Plasma Cell Cheilitis of Lips: Causes, Diagnosis, and Management

Introduction

Plasma cell cheilitis (PCC) is a rare condition that affects the lips inside the mouth. More specifically it affects the inner lining of the lower lip or labial oral mucosa. PCC is sometimes referred to by other names like plasmacytosis circumorificialis and plasmacytosis mucosae. All these terms describe the same condition. The article talks about the causes, features, diagnosis, and management of the condition.

What Is Plasma Cell Cheilitis?

Plasma cell cheilitis, or PCC, is an unusual mouth problem that comes under a category called plasma cell mucositis, which involves certain cells in the mouth lining. However, diagnosing it can be difficult because it can look like other lip issues during a regular dental checkup.

In PCC, the specific signs are seen on the inner side of the lower lip, especially in the labial oral mucosa. It often appears as distinct plaques or patches. This condition is more common in older people, and they usually notice symptoms like lip bleeding, crusting, or having a red, inflamed lip. Sometimes, it can lead to mouth sores that are painful, cracked, or easily worn down on the labial mucosa, and have a pricking sensation.

To confirm the condition, oral pathologists do a microscopic examination. They check for plasma cells clumped together, which is a clear sign of PCC. PCC does not only show up in the mouth but also in different parts of the body, like the cheeks (buccal mucosa), the roof of the mouth (palate), the tongue, the flap that covers the windpipe (epiglottis), the voice box (larynx), and even in other sensitive areas like the genitals (vulva). PCC can develop in various soft tissues, especially around openings in the body. In the past, a group of these orifice-related conditions was collectively called plasma cell orificial mucositis.

What Are the Causes of Plasma Cell Cheilitis?

Plasma Cell Cheilitis (PCC) often affects older adults, especially men more than women. The exact cause of PCC remains unknown. However, researchers have come up with a couple of theories. One theory suggests that inflammatory cells like T cells or macrophages might disrupt the growth or behavior of B cells in the affected area, leading to PCC. Another theory proposes that PCC could develop in response to external factors, such as local lip injuries or damage from exposure to sunlight on the skin.

Several factors might contribute to the development of this condition including:

• Habitual Biting: Engaging in behaviors like frequent lip biting or chewing gum can increase the risk of developing PCC.

• Denture-Related Irritation: In elderly patients who wear dentures, consistent contact between the denture surface and the lip can lead to mechanical friction and irritation, potentially contributing to PCC.

• Foreign Bodies: Frequent exposure to foreign substances like candies, gummy materials, or abrasive toothpaste may play a role in the development of PCC.

• Underlying Health Conditions: Certain systemic diseases, such as hypertension (high blood pressure) and metabolic disorders, could be associated with PCC. However, the exact relationship between these conditions and PCC is still being studied.

• Sunlight Exposure: Some studies have suggested that exposure to sunlight, especially on the lower lip, might be a significant factor in the development of PCC. This is similar to another condition called actinic cheilitis, where chronic sun exposure is believed to be a primary cause.

What Are the Tissue Changes in Plasma Cell Cheilitis?

• In microscopic examination of the tissue, PCC shows an increase in mature plasma cells. These cells are scattered throughout the tissue, often forming dense, band-like clusters just beneath the surface.

• A distinctive feature that pathologists look for when diagnosing PCC is specific changes in the tissue. They might observe things like dyskeratosis (abnormal cell changes), swelling between cells, erosion, ulcers, areas where the outer layer of cells is too thick (hyperkeratosis), and damage to cell junctions in the tissue.

• To ensure a proper diagnosis and treatment plan oral pathologists, dentists or oral and maxillofacial surgeons work together and compare these tissue changes with the patient's clinical symptoms.

What Is the Differential Diagnosis of Plasma Cell Cheilitis of Lips?

Identifying Plasma Cell Cheilitis (PCC) can sometimes be challenging because it shares similar clinical characteristics with various other oral lesions. Dentists and oral surgeons might initially misdiagnose or confuse PCC with these conditions. A definitive diagnosis often requires a histopathological correlation performed by an oral pathologist. Following are some of the potential conditions that may resemble PCC:

Actinic Cheilitis:

• Actinic cheilitis is a lip condition characterized by chronic sun damage, causing dryness, and cracking, and potentially leading to precancerous changes.

• Actinic cheilitis and PCC can both cause lip problems, especially on the lower lip. They share characteristics like redness and swelling.

