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Lichen Sclerosus - Causes, Symptoms, Diagnosis, Treatment, and Complications

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Lichen sclerosus is a rare autoimmune disorder that causes skin shrinkage and hypopigmentation.

Medically reviewed by

Dr. V. Srikanth Reddy

Published At January 12, 2024
Reviewed AtJanuary 12, 2024

What Is Lichen Sclerosus?

Skin conditions such as lichen sclerosus can affect the vulva (the region outside the vagina), anus, or penis. The outer layer of the skin becomes thin, red, itchy, and discolored. Blisters and sores may develop (typically due to continuous itching). These symptoms hardly affect other bodily parts. If left untreated, lichen sclerosus can leave scars that make it uncomfortable or difficult to pee, have a bowel movement, or have intercourse. Although lichen sclerosus has no known treatment, its symptoms can be managed. Although medical professionals can treat symptoms, they could reappear later. If lichen sclerosus is not treated, it raises the likelihood of getting squamous cell carcinoma, a kind of skin cancer.

What Causes Lichen Sclerosus?

Although the exact etiology of lichen sclerosus is unknown, potential causes include hormonal, irritating, traumatic, hereditary, and viral factors. Lichen sclerosus is an autoimmune condition. Antibodies against a particular protein are linked to autoimmune disorders. In 60 to 80 % of women with vulval lichen sclerosus, antibodies to extracellular matrix protein-1 (ECM-1) have been found. Other cases might be explained by antibodies to unidentified proteins, explaining why lichen sclerosus presents differently in some instances and how it responds to therapy. These antibodies, however, could be epigenetic, meaning that they are the product of illness rather than its cause. In guys who got circumcised as infants, male genital lichen sclerosus is uncommon. Urine occlusion under the foreskin has been hypothesized to be the source of the injury, which may occur periodically throughout time.

What Are the Symptoms of Lichen Sclerosus?

The symptoms of lichen sclerosus are:

  • Ulcers and Sores: If scratched, there is a risk of developing blisters, fissures, or cracks in the genital area. Even the slightest contact can cause the skin to bleed, bruise, or split.

  • Scarring: Untreated lichen sclerosus can cause scarring around the vulva and cause the clitoral hood to seem flat, changing the appearance of the anatomy. The penis or anus might be scarred. Scarring can result in painful sex (dyspareunia) and issues with urinating or passing gas.

The neck, wrists, hips, upper back, and shoulder are usually affected. Clinical signs of oral mucosa involvement include bluish-white papules on the buccal mucosa or under the tongue. Male genital LS (both boys and men) manifests itself in the foreskin, glans penis, and coronal sulcus penis. Extragenital lesions can appear elsewhere on the skin and are generally asymptomatic.

What Is the Diagnosis of Lichen Sclerosus?

Lichen sclerosus is often diagnosed based only on clinical appearance by a skilled practitioner. More insights can be obtained using dermoscopy. Skin biopsies are often advised. Histopathology might support the lichen sclerosus diagnosis that has been suggested. Lichen sclerosus may be diagnosed with or coexist with another skin disorder. A biopsy may be performed on a focused location to check for SIL or malignancy. During a follow-up, a biopsy could also be advised to assess problems or explain the treatment's insufficient effectiveness.

What Is the Treatment for Lichen Sclerosus?

Patients with lichen sclerosus should see a specialist who specializes in the ailment for an accurate diagnosis and treatment suggestions. They should get familiar with the position and look of their lichen sclerosus. When using topical treatment, women should utilize a mirror. Photographs may aid in the monitoring of activities and therapy.

General Measures that Need to Be Followed Are:

  • Once or twice a day, gently wash the hands.

  • If necessary, use a non-soap cleaner.

  • Avoid wearing tight garments, rubbing, and itching.

  • Activities like riding a bike might increase symptoms.

  • Seek medical help and treatment.

  • Emollients can be used to reduce dryness and irritation, as well as to function as a barrier to protect delicate skin in the vaginal and anal regions from urine and feces.

Topical Treatment:

  • In postmenopausal women, use intravaginal estrogen cream or pessaries. These alleviate the symptoms of atrophic vulvovaginitis.

  • Instead of or in place of topical steroids, use tacrolimus ointment and pimecrolimus cream as calcineurin inhibitors. They usually produce searing pain (at least for the first several days). There are early concerns that these drugs may hasten cancer formation.

  • Topical retinoid is not well tolerated on vaginal skin but may be used on other lichen sclerosus-affected areas. It helps to minimize scaling and dryness.

Surgery:

  • For high-grade squamous intraepithelial lesions or malignancies, surgery is required.

  • Circumcision is helpful for males with lichen sclerosus of the prepuce and glans of the penis. If topical steroids alone have not reduced symptoms and signs, this procedure should be performed as soon as possible. Reconstructive surgery may be required if the urethra is stenosed or scarred.

  • Suppose dilators have not been beneficial in reducing urinary issues and allowing intercourse in females. In that case, the removal of vulval and vaginal adhesions and scarring from vulval lichen sclerosus may be performed on occasion.

Among the procedures are:

  1. Simple perineotomy (adhesion division).

  2. Fenton technique (transversely repaired incision).

  3. Perineoplasty (excision of affected tissue and advancement of vaginal mucosa).

  4. Unfortunately, lichen sclerosus can block the vaginal opening again after surgery appears to be effective.

Oral Antibiotics:

Systemic treatment is infrequently used when lichen sclerosus is severe, acute, and not responsive to topical therapy.

Among the options are:

  • Corticosteroids, intralesional, or systemic.

  • Oral retinoids include Acitretin, Isotretinoin, Methotrexate, and Cyclosporin.

What Are the Complications of Lichen Sclerosus?

The complications of lichen sclerosus are:

  • LS is a continuously relapsing condition that, if left untreated, can result in scarring and a total loss of conventional vulvar architecture in women, including introital stenosis, fusion and resorption of the labia minora, and urethral strictures in men. Tissue adhesion and sclerosis cause ripping and, as a result, loss of sexual function and dysuria, constipation, itchiness, and discomfort.

  • Furthermore, vulvar lichen sclerosis may progress to vulvar squamous cell carcinoma in the afflicted area, with a risk of up to 5 percent; however, its relationship with penile SCC is unknown.

  • This probability of transformation is not connected with extragenital lesions. There have been reports of melanoma and basal cell cancer.

Conclusion

LS is a mucocutaneous autoimmune condition characterized by skin shrinkage and hypopigmentation. Women are more likely than men to have LS. In children, the prognosis for genital LS is favorable. Occasionally, the illness will resolve on its own. LS is typically responsive to local corticosteroid therapy. Early identification and treatment may help to avoid the consequences of illness.

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Dr. V. Srikanth Reddy
Dr. V. Srikanth Reddy

Dermatology

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