What is Cheilitis Granulomatosa (Miescher Cheilitis)?

Cheilitis Granulomatosa (CG) is an uncommon long-term illness that causes recurring hard swelling on one or both lips. This swelling is the result of an accumulation of immune cells, which can be seen under a microscope. Cheilitis Granulomatosa of Miescher (CGM) refers to the condition when this swelling is the only symptom.

Melkersson-Rosenthal Syndrome (MRS) is a specific condition that features three main symptoms: regular or constant swelling of the face and lips, a tongue that appears folded or cracked, and frequent facial nerve paralysis. However, not all cases of MRS show all these symptoms. Importantly, CGM is frequently found as the single symptom manifestation of MRS.

It’s essential to rule out other conditions that fall under the umbrella of orofacial granulomatosis diseases. This group, first identified separately in 1985, includes a range of conditions that cause a non-infectious and non-destructive accumulation of immune cells in the lips, mouth, and face. This group includes CG, as well as sarcoidosis and Crohn’s disease.

What Causes Cheilitis Granulomatosa (Miescher Cheilitis)?

The exact cause of CG is not known yet. Several potential causes have been suggested, including genetic factors, inflammation, allergies, and bacterial or microbial factors. However, the specific cause is still unclear.

The following are some of the factors that might contribute to CG:

Genetic factors: Some people with CG have other family members with the condition, suggesting a possible genetic link. However, this hasn’t been proven yet, and no direct link has been found between CG and any particular genes compared to those without the disease.

Immune system factors: CG is an inflammation that’s mainly driven by a specific type of immune response in the body. It seems that the body’s initial defense against bacteria may react improperly to certain substances, leading to a continuous inflammatory response.

Allergic factors: Allergies to substances such as dental materials, food items, and food additives could be triggers for CG. However, the link is not well-established yet.

Microbial factors: Certain microbes might lead to an improper immune response, leading to CG. These include Mycobacterium tuberculosis, Borrelia burgdorferi, Saccharomyces cerevisiae, and Candida albicans. However, studies have provided mixed results, so the role of these microbes in causing CG isn’t clear.

Some early research suggested that CG might be a symptom of Crohn’s disease that affects the intestines. However, given that less than 1% of people with Crohn’s disease also have CG, it’s questionable whether this is the case.

Risk Factors and Frequency for Cheilitis Granulomatosa (Miescher Cheilitis)

CG, or Central Giant Cell Granuloma, is a rare disease that is not very common and we don’t have exact numbers on how frequently it occurs. We do know from one study that it affects about 0.08% of the general population. It can start at any age but is usually seen in adults between the ages of 20 to 40. It’s rare in children, but there have been a few cases reported. As far as we know, it affects men and women equally, although some research suggests it might be slightly more common in women. There have also been a few cases where it ran in families.

  • CG, or Central Giant Cell Granuloma, is a rare disease.
  • It may affect about 0.08% of the population.
  • It most often occurs in adults between the ages of 20 to 40.
  • While rare in children, there have been some cases reported.
  • It seems to affect both men and women equally, though some research suggests it might be slightly more common in women.
  • A few familial cases of the disease have been reported.

Signs and Symptoms of Cheilitis Granulomatosa (Miescher Cheilitis)

Melkersson-Rosenthal Syndrome (MRS) is characterized by three primary symptoms. These include repetitive or continuous swelling in the mouth or face, paralysis of the facial nerve, and the occurrence of a ‘scrotal’ or ‘fissural’ tongue.

The swelling typically begins with recurrent episodes that resemble angioedema, a condition that causes sudden and severe swelling below the skin. Over time, after numerous episodes, this swelling can become permanent and firm. It is a non-red, non-painful swelling that may affect one or both lips, and in rare instances, it could interfere with speech or result in drooling.