• Actinic cheilitis is primarily associated with sun damage, while PCC may have different causes. Histopathological examination can help differentiate between them.

Allergic Contact Cheilitis:

• Allergic contact cheilitis is a lip inflammation caused by an allergic reaction to substances that come in contact with the lips, such as cosmetics or certain foods.

• Both PCC and allergic contact cheilitis can lead to lip inflammation and discomfort.

• Allergic contact cheilitis is triggered by an allergic reaction to a substance that comes in contact with the lips, while PCC is more complex and not primarily allergy-related.

Exfoliative Cheilitis:

• Exfoliative cheilitis is a lip disorder where the skin on the lips becomes excessively dry, leading to peeling and discomfort.

• Both conditions can result in peeling or exfoliation of the lip skin.

• Exfoliative cheilitis is characterized by persistent peeling of the lip, while PCC may present with specific tissue changes that can be identified through histopathology.

Oral Lichen Planus:

• Oral lichen planus is a chronic inflammatory condition that affects the oral mucosa, causing white, lace-like patterns, and discomfort in the mouth.

• Oral lichen planus and PCC can both affect the lips and cause discomfort.

• Oral lichen planus typically shows specific clinical patterns and often requires a biopsy for definitive diagnosis. PCC may have distinct histopathological features.

Granulomatous Cheilitis:

• Granulomatous cheilitis is a lip inflammation characterized by the formation of granulomas (small nodules) and swelling.

• Both conditions can lead to lip swelling and inflammation.

• Granulomatous cheilitis is characterized by the presence of granulomas (small nodules) in the lip tissue, while PCC may exhibit different cellular changes under the microscope.

Squamous Cell Carcinoma of the Lip (Lip Cancers):

• Squamous cell carcinoma of the lip refers to cancerous growths on the lip, often characterized by persistent sores, lumps, or changes in lip color that require medical attention.

• PCC and lip cancer can both manifest as lip lesions, potentially causing bleeding or changes in appearance.

• Squamous cell carcinoma is a type of cancer, while PCC is not cancerous. Biopsy and further testing are necessary to distinguish between the two.

How to Manage Plasma Cell Cheilitis of Lips?

Managing Plasma Cell Cheilitis (PCC) involves various approaches to alleviate symptoms and eliminate the condition. The following are the key aspects of PCC management:

Diagnosis and Confirmation:

• Biopsy: A biopsy is often necessary to confirm the diagnosis. Dentists and pathologists should consider PCC as a possibility when evaluating lip lesions.

• Immunohistochemical Analysis: In some cases, immunohistochemical analysis may be used to confirm the diagnosis by examining plasma cell production.

Treatment Options:

Various therapeutic and surgical approaches have been proposed by dental and oral pathology specialists for managing PCC:

• Surgical Excision: This is the primary treatment strategy for PCC, involving the removal of affected tissue.

• Radiation Therapy: In some cases, radiation therapy may be used in conjunction with surgical excision.

• Electrocauterization: Lesions can be treated by cauterizing (burning) them using an electrical current.

• Cryotherapy: This involves freezing the lesions to remove them.

• Topical Fusidic Acid: The application of fusidic acid directly to PCC lesions may be considered by oral surgeons.

• Systemic/Topical Corticosteroid Therapies: Corticosteroids, either applied topically or administered systemically, can be used to reduce inflammation and manage symptoms. Some cases have reported partial success with topical immunomodulatory agents like tacrolimus or pimecrolimus.

Corticosteroids and immunomodulatory agents can provide relief, but they may not be the primary treatment choice for many oral surgeons. Surgical excision, radiation therapy, laser therapy, electrocauterization, or cryosurgery are often preferred because they offer more comprehensive and advanced approaches to effectively manage and eliminate PCC lesions.

The choice of treatment depends on the severity of the condition and the patient's specific needs. Patients need to consult with their oral surgeon or dentist to determine the most suitable treatment plan based on their individual needs and the characteristics of their PCC lesions.

Conclusion

Plasma Cell Cheilitis (PCC), characterized by ulcerative oral lesions on the lip, is generally considered a benign disorder. While diagnosing it can be quite challenging due to its resemblance to other oral conditions. Unlike many other oral lesions, PCC rarely progresses to malignancy or cancer. PCC needs timely diagnosis and appropriate management by an oral surgeon.