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Lichen sclerosus

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lichen sclerosus

Perianal Lichen Sclerosus (LS)

Lichen sclerosus (LS) is a chronic inflammatory skin disease that primarily affects the anogenital areas. The disease causes a progressive change in the connective tissue of the skin, leading to inflammation, scarring, and loss of elasticity. Although LS is best known for affecting the external genitalia (vulva/penis), it is important to recognize that it can also involve the skin around the anus (perianal Lichen Sclerosus).

LS is a benign but chronic condition. Without proper and ongoing treatment, there is a risk of permanent anatomical changes and, in rare cases, the development of cell changes.

What causes Lichen Sclerosus?

The exact cause of lichen sclerosus is still being researched, but the strongest theory points to an autoimmune origin combined with other predisposing factors.

  • Autoimmune dysfunction (main cause): The body's immune system produces antibodies that mistakenly attack the delicate skin cells (fibroblasts and keratinocytes) in the affected areas. This leads to a chronic inflammatory cascade, resulting in the characteristic thickening, scarring, and atrophy (shrinkage) of the skin.
  • Hormonal connection: The disease is more common during periods of low estrogen levels (before puberty and after menopause in women), indicating a possible hormonal sensitivity in the tissue, although this is not the only cause.
  • Genetic predisposition: Clinical observations suggest that there may be a hereditary component, as the disease is sometimes seen in several members of the same family or in association with other autoimmune disorders.
  • Important: Not contagious: LS is not an infection and cannot be transmitted sexually or through ordinary contact under any circumstances.
 Symptoms of Perianal Lichen Sclerosus

The symptoms develop gradually. In the perianal area, the symptoms often have a major impact on the patient's quality of life.

Symptom Description and Consequence
Itching (Pruritus) The most bothersome symptom. The itching is often intense, burning, and typically worsens at night, which can disrupt sleep and daily functioning. Constant scratching can worsen the inflammation and cause further tears.
Pain and Discomfort Pain may occur during bowel movements (defecation) or at rest. This is often due to tightness of the skin, anal fissures (painful cracks), or secondary infection.
Characteristic Skin Changes The skin takes on a whitish, pale, parchment-like appearance and feels thin and wrinkled (atrophic). During active phases, redness and swelling (erythema) may be observed.
Callus Formation and Tightness The chronic inflammation leads to the formation of hard, fibrous scar tissue. In the perianal area, this can cause a loss of elasticity and a narrowing (stricture) of the rectal opening, which can lead to bowel movement problems.
Fissures and Sores Atrophic and tight skin is very fragile. Even slight stretching or drying can cause painful cracks and sores that are difficult to heal.
Diagnosis and assessment (The procedure)

An accurate diagnosis is essential in order to begin the right treatment and rule out other skin diseases.

  • Clinical examination: The diagnosis is initially made by a physician or surgeon with experience in anogenital skin diseases. The examination focuses on identifying the typical white, atrophic changes and scarring around the anus.
  • Tissue sample (biopsy) – Standard: To confirm the diagnosis and exclude premalignant (cell changes) or malignant changes, a biopsy is often necessary.
    • Procedure: A small piece of tissue is removed under local anesthesia and sent for microscopic analysis. This is a quick and minimally invasive procedure.
    • Purpose: The biopsy confirms the histopathological signs of LS and provides reassurance that it is not another, more serious condition.
Treatment of Perianal Lichen Sclerosus (The Strategy)

The goal of treatment is to reduce inflammation, restore skin elasticity, and minimize the risk of long-term complications. Treatment is lifelong and requires patient commitment.

1. Local steroid treatment (basic treatment)

  • Preparation: Strong steroid preparations (most often clobetasol propionate in ointment or cream form) are the most effective and primary treatment.
  • Mechanism of action: Steroids significantly suppress the autoimmune reaction in the skin, thereby reducing inflammation, itching, and scarring.
  • Treatment plan (example):
    • Initial Intensive Phase: Typically 1-2 times daily for a period of 4-12 weeks, until symptoms are fully controlled.
    • Maintenance phase: Once the skin has settled down, the dose is reduced to 1-2 times per week. This maintenance is critical to prevent relapse and scarring.

2. Complementary and Supportive Treatment

  • Emollients (barrier creams): The use of fatty creams or pure petroleum jelly is essential. These agents keep the skin supple and moisturized and act as a barrier that reduces irritation from stool and friction.
  • Calcineurin inhibitors: Creams such as Tacrolimus or Pimecrolimus can be used as an alternative to steroids, especially for maintenance treatment or if steroids are not tolerated/are not sufficiently effective.
  • Hygiene and Care:
    • Avoid irritants: Only use lukewarm water and mild bath oil in the area. Perfumed soaps, wet wipes, or harsh cleaning products should be avoided.
    • Gentle drying: Avoid rubbing the skin dry. Pat gently or use a hair dryer on a low heat setting.

