Lichen sclerosus

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

Synonyms and keywords: Lichen sclerosus et athrophicus, white-spot disease; 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

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. ^[1]

Historical Perspective

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.

Classification

Pathophysiology

Causes

Differentiating Lichen Sclerosus from other Diseases

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.

Epidemiology and Demographics

Age

Gender

Women are more commonly affected than men, particularly around and after menopause, but girls may also get the disease.

Race

Risk Factors

Natural History, Complications and Prognosis

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.

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.

Diagnosis

Diagnostic Criteria

Symptoms

Physical Examination

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.

Skin

Trunk

Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]

Genitalia

Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]
Lichen sclerosus et atrophicus. Adapted from Dermatology Atlas.^[2]

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

LS is usually treated with application of potent steroids, which may cause relief and prevent scarring. ^[3] Other options are cryotherapy, and laser therapy. Occasionally, cancer may develop on the patches.[5] Periodic consultation is therefore necessary.

Surgery

Prevention

References

↑ Pugliese JM, Morey AF, Peterson AC (2007). "Lichen Sclerosus: Review of the Literature and Current Recommendations for Management". PMID 17936829.
↑ ^2.00 ^2.01 ^2.02 ^2.03 ^2.04 ^2.05 ^2.06 ^2.07 ^2.08 ^2.09 ^2.10 ^2.11 ^2.12 ^2.13 ^2.14 ^2.15 ^2.16 ^2.17 ^2.18 ^2.19 ^2.20 ^2.21 ^2.22 ^2.23 ^2.24 ^2.25 ^2.26 ^2.27 ^2.28 ^2.29 "Dermatology Atlas".
↑ Goolamali SK, Goolamali SI (1997). "Lichen sclerosus". Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and Gynaecology. 17 (1): 5–12. PMID 15511755.

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

Medical information

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.

Template:WikiDoc Sources

[1] Pugliese JM, Morey AF, Peterson AC (2007). "Lichen Sclerosus: Review of the Literature and Current Recommendations for Management". PMID 17936829.

[Dermatology_Atlas-2] 2.00 ^2.01 ^2.02 ^2.03 ^2.04 ^2.05 ^2.06 ^2.07 ^2.08 ^2.09 ^2.10 ^2.11 ^2.12 ^2.13 ^2.14 ^2.15 ^2.16 ^2.17 ^2.18 ^2.19 ^2.20 ^2.21 ^2.22 ^2.23 ^2.24 ^2.25 ^2.26 ^2.27 ^2.28 ^2.29 "Dermatology Atlas".

[3] Goolamali SK, Goolamali SI (1997). "Lichen sclerosus". Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and Gynaecology. 17 (1): 5–12. PMID 15511755.

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v t e Diseases of the skin and subcutaneous tissue (integumentary system) (L, 680-709)
Infections	Template:Navbox subgroup
Bullous disorders	acantholysis (Pemphigus, Transient acantholytic dermatosis) · Pemphigoid (Bullous, Cicatricial, Gestational) · Dermatitis herpetiformis
Inflammatory	Template:Navbox subgroup
Radiation-related disorders	Sunburn · actinic rays (Actinic keratosis, Actinic cheilitis) · Polymorphous light eruption (Acne aestivalis) · Radiodermatitis · Erythema ab igne
Pigmentation disorder	hypopigmentation (Albinism, Vitiligo) · hyperpigmentation (Melasma, Freckle, Café au lait spot, Lentigo/Liver spot, Acanthosis nigricans, Acral acanthotic anomaly)
Other skin	keratosis/hyperkeratosis (Seborrheic keratosis, Callus) · other epidermal thickening (Ichthyosis acquisita, Palmoplantar keratoderma) skin ulcer (Pyoderma gangrenosum, Bedsore) Cutaneous Markers of Internal Malignancy (Florid cutaneous papillomatosis, acanthosis nigricans, sign of Leser-Trelat) atrophic (Lichen sclerosus, Acrodermatitis chronica atrophicans) necrobiosis (Granuloma annulare, Necrobiosis lipoidica) · other granuloma (Granuloma faciale, Pyogenic granuloma) cutaneous vasculitis (Livedoid vasculitis, Erythema elevatum diutinum)
Connective tissues	collagen disease: Keloid localized connective tissue disorders: Lupus erythematosus (Discoid lupus erythematosus, Subacute cutaneous lupus erythematosus) · Scleroderma/Morphea · Linear scleroderma · Calcinosis cutis · Sclerodactyly · Ainhum
see also congenital, neoplasia