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Editors
Lichen Sclerosus
General features
- A chronic, intrinsic disease of the skin and mucosa that is based on an autoimmune process.
- It has previously been called lichen sclerosus et atrophicus (LSA), krauroris vulvae (in the genital area in women) and balanitis xerotica obliterans (BXO, in the penile area).
- The disease most often occurs in the genital area, more rarely in the perianal area and very rarely also in other places on the skin (extragenital form of the disease).
- It may occur in any age group; there are two peak incidences, in children below school age and in middle-aged adults.
- In children, the disease often heals by teenage.
- In adults, it is more chronic, but permanent remission is still possible.
- The disease may impair the quality of life, causing significant psychosocial suffering and genital functional problems.
- Patients often also have other autoimmune diseases (such as autoimmune thyroiditis Chronic Autoimmune Thyroiditis, megaloblastic anaemia Megaloblastic Anaemia or alopecia areata Hair Loss and Balding).
Symptoms
- There are three different clinical pictures.
Vulvar lichen sclerosus
- White, shiny patches or plaques, where the skin or the mucosa may be atrophic, thickened or wrinkled. There are often punctate or linear haematomas (Image 1).
- Typically in hairless areas of the vulva (labia minora, clitoris, urethral orifice)
- May be locally confined or extensively spread in the vulvar area (labia majora, perineum) or perianally. Does not occur on the vaginal mucosa.
- Symptoms: itching, pain, ulceration and haematomas, dyspareunia, difficulty urinating
- Pain in the genital area on defecation may lead to constipation. In children, constipation is a common primary symptom.
- Untreated, the disease may cause vulvar scarring and constriction (dyspareunia Dyspareunia in Women, decreased sexual desire).
Penile lichen sclerosus
- White, thickening and scarring patches or plaques on the foreskin and/or the glans
- Typically at the end of the penis, at the fold of the foreskin (Images 2 3 4 5). May cause scarring of the urethral orifice.
- Symptoms: pain on erection, itching, ulceration and haematomas, decreased urine stream
- Untreated may cause difficulty urinating, phimosis (the foreskin can no longer be retracted), erection problems, decreased sexual desire.
Extragenital lichen sclerosus
- Lichen sclerosus lesions (white, shiny, asymptomatic plaques) outside the genital area.
- Typical sites: thighs, buttocks, lower abdomen, beneath the breasts, shoulders and upper back, antecubital fossae
- Individual plaques often look wrinkled and dry.
- Haematomas occur more easily on the plaque areas.
- Rare
- Occurs in approx. 10% of patients with genital lichen sclerosus but may also occur independently.
Differential diagnosis
- Genital area
- Extragenital lesions
Workup
- Clinical diagnosis is often sufficient.
- As the histopathology of lichen sclerosus is often diagnostic, in unclear cases the diagnosis can be confirmed by skin biopsy.
- Skin biopsy is usually not necessary in children.
- If a poorly healing ulcer or raised nodular lesions appear on a chronic genital lichen sclerosus lesion, skin biopsy is recommended to exclude malignancy.
- Slowly healing traumatic haematomas frequently appear on lichen sclerosus lesions. They do not signify malignancy.
- In certain cases (if clinical suspicion arises), the following should be examined to exclude other autoimmune diseases: TSH, free T4, TPOAb, complete blood count, transcobalamin II-bound vitamin B12, antinuclear antibodies.
Treatment
Adults
- Intermittent treatment (3-6 months) with a (potent or) very potent glucocorticoid ointment until the symptoms subside
- The treatment can, for example, start with 2-week courses of treatment with 2-week breaks in between, followed by maintenance treatment once or twice weekly.
- The following regimen is frequently used:
- A very potent glucocorticoid ointment every night for 1 month, then every other day for 1 month and then twice weekly for 1 month
- An ointment (unguent) is usually better tolerated in the genital area than a cream.
- An assessment should be performed every 3-6 months to see whether the disease has subsided. If the symptoms recur, treatment should be resumed.
