. 2004;5(2):105-25.

Wong YW, Powell J, Oxon MA. Lichen sclerosus. A review. Minerva Med. 2002 Apr;93(2):95-9. Review.

Powell JJ, Wojnarowska F. Lichen Sclerosus. Lancet. 1999;353:1777-83. Murphy FR, Lipa M, Haberman HF. Familial vulvar dystrophy of Lichen Sclerosus type. Arch Dermatol 1982;118:329-31

Marren P, Yell J, Charnock FM, et al. The association between Lichen Sclerosus and antigens of the HLA system. Br. J. Dermatol 1995;132:197-203

Goolamali SK, Barnes EW, Irvine WJ, et al. Organ specific antibodies in patients with Lichen Sclerosus et Atrophicus. BMJ 1974;4:78-9.

Meyrick Thomas RH, Riedley CM, McGibbon DH, et al. Lichen Sclerosus et Atrophicus and autoimmunity: a study of 350 women. Br. J. Dermatol 1988;118:41-6

Marren P, Cherry C, Day A et al. Lichen Sclerosus: the patient, the hormon influenceonal i and disease impact (abstract) Br J Dermatol 1995;21:133.

Lorenz B, Kaufman RH, Kutzner SK. Lichen Sclerosus. Therapy with clobetasol propionate.J Reprod Med. 1998 Sep;43(9):790-4.

Marren P, Millard PR, Wojnarowska F.Vulval Lichen Sclerosus: lack of correlation between duration of clinical symptoms and histological appearances J Eur Acad Dermatol Venereol. 1997;(8):212-6

Fischer G, Rogers M. Treatment of childhood vulvar Lichen Sclerosus with potent topical corticosteroid. Pediatr Dermatol. 1997 May-Jun;14(3):235-8.

Powell J, Wojnarowska F. Childhood vulvar Lichen Sclerosus: an increasingly common problem. J Am Acad Dermatol. 2001 May;44(5):803-6.

Garcia-Bravo B, Sanchez-Pedreno P, Rodriguez-Pichardo A, Camacho F. Lichen Sclerosus et Atrophicus. A study of 76 cases and their relation to Diabetes. J Am Acad Dermatol. 1988 Sep;19(3):482-5.

Bracco GL, Carli P, Sonni L, et al. Clinical and histologic effects of topical treatments of vulval Lichen Sclerosus. A critical evaluation. J Reprod. Med. 1993;38:37-40.

Mazdisnian F, Degregorio F, Palmieri A. Intralesional injection oftriamcinolone in the treatment of Lichen Sclerosus. J Reprod. Med. 1999;44:332-334.

Sideri M, Origoni M, Spinaci L, et al. Topical testosterone in the treatment of vulvar Lichen Sclerosus. Int J Gynaecol obstet. 1994;46:53-56.

Ayhan A., Urman B, Yuce K. et al. Topical testosterone for Lichen Sclerosus. Int J Gynaecol Obstet. 1989;30(3):253-255.

Bousema MT, Romppanen U, Geiger JM. Acitretin in the treatment of servere Lichen Sclerosus et atrophicus of the vulva: a double-blind placebo controlled study. J Am Acad Dermotol 1994; 30:225-231.

Hillemans P, Untch M, Prove F, et al. Photodynamic therapy of vulvar Lichen Sclerosus with 5-aminolevulinic acid. Obstet. Gynecol 1999;93(1):71-74.

Assmann T, Becker-Wegerich P, Greve M, et al. Tacrolimus ointment for the treatment of vulvar Lichen Sclerosus. J. Ann Acad Dermatol 2003;48(6):935-937

Bohm M, Frieling U, Luger TA, et al. Successful treatment of anogenital Lichen Sclerosus with topic tacrolimus. Arch Dermatol 2003;139(7):922-924.

Goldstein AT, Marinoff SC, Christopher K. Pimecrolimus for the treatment of vulvar Lichen Sclerosus in a premenarchal girl. J Pediatr Adolesc Gynecol 2004;17(1):35-37.

Rouzier R, Haddad B, Deyrolle C, et al. Perineoplasty for the treatment of introital stenosis related to vulvar Lichen Sclerosus. Am J Obstet. Gynecol 2002;186(1):49-52.

Abramov Y, Elchalal U, Abramov D, et al. Surgical treatment of vulvar Lichen Sclerosus: a review. Obstet Gynecol Surv. 1996;51(3):193-199.

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