Cheilitis Granulomatosis

What Is Chelitis Granulomatosis?

Cheilitis granulomatosis is a persistent idiopathic lip swelling due to granulomatous inflammation. When only the swelling of the lip is there, it is called cheilitis granulomatosis of Miescher. A triad of recurrent or persistent orofacial edema, plicated or fissured tongue, and relapsing facial nerve paralysis can also occur. It is called Melkersson- Rosenthal syndrome. It is a rare congenital anomaly and there is no sex predilection.

What Is the Etiology of Cheilitis Granulomatosis?

The etiology of cheilitis granulomatosis is unknown. However, several etiologies are proposed to cause the disease.

They include,

• Genetic predisposition.

• Immunologic factors include T helper1- mediated immune response.

• Allergic reaction to dental materials, foodstuffs, and food additives.

• Cancer or infection may result in the obstruction of lymphatics around the lips.

• Hypersensitivity to ultraviolet-B radiation.

• As part of the spectrum of Crohn's disease.

What Is the Prevalence of Cheilitis Granulomatosis?

Cheilitis granulomatosis is rare and can occur in all age groups. But the peak is reported to be between 20 to 40 years of age. It rarely affects children. It has an equal sex distribution.

What Are the Clinical Features of Cheilitis Granulomatosis?

The clinical features of this condition are highly variable. Miescher's Cheilitis granulomatosis is confined to the lip. The granulomas in other granulomatous cheilitis are widespread. One of the earliest manifestations of granulomatous cheilitis is sudden diffuse or nodular swellings of the lip or face. The episode goes away within hours or days in most cases. In some cases, the forehead, eyelids, or one side of the scalp can also be involved. Amber-colored vesicles are rarely noted. A fissured or plicated tongue is seen in 20 to 40 % of patients. In addition, they may develop edema, paresthesia, erosions, or taste alterations. Cobblestone appearance of buccal mucosa may also be seen. At first, the lip swelling may feel nontender, soft, firm, or nodular on touching. At each episode, the swelling may increase in size, become more persistent, and remain permanent. But later, the enlarged lip appears cracked and fissured with reddish-brown discoloration and scaling. Finally, the fissured lip becomes painful and obtains the consistency of firm rubber. The swelling regresses slowly after some years.

Other symptoms that may appear along with cheilitis granulomatosis include-

• Fever.

• Headache.

• Visual disturbances.

• Regional lymph nodes are enlarged in 50 % of patients.

• Facial palsy (weakness or paralysis of the facial muscles) of the lower motor neuron type occurs in 30 % of patients. This can be unilateral or bilateral, partial or complete. In addition, it can be intermittent and indicates progression to orofacial granulomatosis.

What Is the Histopathology of Cheilitis Granulomatosis?

The diagnosis can be made based on the clinical features, and histopathological examination is unnecessary. However, the histopathological features include granulomatous infiltration with epithelioid cells, multinucleated giant cells, lymphedema, and fibrosis. There can be an absence of some of these features, and their absence should not exclude the diagnosis of cheilitis granulomatosis.

How to Evaluate for Cheilitis Granulomatosis?

This disease is mainly diagnosed clinically. Histopathology is used for confirmation. Other investigations, such as high-resolution computed tomography scans of the chest and serum angiotensin-converting enzyme levels, are used to rule out sarcoidosis. Some allergens can cause the disease, and patch tests can diagnose them. Purified protein derivative protein tests and chest radiography can be used to help exclude tuberculosis.

Decreased iron, hemoglobin folate levels, and increased C-reactive protein are noted in orofacial granulomatosis.

Dental films may be used to assess for the presence of a chronic dental abscess.

Decreased iron, hemoglobin, ferritin, folate, elevated C-reactive protein, celiac antibodies, serum IgE and alkaline phosphatases are associated with orofacial granulomatosis.

A biopsy of the swollen lip or orofacial tissues shows only lymphoedema and perivascular lymphocytic infiltration during the early stages and later may only show granulomas. Therefore, it may be helpful to exclude Crohn's disease, sarcoidosis, and lymphoma.

Using investigations like colonoscopy or gastroduodenoscopy with biopsies in patients without gastrointestinal symptoms to eliminate the presence of Crohn's disease is also controversial.

What Is the Treatment for Cheilitis Granulomatosis?

The rarity of the disease and also the absence of knowledge concerning its etiology are the reason for the lack of adequate medical treatment for this disease. Therefore, the proposed treatments mainly focus on symptomatic approaches intended to avoid recurrences and improve the quality of their life.

If the condition is related to an allergy, the responsible allergic components or causative substances should be avoided long-term. If it is due to an underlying disease, systemic treatment can reduce the swelling of the lips.

Corticosteroid therapy is the classic treatment for this condition. It can be administered locally, topically, or intralesionally. Intralesional corticosteroids need to be repeated every few months. At times, systemic corticosteroids can be given for short courses. Unfortunately, despite the best treatment, recurrence is very common.

Anti-inflammatory or immunomodulatory effects of Tacrolimus, oral Clofazimine, Thalidomide, Dapsone, and Doxycycline have only inconsistent results.

Immunosuppressive treatments with drugs such as Mycophenolate mofetil and Azathioprine have inconsistent positive results.

Fumaric acid has good results in 50 % of the cases.

Surgical reduction, such as cheiloplasty reduction (lip reduction surgery), is recommended only after the lesions are fixed and are not evolving. It is reserved for those cases that are resistant and complicated by a significant lip deformation.

What Is the Differential Diagnosis of Cheilitis Granulomatosis?

The differential diagnosis of Cheilitis Granulomatosis may include-

• Acquired or hereditary angioedema.

• Glandular cheilitis.

• Dental abscess.

• Crohn's disease.

• Facial Palsy

• Cutaneous manifestations of various granulomatous disorders include sarcoidosis, tuberculosis, leishmaniasis, leprosy, and Wegener's granulomatosis.

The histopathology of angioedema is different. Differentiation from other disorders has already been discussed above.

Conclusion

Cheilitis granulomatosis is a rare disorder, and the etiopathogenesis remains obscure; therefore, the treatment remains uncertain. The patients should be counseled about the possibility of this condition, later representing Melkersson-Rosenthal syndrome also. They must be cautioned to report if facial paresis develops later in life. Proper explanations of the availability of the limited therapeutic options are also important.