- In milder cases, shorter courses or less frequent application can be used.
- Maintenance treatment is often necessary to control the symptoms and to prevent scarring, e.g. a potent or very potent glucocorticoid ointment once or twice weekly.
- Calcineurin inhibitor ointments can also be tried in courses and/or for maintenance treatment (in courses of 3-6 months).
- Response to treatment can be assessed based on the clinical picture and the reduction of symptoms. Lightening of the skin and any scars that have developed may prove to be permanent.
Children
- Treatment is fairly similar to that in adults.
- Response can often be achieved with potent glucocorticoid ointments used in courses of 1-3 months, gradually spacing out the treatment.
- Courses should be repeated, as necessary.
- Maintenance treatment once or twice weekly, as necessary
- Calcineurin inhibitor ointments can also be considered as a therapeutic option.
Things to consider in treatment
- Lichen sclerosus may predispose the patient to a yeast infection causing vulvovaginitis Vulvovaginitis, which should be treated, depending on the symptoms, with courses of topical or systemic antifungal medication. Some patients may need prophylactic treatment (such as fluconazole once a week in doses of either 150 mg or defined by the patient's weight).
- Potential adverse effects of glucocorticoid ointments: haematomas, striae, erythematous skin atrophy, erythematous papules or pustules, recurrent vulvar yeast infections
- A good rule of thumb for safe treatment: after initial intensive treatment, at a quiet stage (maintenance treatment or treatment “as necessary”) a 30-g tube (of very potent or potent glucocorticoid ointment) should be enough for 3 months in an adult and 6 months in a child.
- In penile lichen sclerosus that is resistant to treatment, symptoms usually subside after circumcision.
- In addition to a medicated ointment, the following may be useful:
- reducing irritating factors (such as tight underwear or mechanical irritation)
- washing with water only or using mild washing liquids
- regular use of skin oil or a non-medicated emollient ointment
- treatment of incontinence and maceration, as necessary Inguinal and Genital Skin Problems.
Specialist consultation
- In severe cases and cases resistant to treatment, a specialist (dermatologist, gynaecologist, urologist) with expertise in the disease should be consulted.
- In cases of refractory penile disease and difficulty urinating, a urologist should be consulted (circumcision).
- Specialized care should be consulted, as necessary, if scarring causes permanent functional defects, such as dyspareunia, in a woman (gynaecological operative treatment).
References
- Lewis FM, Tatnall FM, Velangi SS et al. British Association of Dermatologists guidelines for the management of lichen sclerosus, 2018. Br J Dermatol 2018;178(4):839-853. [PubMed]
- Kirtschig G, Cooper S, Aberer W et al. Evidence-based (S3) Guideline on (anogenital) Lichen sclerosus. J Eur Acad Dermatol Venereol 2017;31(2):e81-e83. [PubMed]
- Pergialiotis V, Bellos I, Biliou EC et al. An arm-based network meta-analysis on treatments for vulvar lichen sclerosus and a call for development of core outcome sets. Am J Obstet Gynecol 2020;222(6):542-550.e6. [PubMed]
- Balakirski G, Grothaus J, Altengarten J et al. Paediatric lichen sclerosus: a systematic review of 4516 cases. Br J Dermatol 2020;182(1):231-233. [PubMed]
- van der Meijden WI, Boffa MJ, Ter Harmsel WA et al. 2016 European guideline for the management of vulval conditions. J Eur Acad Dermatol Venereol 2017;31(6):925-941. [PubMed]
- Morrel B, van Eersel R, Burger CW et al. The long-term clinical consequences of juvenile vulvar lichen sclerosus: A systematic review. J Am Acad Dermatol 2020;82(2):469-477. [PubMed]
- Halonen P, Jakobsson M, Heikinheimo O et al. Lichen sclerosus and risk of cancer. Int J Cancer 2017;140(9):1998-2002. [PubMed]