Written by: Joanne Byron, BS, LPN, CCA, CHA, CHCO, CHBS, CHCM, CIFHA, CMDP, COCAS, CORCM, OHCC, ICDCT-CM/PCS
This article is written for all those people Joanne knows suffering from this autoimmune disease. It is painful and life-altering in many ways. 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 “Lichen Planus”?
Lichen planus (LP) is an autoimmune disease presenting as an inflammatory skin condition that can affect the hair, mucous membranes, nails and skin. It can be painful and embarrassing with symptoms that can exacerbate easily.
Lichen planus occurs when the immune system attacks cells of the skin or mucous membranes. It's not clear why this abnormal immune response happens. The condition is NOT contagious.
People may complain about bleeding and swollen gums. The dentist, after an oral exam, may see lesions prompting biopsy. Oral lichen planus may predispose to the development of squamous cell carcinoma within lesions.
Other presentations may be lesions on the scalp with hair loss in that area. LP of the skin can present with various characteristics such as purplish, flat bumps, most often on the inner forearm, wrist or ankle, and sometimes the genitals.
Additional signs and symptoms are itching, blisters that break to form scabs or crusts, lacy white patches in the mouth or on the lips or tongue, painful sores in the mouth or vagina, hair loss, change in scalp color, nail damage or loss. There are four forms of oral lichen planus:
- Bullous; and
Coding Lichen Planus
ICD-10-CM categorizes LP in Chapter 12: Diseases of the Skin and Subcutaneous Tissue. Category L43 is Lichen Planus and codes within the L43 range have an Exclude 1 note: lichen planopilaris (L66.1).
L43.0 Hypertrophic lichen planus
L43.1 Bullous lichen planus
L43.2 Lichenoid drug reaction
L43.3 Subacute (active) lichen planus
L43.8 Other lichen planus
L43.9 Lichen planus, unspecified
Subtypes of lichen planus include actinic, annular, atrophic, eruptive, follicular, hypertrophic, inverse, linear, palmoplantar, pemphigoides, pigmentosus, ulcerative, vesiculobullous and vulvovaginal.
According to the National Organization for Rare Disorders or “NORD,” it is a rare, chronic, inflammatory autoimmune skin and mucous membrane disease. Between 50 and 70 percent of patients show symptoms involving the mucous membranes of the mouth, vagina and esophagus.
LP on mucous membranes can present as red, painful sores, or lesions that have a net-like, white pattern.
- Oral symptoms often occur before skin lesions develop.
- Oral symptoms, consisting of a dryness and metallic taste or burning in the mouth, may appear first and may be the only evidence of the disease.
Anyone can develop lichen planus. But the condition most often affects middle-aged adults. Oral lichen planus most often affects middle-aged women.
What Autoimmune Disease Causes Lichen Planus?
Lichen planus (LP) is thought to be caused by a T cell–mediated autoimmune reaction against basal epithelial keratinocytes in people with genetic predisposition. Other factors include antigen-presenting cells, adhesion molecules and inflammatory cytokines.
According to a study by the National Institutes of Health, vitamin D deficiency results in impaired T cell proliferation and may therefore lead to the development of oral lichen planus (OLP).
What Can Trigger Lichen Planus?
The trigger of lichen planus is a hyperactive immune system. This condition occurs when the immune system begins to attack mucous membrane or skin cells which are not actually a threat to your body. This is an idiopathic condition, meaning there is no precisely known cause. However, medical professionals are aware of several conditions that may trigger it. With the COVID-19 pandemic, many LP patients complain about severe exacerbation of their disease following each COVID-19 vaccine or booster. This is a rare case of new‐onset lichen planus arising after the COVID‐19 vaccination. The vaccination induces a Th1 cell response and a subsequent various cytokines secretion that may play a key role in the development of this condition. The exact pathogenesis is yet to be uncovered, as stated in the National Library of Medicine article July 2021 “New‐onset cutaneous lichen planus triggered by COVID‐19 vaccination.”
Other factors which can influence a flare-up and trigger an exacerbation are:
- Flu vaccinations
- Hepatitis C infection
- Heart and joint medications
- Certain metals, chemicals and pigments
This classic presentation is known as Ps of LP:
- Planar (flat-topped);
- Purple Papules; and
Some patients may be asymptomatic; however, most people experience intense pruritus, a hallmark of lichen planus.
Arriving at a Diagnosis of Lichen Planus
A diagnosis of lichen planus is reached on the basis of the typical clinical appearance and a punch biopsy of the lesion. Lesions can appear to present as other diseases, such as lupus erythematosus or carcinoma. When healthcare practitioners encounter lichen planus, the patient typically should be referred to the dermatologist for definitive management or other specialist experienced in the treatment of this disease.
- Cutaneous LP typically clears spontaneously within 1 to 2 years, so treatment is aimed at reducing pruritus and time to resolution.
- Many drugs are used to treat the skin manifestations with not much difference among them in effectiveness.
- Oral LP may spontaneously resolve within 5 years, but many cases are chronic and never resolve.
- Vulvovaginal LP can render intercourse impossible or extremely painful. Prescribing the wrong topical treatment can cause more harm than good.
Lichen planus is a rare disease and is a difficult disorder to cure, as stated in a May 1, 2022 article “Lichen Planus” posted to the National Library of Medicine. Other scientific references indicate that there is no cure. Patients suffering from this condition find few options for support. 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 where you may want to refer your patients to engage with others.