However, it’s important to note that the full triad is not always present and is found in only a quarter to a third of people with MRS. Even in these cases, all the symptoms usually do not appear at the same time. The disease is characterized by flare-ups, with persistent lip and occasional facial swelling. In the early stages of the disease, it’s more common to see single or two-symptom forms, which account for 40% of cases.

n around 28% of cases, MRS appears in an incomplete form known as Cheilitis Granulomatosa (CGM) or Miescher syndrome. This is actually the most common form of MRS and usually affects the upper lip more than the lower lip. Changes in the mouth, palate, sublingual, and gum mucosa (mucus membrane) are also reported occasionally.

Keep in mind that oral signs of other diseases like sarcoidosis and Crohn’s disease can look a lot like CGM or MRS.

Testing for Cheilitis Granulomatosa (Miescher Cheilitis)

Granulomatous cheilitis (CG) is primarily diagnosed based on clinical signs. Detailed tissue examination (histopathology) can support this diagnosis if results are positive. Further tests may be needed to rule out other conditions like sarcoidosis. These tests may include a detailed chest scan (high-resolution computed tomography scan) and blood tests to measure angiotensin-converting enzyme levels. A purified protein derivative (PPD) test might also be done based on the specifics of each case.

However, the timeframe between the beginning of lip swelling and the typical symptoms of Crohn’s disease can vary greatly and is unpredictable. Therefore, the necessity of certain tests – such as a colonoscopy or examination of the upper digestive system (gastroduodenoscopy) with tissue biopsies – in patients without digestive issues is still a topic of debate.

Treatment Options for Cheilitis Granulomatosa (Miescher Cheilitis)

This disease is not very common, which is why there’s no specific treatment and few controlled studies. At the moment, there’s no cure. The aim of the treatments we do have is just to reduce the number of flare-ups, especially during the swelling stage. The treatments try to make patients more comfortable and improve their quality of life, which can be significantly affected by the persistent swelling of the lips and face. It’s rare, but the disease can sometimes go away on its own.

Steroid therapy, a common treatment, can be applied directly to the affected area, given orally, or injected into the lesions. Sometimes, a short course of systemic steroids (prednisolone: 0.3 to 0.7 mg/kg/day; 25 to 50 mg/day) may be prescribed. Several other treatments have been tested, including topical tacrolimus, oral clofazimine, thalidomide, dapsone, and doxycycline. These aim to reduce inflammation or modify the immune system’s responses, but their results have been inconsistent.

Some patients have been treated with immunosuppressive medications like mycophenolate mofetil and azathioprine, or drugs that work against tumor necrosis factor-alpha (TNF-alpha). These treatments have been tried alone and in combination with others, but again, their results were unpredictable and varied widely.

Another treatment, fumaric acid esters, worked well in about half of patients who hadn’t responded to other treatments.

Lastly, surgical reduction of the lips (cheiloplasty reduction) might be an option if the lesions have stopped changing and are stable.

Swollen lips can have a variety of causes, such as:

  • Acquired angioedema due to a deficiency in C1, a type of protein
  • Hereditary angioedema, a rare genetic condition
  • Glandular cheilitis, inflammation of the salivary glands near the lips
  • Dental abscess, a collection of pus caused by a bacterial infection in the tooth
  • Crohn’s disease, a type of inflammatory bowel disease
  • Various granulomatous disorders, conditions that cause inflammation in the body, including sarcoidosis, tuberculosis, leishmaniasis, leprosy, and Wegener granulomatosis

Taking a closer look at the lip swelling could reveal clues about possible underlying causes. For example, angioedema does not result in a specific type of inflammation called granulomas. Also, conditions like hereditary angioedema often run in families and cause occasional stomach pain. On the other hand, a thorough dental examination and swabs can help rule out a dental abscess.

What to expect with Cheilitis Granulomatosa (Miescher Cheilitis)

Due to a lack of studies, limited knowledge of the disease process, and mild improvement after treatment, the overall outlook for those with CG spectrum or OFG disease isn’t very positive. A single symptom form of the disease, known as CGM, may improve with the previously mentioned treatments. However, MRS has the worst expectations and there is no solid research showing successful treatment of MRS neurological complications.

