Written by: Joanne Byron, BS, LPN, CCA, CHA, CHCO, CHBS, CHCM, CIFHA, CMDP, COCAS, CORCM, OHCC, ICDCT-CM/PCS
This article is written as “Part 2” of the previous article “The Pain of Lichen Planus." After research, we found that many practitioners, coders and billers know little about this disease. We hope this article will help practitioners document and code this condition correctly and direct patients with chronic LP for emotional support and refer appropriately to more experienced specialists for treatment.
What Is the Difference Between Lichen Planus and Lichen Sclerosus?
Lichen planus and lichen sclerosus are both idiopathic conditions that affect your skin, especially mucus membranes. The difference between lichen planus and lichen sclerosus is that lichen sclerosus rarely affects the mucous membranes in your mouth.
Lichen Sclerosus et Atrophicus
Lichen sclerosus et atrophicus is an acquired chronic inflammatory dermatosis commonly affecting the vulvar and perianal regions. It is associated with an increased risk of vulvar cancer even though it is not a premalignant condition itself. It is a rare skin condition characterized by white, blotchy patches which often lead to scarring and tightening of the skin around the genitals and anus.
It is a chronic disease, so once it develops patient’s often have it the rest of their lives. People with white, brown or black skin can get lichen sclerosus.
The etiology of LS remains unknown. According to the National Library of Medicine’s 2020 publication Etiology, Clinical Features, and Diagnosis of Vulvar Lichen Sclerosus: A Scoping Review, several mechanisms have been studied such as: Genetic predisposition; autoimmune disease; Koebner phenomenon; hormones(low estrogen levels).
Lichen sclerosus is not contagious and cannot be spread through sexual intercourse.
Females are six times more likely to be affected than males. However, it can develop in people of any age, including infants and children. “It’s relatively rare—its exact prevalence is not known but estimated between 1 in 300 and 1 in 1000 individuals,” says Dr. Amanda Zubek, a Yale Medicine dermatologist. “When caught early, prompt treatment can prevent a lifetime of debilitating itch and painful genital scarring.”
Women between the ages of 40 and 60 are mostly affected and the disease is also called “lichen sclerosus et atrophicus.” When this condition is found in males, the disease is known as balanitis xerotica obliterans.
Fissures, cracks, and purplish patches (ecchymoses) appear frequently. The earliest areas of lichen sclerosus exhibit a porcelain white appearing center surrounded by redness. This grows together to form larger areas of lichen sclerosus.
Areas that are prone to rubbing and friction can develop blisters or bruising. The long-term results of lichen sclerosus are areas of shiny, thin skin that have a tendency to be dry, crack, or bleed. Patients may present with symptoms such as:
- Painful intercourse
- Smooth, white patches on the affected area
- Blotchy, wrinkled patches
- Tearing, bleeding, blistering or ulcerated sores
- Discomfort and pain
- Itching (pruritus), which can be severe
Vulvar lichen sclerosus (VLS) is a chronic inflammatory disorder that affects women of all ages. Vulvar lichen sclerosus requires aggressive treatment and lifelong monitoring to avert scarring and minimize risk of squamous cell carcinoma.
The exact cause of lichen sclerosus is not known. Most research indicates it is an autoimmune condition. Autoimmune disorders arise when the body’s natural defenses against “foreign” or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons. Some cases of lichen sclerosus may be linked to formation of certain antibodies (e.g., a thyroid protein (thyroglobulin) or certain cells that line the walls of organs).
Diagnosing This Condition
Lichen sclerosus is likely not recognized by many practitioners since it is a rare disease. It is typically diagnosed by looking at the skin of the affected area. For those suffering from the symptoms of lichen sclerosus, a thorough clinical evaluation, identification of characteristic physical features, and a detailed patient history should be conducted. Providers should focus on the functional impact of the lichen sclerosus and treatment to date, including over the counter products that might be applied. In order to be sure of the diagnosis, a skin biopsy may be needed. Biopsies may also be performed if squamous cell carcinoma is suspected.
Coding of Lichen Sclerosus et Atrophicus
Lichen sclerosus et atrophicus
Excludes 2: lichen sclerosus of external female genital organs (N90.4)
Upon examination, leukoplakia may be evident. Usual symptoms of leukoplakia are pruritus, burning, or stinging of the vulva, white or grey patches of thickening or thinning on the vulvar skin. Females would be assigned code N90.4 and males N48.0.
Leukoplakia of vulva
Dystrophy of vulva
Kraurosis of vulva
Lichen sclerosus of external female genital organs
Leukoplakia of penis
Balanitis xerotica obliterans
Kraurosis of penis
Lichen sclerosus of external male genital organs
Excludes 1: carcinoma in situ of penis (D07.4)
Vaginal Lichen Planus
“Lichen planus is a multisystem disease. Often genital involvement is missed or misdiagnosed. It can be rapidly progressive with high patient morbidity. This case highlights the importance of a multidisciplinary approach and the effectiveness of combined surgical and medical treatment with close patient follow-up and support.” – a quote from the National Library of Medicine article “Vaginal lichen planus: preservation of sexual function in severe disease.”
In lichen planus, your body’s immune system attacks parts of your body instead of protecting your body from foreign invaders like bacteria or viruses. In a healthy immune system, special cells in it called T cells help protect your body from infection. In people with lichen planus, their immune system’s T cells attack a protein in their skin and mucus membranes. No one knows why the T cells attack the protein.
Some mental/emotional signs of stress that can exacerbate vaginal lichen planus symptoms include:
- Difficulty relaxing
- Use of alcohol, tobacco or drugs to relax
- A negative opinion of self
- Feeling overwhelmed
- Difficulty concentrating
Mucosal lichen planus of the vulva is a rare but increasingly recognized condition. It has potentially severe complications such as fusion of the labia and vagina and the risk of developing squamous cell carcinoma (SCC) may be increased. An association between hepatitis B and C infection and skin or oral lichen planus appears to exist in certain geographical areas, according to the National Library of Medicine article “Mucosal vulval lichen planus: outcome, clinical and laboratory features.”
Three types of lichen planus (LP) occur on the vulva:
- Classic; and
Coding Vaginal Lichen Planus
When coding vulvar LP, review documentation to determine appropriate code selection. The following codes can be considered to report these conditions:
L43.0 Hypertrophic lichen planus
L43.8 Other lichen planus
L43.9 Lichen planus, unspecified
Patients suffering from this condition find few options for support. Genital conditions are often embarrassing for the patient to mention to their practitioner. When the patient has oral LP, get a thorough history and ask about genital symptoms, which may help the patient reveal important clinical information for further investigation and help.
There is a website where patients can share “what works” and give support to one another. Check out https://www.stuffthatworks.health/lichen-planus to see if this is a site you may want to refer your patients to in order engage with others.