3. Surgical Intervention (In Case of Complications)

Surgery is a rare exception and is only used if the disease has caused serious complications that cannot be resolved medically.

  • Treatment of stricture: If scarring has led to a significant narrowing of the rectal opening (anal stenosis), surgical widening (anoplasty/sphincterotomy) may be necessary to restore normal bowel function.
  • Removal of Cellular Changes: If repeated biopsies show persistent severe cellular changes (premalignant lesions), surgical removal of the affected tissue is necessary to prevent the development of squamous cell carcinoma.
Long-term perspective and self-care
  • Lifelong Control: LS is a chronic condition that requires lifelong attention and maintenance treatment. The goal is to keep the disease in remission and thereby achieve complete freedom from symptoms.
  • Regular Oncological Checkups: The increased, albeit small, risk of developing squamous cell carcinoma (skin cancer) in chronic LS-affected tissue means that regular checkups (typically annually) with your surgeon or dermatologist are mandatory —even when you are symptom-free.
  • Persistence pays off: Follow your doctor's instructions carefully. It may take months to see the full effect of steroid treatment, but persistence is the key to preventing lasting damage.

 

 

 

Reference list: Perianal Lichen Sclerosus
 
1. Pathophysiology and Epidemiology
  1. General Understanding of Lichen Sclerosus (Autoimmune and Chronic Nature):
    • Source: Kirtschig, G. (2016). Lichen Sclerosus—Looking Back and Moving Forward. Acta Dermato-Venereologica, 96(2), 149–156.
    • Link: https://pubmed.ncbi.nlm.nih.gov/26658925/
    • Supports: The description of LS as a chronic, inflammatory skin disease, the autoimmune hypothesis, and that it leads to loss of elasticity and scarring (atrophy/fibrosis).
  2. Lichen Sclerosus’ Connection to Other Autoimmune Diseases and Hormones:
    • Source: Kreuter, A. (2018). Lichen sclerosus. The Lancet, 391(10118), 350–358.
    • Link: https://pubmed.ncbi.nlm.nih.gov/28993188/
    • Supports: The theory of an autoimmune origin, possible genetic predisposition, and the observed hormonal connection (e.g., menopause).
2. Diagnosis and Symptoms
  1. Clinical Picture, Role of Biopsy, and Presentation in the Perianal Area:
    • Source: Meffert, J. J., & Crotty, K. (2020). Extragenital and anogenital lichen sclerosus: An update. Journal of the American Academy of Dermatology, 83(2), 335–343.
    • Link: https://pubmed.ncbi.nlm.nih.gov/31336087/
    • Supports: Symptoms (intense itching, pain, fissures) and characteristic skin changes (white, parchment-like appearance, scarring). The source also confirms the importance of a biopsy to confirm the diagnosis and rule out malignancy.
3. Treatment and Complications
  1. Local steroid treatment as first choice (clobetasol propionate):
    • Source: Kirtschig, G., Becker, K., Günthert, A., Jasaitiene, D., Neumann, C., Röcken, M., Rueff, F., & Stockfleth, E. (2015). S1 Guideline: Lichen sclerosus. Journal of the German Dermatological Society, 13(10), 1076–1092.
    • Link: https://pubmed.ncbi.nlm.nih.gov/26458564/
    • Supports: The treatment strategy that establishes strong local steroids (e.g., clobetasol propionate) as the primary and most effective treatment in both the intensive and maintenance phases.
  2. Risk of Cancer Development (Squamous Cell Carcinoma – SCC) and Control:
    • Source: Yesudian, P. D., & Sugunendran, H. (2019). Anogenital lichen sclerosus and its malignant potential. Current Dermatology Reports, 8(3), 195–203.
    • Link: https://pubmed.ncbi.nlm.nih.gov/32047879/
    • Supports: The claim of increased risk of developing squamous cell carcinoma in chronic LS tissue, necessitating regular oncological monitoring.
  3. Surgical Intervention for Complications (Stricture/Anal Stenosis):
    • Source: Al-Daraji, W., & Singh, N. (2015). Perianal lichen sclerosus: a brief overview and update. Pathology, 47(7), 676–680.
    • Link: https://pubmed.ncbi.nlm.nih.gov/26620953/
    • Supports: The necessity of surgical intervention in rare cases where scarring has led to a functionally limiting stricture (narrowing) that requires dilation.
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