Lichen sclerosus

Published on 19/03/2015 by admin

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Lichen sclerosus

Fiona M. Lewis

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Lichen sclerosus (LS) is a chronic scarring dermatosis that has a predilection for the anogenital skin. It is much more common in females and has two peaks of incidence – in girls before puberty and in women after the menopause – but can occur in men and extragenitally. It appears to be lymphocyte mediated and an autoimmune etiology is likely given the strong association with other autoimmune disorders and the presence of circulating antibodies to extracellular matrix protein 1. A small number of patients with genital LS (<5%) will develop a squamous cell carcinoma (SCC).

Management strategy

The aims of management are to alleviate symptoms, reduce scarring, and, by careful follow-up, to detect any premalignant and malignant change.

The most common symptom in LS in females is pruritus, but soreness and pain can occur if the skin fissures. In children, constipation is often the presenting symptom. White atrophic plaques are the typical clinical feature with ecchymosis being a frequent observation. Occasionally, the plaques may be thickened and hyperkeratotic. The perianal skin may be involved in a figure of eight pattern. If untreated, scarring will lead to changes in the vulval architecture. The labia minora may be lost and the clitoral hood can fuse leading to burial of the clitoris and occasionally to clitoral pseudocyst formation. Narrowing of the introitus can cause dyspareunia, and fusion of the labia minora may eventually lead to difficulties with micturition.

In males, tightening of the foreskin causes phimosis and subsequently problems with erection and intercourse. Meatal stenosis leads to difficulties with micturition. Perianal disease is rarely a feature in men.

Secondary pain syndromes can develop in both sexes and it is important to recognize these sensory disorders (vulvodynia, penile dysesthesia) as management must be directed to these rather than alter the treatment for LS. Any psychosexual issues must be recognized and addressed.

The first-line treatment for LS in adults and children and in both sexes is an ultrapotent topical steroid. There are no randomized trials comparing formulation or frequency of application but the general recommended regimen is clobetasol propionate 0.05% ointment once a day for a month, on alternate days for a month and then twice weekly for a further month. After this 3-month period, the patient can apply treatment once daily if any symptoms occur. A 30 g tube should last at least 3 months in an adult and 6 months in a child. Most patients use much less than this. With successful treatment, the ecchymosis, fissuring and hyperkeratosis should resolve and scarring should not progress. The pallor will often remain and any scarring present before treatment is started will not reverse. There is no direct evidence that shows that treatment can reduce the risk of developing an SCC.

Soap substitutes, e.g., emulsifying ointment, are helpful symptomatically and barrier preparations are recommended if there is urinary incontinence. Stool softeners can be helpful in children if constipation is an issue.

In women, surgery is only recommended for excision of any malignant lesion that develops, or to release labial adhesions that are causing severe introital narrowing. However, in males circumcision may be a very helpful part of management.

Specific investigations

A diagnosis of LS can often be made on the classic clinical appearances. Biopsy is mandatory if there are unusual or atypical features or in those who have failed to respond to first-line treatment.

First-line therapy

imageUltrapotent topical steroids A
imageEmollients E
imageUse of soap substitutes E

Second-line therapies

imageCircumcision B
imageTopical tacrolimus B
imageTopical pimecrolimus A

Third-line therapies

imageAcitretin A
imageCO2 laser B

Other therapies

imageIntralesional triamcinolone D
imageSurgery for clitoral phimosis D
imageCryosurgery C
imageLow dose UVA1 phototherapy C
imagePhotodynamic therapy with 5-aminolevulinic acid C
imageNarrowband UVB E
imageCyclosporine D
imageHydroxycarbamide E
imageHydroxychloroquine E
imageMethotrexate E
imageAntibiotics D
imageTopical calcipotriol B
imageTopical tretinoin B
imageOxatomide gel B
imageOral calcitriol E
imagePotassium p-aminobenzoate D
imageTopical estrogens E
imageTopical testosterone B
imageOral stanozolol B
imageIcilin E
imageFat transfer – adipose derived mesenchymal cells and platelet rich plasma B
imageFocused ultrasound therapy B

[/level-membership-for-dermatology-category][not-level-membership-for-dermatology-category]

Lichen sclerosus

Fiona M. Lewis

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Lichen sclerosus (LS) is a chronic scarring dermatosis that has a predilection for the anogenital skin. It is much more common in females and has two peaks of incidence – in girls before puberty and in women after the menopause – but can occur in men and extragenitally. It appears to be lymphocyte mediated and an autoimmune etiology is likely given the strong association with other autoimmune disorders and the presence of circulating antibodies to extracellular matrix protein 1. A small number of patients with genital LS (<5%) will develop a squamous cell carcinoma (SCC).

Management strategy

The aims of management are to alleviate symptoms, reduce scarring, and, by careful follow-up, to detect any premalignant and malignant change.

The most common symptom in LS in females is pruritus, but soreness and pain can occur if the skin fissures. In children, constipation is often the presenting symptom. White atrophic plaques are the typical clinical feature with ecchymosis being a frequent observation. Occasionally, the plaques may be thickened and hyperkeratotic. The perianal skin may be involved in a figure of eight pattern. If untreated, scarring will lead to changes in the vulval architecture. The labia minora may be lost and the clitoral hood can fuse leading to burial of the clitoris and occasionally to clitoral pseudocyst formation. Narrowing of the introitus can cause dyspareunia, and fusion of the labia minora may eventually lead to difficulties with micturition.

In males, tightening of the foreskin causes phimosis and subsequently problems with erection and intercourse. Meatal stenosis leads to difficulties with micturition. Perianal disease is rarely a feature in men.

Secondary pain syndromes can develop in both sexes and it is important to recognize these sensory disorders (vulvodynia, penile dysesthesia) as management must be directed to these rather than alter the treatment for LS. Any psychosexual issues must be recognized and addressed.

The first-line treatment for LS in adults and children and in both sexes is an ultrapotent topical steroid. There are no randomized trials comparing formulation or frequency of application but the general recommended regimen is clobetasol propionate 0.05% ointment once a day for a month, on alternate days for a month and then twice weekly for a further month. After this 3-month period, the patient can apply treatment once daily if any symptoms occur. A 30 g tube should last at least 3 months in an adult and 6 months in a child. Most patients use much less than this. With successful treatment, the ecchymosis, fissuring and hyperkeratosis should resolve and scarring should not progress. The pallor will often remain and any scarring present before treatment is started will not reverse. There is no direct evidence that shows that treatment can reduce the risk of developing an SCC.

Soap substitutes, e.g., emulsifying ointment, are helpful symptomatically and barrier preparations are recommended if there is urinary incontinence. Stool softeners can be helpful in children if constipation is an issue.

In women, surgery is only recommended for excision of any malignant lesion that develops, or to release labial adhesions that are causing severe introital narrowing. However, in males circumcision may be a very helpful part of management.

Specific investigations

A diagnosis of LS can often be made on the classic clinical appearances. Biopsy is mandatory if there are unusual or atypical features or in those who have failed to respond to first-line treatment.

First-line therapy

imageUltrapotent topical steroids A
imageEmollients E
imageUse of soap substitutes E

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