Published on 19/03/2015 by admin
Filed under Dermatology
Last modified 19/03/2015
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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
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).
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.
Biopsy
Autoimmune screen if clinically indicated
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.
Fung MA, LeBoit PE. Am J Surg Pathol 1998; 22: 473–8.
Nine out of 68 cases of LS reviewed were found to have sections that showed a transition from a lichenoid reaction to pathognomonic LS (dermal sclerosis or edema), thus demonstrating the early histopathological signs of LS. A psoriasiform lichenoid pattern, basilar epidermotropism, loss of papillary dermal elastic fibres, basement membrane thickening and epidermal atrophy were shown to be much more suggestive of LS than lichen planus.
Raspollini MR, Asirelli G, Moncini D, Taddei GL. Am J Obstet Gynecol 2007; 197: 592e1–5.
A retrospective study comparing the histological features of eight cases of LS that developed SCC with eight cases of LS followed for at least 9 years without any malignant change. Labelling for MIB1 and p53 was increased in the cases that subsequently developed an SCC.
Chi CC, Kitschig G, Baldo M, Brackenbury F, Lewis F, Wojnarowska F. Cochrane Database Syst Rev 2011; (7): CD008240.
A Cochrane review including seven randomly controlled trials with a total of 249 patients and investigating six treatments. This concluded that topical clobetasol propionate 0.05% and mometasone furoate were effective in treating LS, when compared to placebo. No significant benefit was found for topical testosterone, dihydrotestosterone, and progesterone.
Bracco GL, Carli P, Sonni L, Maestrini G, De Marco A, Taddei GL, et al. J Reprod Med 1993; 38: 37–40.
A randomized study of 79 adults treated for 3 months. Remission of symptoms occurred in 75% treated with clobetasol 0.05%, 20% treated with 2% testosterone, and 10% treated with 2% progesterone or placebo.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
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Ultrapotent topical steroids
Emollients
Use of soap substitutes
