Elsevier

The Lancet

Volume 353, Issue 9166, 22 May 1999, Pages 1777-1783
The Lancet

Seminar
Lichen sclerosus

https://doi.org/10.1016/S0140-6736(98)08228-2Get rights and content

Summary

Lichen sclerosis is a chronic inflammatory skin disease that causes substantial discomfort and morbidity, most commonly in adult women, but also in men and children. Any skin site may be affected (and, rarely, the oral mucosa) but lichen sclerosus is most common in the anogenital area, where it causes intractable itching and soreness. In children, the disorder may be confused with changes seen in sexual abuse. Progression to destructive scarring is common. There is increased risk of developing vulval cancer, and there are links with penile cancer. Patients should be kept under long-term review. Lichen sclerosus can occur without symptoms, and the exact prevalence is uncertain. It occurs most commonly in women at times of low sex hormone output. The underlying cause is unknown, but there seems to be a genetic susceptibility and a link with autoimmune mechanisms. The wart virus and the spirochaete borrelia have been suggested but not substantiated as infective triggers. The Koebner phenomenon is known to occur (lichen sclerosus occurs in skin already scarred or damaged), so trauma, injury, and sexual abuse have been suggested as possible triggers of symptoms in genetically predisposed people. The treatment of choice for anogenital lichen sclerosus is potent topical corticosteroid ointment for a limited time. Circumcision may be indicated in men, and surgery may be considered in women, to relieve effects of scarring or to treat coexisting carcinoma. Current research aims to identify a treatable cause of lichen sclerosus, to identify patients at risk of scarring and of malignant disorders, and to find target pathways for therapeutic intervention.

Section snippets

Epidemiology

Onset of lichen sclerosus has been reported at all ages, although it is not common under 2 years old. The mean age of onset is the fifth to sixth decade in women.2, 3, 4, 5 Lichen sclerosus is more common in women than men: some studies give a female to male ratio of ten to one, others six to one.4, 5, 6 In women, the disorder is most common when endogenous oestrogen production is low, which led to attempts to confirm a protective effect of oestrogen.7 No association with age at menarche or

Clinical features

Lichen sclerosus most commonly affects the anogenital area (85–98% of cases), with extragenital lesions in 15–20% of patients.5, 6, 16

In women, genital lesions may appear as a figure-of-eight pattern around the vulva and anus. Patients report intractable pruritis and soreness of the vulval and perianal areas. Patients may have dysuria, dyspareunia, and pain on defecation. Painful traumatic fissures and tears may occur with sexual intercourse and with defecation. Other patients may have no

Lichen sclerosus and malignancy

Studies of large groups of women with lichen sclerosus5, 6 have shown that the risk of squamous-cell carcinoma (SCC) of the vulva is 4–5%. The association between lichen sclerosus and SCC has been known for decades, but whether lichen sclerosus is actually premalignant is not certain. In excised specimens of vulval SCC, Zaino and colleagues26 found evidence of lichen sclerosus in adjacent skin in 25% of cases, and Liebowitch and colleagues27 found similar evidence in up to 61% of 78 cases. In

Histological changes

Lichen sclerosus has specific histological features, so biopsy is worthwhile not only to rule out malignant change, but also to differentiate lichen sclerosus from erosive lichen planus, scarring cicatrical pemphigoid, lichen simplex, and morphoea. The classic histological features are hydropic degeneration of the basal cells and a pale-staining homogeneous zone in the upper dermis, below which is a band of inflammatory cells that are mainly monocytic (figure 6). Some researchers have proposed

Genetic factors

Familial lichen sclerosus has been reported in identical twins42 and non-identical twins,43 sisters, mothers, and daughters. The HLA complex seems to govern susceptibility to inflammatory disease, but no study of the HLA complex has confirmed a link with the expected autoimmune haplotype (HLA A1, B8, DR3). Large studies have not found an association between lichen sclerosus and the class I HLA antigens, although smaller studies suggested some association. However, in a group of 84 female

Clinical management

The panel summarises the best approach to management. Information sheets for the patient and the general practitioner are helpful. The patient should be aware that as yet there is no cure for lichen sclerosus, but treatment can be offered to relieve symptoms. Long-term follow-up is advisable because of the small association with malignant disorders. Support groups are helpful. Patients are advised to wash with bland emollients (aqueous cream), to avoid topical irritants and occlusive clothing,

Conclusion

We hope that as knowledge of the immunogenetics of lichen sclerosus and the cellular and immunological abnormalities progress, researchers will be able to predict which patients are at risk of scarring and malignant disease. In time, specific targeted therapy may become a reality.

We thank Peter Millard (Oxford Radcliffe Hospitals) for figure 6.

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