Possible Complications When Diagnosed with Cheilitis Granulomatosa (Miescher Cheilitis)

CGM, or Central Giant Cell Granuloma, is generally not harmful. However, it can often be part of a condition known as MRS, or Multiple Respiratory chain Deficiency Syndromes. This could raise the risk of facial paralysis in the future. There’s also a chance that the presence of CGM in a person could indicate the development of Crohn’s disease, a type of inflammatory bowel disease. This could lead to other health complications related to the bowel condition.

Preventing Cheilitis Granulomatosa (Miescher Cheilitis)

The patient should be advised about the potential for swelling of the lips, which might just be a result of their current condition. However, they should also be urged to let their doctor know if they experience facial weakness, as this could indicate a more serious problem. It’s crucial for patients to understand the situation clearly, including the fact that treatment options might be limited.

Frequently asked questions

Cheilitis Granulomatosa (Miescher Cheilitis) is an uncommon long-term illness that causes recurring hard swelling on one or both lips.

CG, or Central Giant Cell Granuloma, is a rare disease.

The signs and symptoms of Cheilitis Granulomatosa (Miescher Cheilitis) include: - Swelling in the mouth or face, which can be repetitive or continuous. - Paralysis of the facial nerve. - The occurrence of a 'scrotal' or 'fissural' tongue. - Recurrent episodes of swelling that resemble angioedema. - Permanent and firm swelling that is non-red and non-painful. - Swelling that may affect one or both lips. - Rare instances of interference with speech or drooling. - Changes in the mouth, palate, sublingual, and gum mucosa (mucus membrane) may also be reported occasionally. - It's important to note that the full triad of symptoms is not always present and is found in only a quarter to a third of people with Cheilitis Granulomatosa.

The exact cause of Cheilitis Granulomatosa (Miescher Cheilitis) is not known yet. Several potential causes have been suggested, including genetic factors, immune system factors, allergic factors, and microbial factors. However, the specific cause is still unclear.

The doctor needs to rule out the following conditions when diagnosing Cheilitis Granulomatosa (Miescher Cheilitis): - Sarcoidosis - Crohn's disease - Acquired angioedema due to a deficiency in C1 - Hereditary angioedema - Glandular cheilitis - Dental abscess - Various granulomatous disorders, including tuberculosis, leishmaniasis, leprosy, and Wegener granulomatosis.

The types of tests that may be needed to diagnose Cheilitis Granulomatosa (Miescher Cheilitis) include: - Clinical examination and evaluation of symptoms - Histopathology (tissue examination) to support the diagnosis - Blood tests to measure angiotensin-converting enzyme levels - High-resolution computed tomography (CT) scan of the chest to rule out other conditions like sarcoidosis - Purified protein derivative (PPD) test may be done based on the specifics of each case It is important to note that the necessity of certain tests, such as colonoscopy or gastroduodenoscopy with tissue biopsies, in patients without digestive issues is still a topic of debate.

Cheilitis Granulomatosa (Miescher Cheilitis) can be treated with various methods. There is no specific cure for the disease, but the aim of treatment is to reduce flare-ups and improve the patient's quality of life. Steroid therapy is a common treatment option, which can be applied topically, orally, or injected into the lesions. Other treatments that have been tested include topical tacrolimus, oral clofazimine, thalidomide, dapsone, and doxycycline, which aim to reduce inflammation or modify the immune system's responses. Immunosuppressive medications like mycophenolate mofetil and azathioprine, as well as drugs that work against tumor necrosis factor-alpha (TNF-alpha), have also been used. Fumaric acid esters have shown success in about half of patients who haven't responded to other treatments. In some cases, surgical reduction of the lips (cheiloplasty reduction) may be considered if the lesions have stabilized.

The prognosis for Cheilitis Granulomatosa (Miescher Cheilitis) is not very positive due to a lack of studies, limited knowledge of the disease process, and mild improvement after treatment. However, the single symptom form of the disease, known as CGM, may improve with treatment. On the other hand, Melkersson-Rosenthal Syndrome (MRS) has the worst expectations and there is no solid research showing successful treatment of MRS neurological complications.

A dermatologist or an oral and maxillofacial specialist.

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