Miescher’s Syndrome - Causes, Symptoms, and Treatments

Introduction

Lip skin is highly delicate and sensitive. As a result, it is prone to develop numerous associated disorders. Miescher’s syndrome is yet another form of a disorder that manifests characteristics features in the lips.

What Is Miescher Syndrome?

Miescher’s syndrome is a peculiar subclass that comes under the category of orofacial granulomatosis. A hallmark feature of this syndrome is that it involves the lips alone. More often used synonyms for Miescher’s syndrome are Miescher’s cheilitis, and Cheilitis granulomatosa. It produces changes in the lips through swelling, thus creating facial deformities. It was first elucidated by Meischer and hence the name. It is not an infectious condition and does not spread from one person to the other. Certain theory suggests it as a version of Melkerson-Rosenthal syndrome exhibiting a single symptom (monosymptomatic version).

What Is Orofacial Granulomatosis?

Orofacial granulomatosis is a less frequently encountered condition that affects the soft tissues restricted within the facial region. It depicts both intra-oral as well as extra-oral manifestations, including periodic bumping of the lips, gums, face, mucosa covering the mouth, and occasionally the soft tissue encircling the eye. In addition, it incorporates a wide range of other conditions like Miescher’s syndrome. At times they are seen in close connection with certain body syndromes, such as Sarcoidosis (abnormal piling up of immune cells forming granulomas), Crohn's disease (affecting the digestive tract), and Wegener’s granulomatosis (immune cells attack own cells).

What Is Melkersson Rosenthal Syndrome?

Melkerson Rosenthal syndrome is not only a skin condition but also has neurological involvement. It is an uncommon neurocutaneous sickness that includes typical symptoms like:

• Bumping of the facial soft tissue.

• Numerous crevices in the tongue (fissured tongue).

• Intermittent facial paralysis (palsy).

It initially begins as an on-and-off condition, and after repeated cycles, it gets transformed into a long-standing permanent condition. The involvement of the facial nerve distinguishes it from other orofacial granulomatous conditions. It is not necessary to have all three classic signs in a single person. When its symptoms are limited to the lip area, it is considered as a variant form - Miescher’s syndrome.

What Are the Symptoms of Miescher Syndrome?

• Visible symptoms of Meischer’s syndrome are restricted to the lip area, sparing other soft tissue structures in the facial area. There will be a sudden bulbing up of the lips. The upper lips become bulbous and fluffy, but lower lips do get involved at times.

• Routinely, these lip swellings remain for a few hours or sometimes up to a few days. It depicts a typical recurrence pattern.

• The size and nature of the swelling intensify upon subsequent attacks. When encountered for the first time, it is depicted in the form of soft, fluffy, and fluctuant bulbing up of the lips with no associated soreness or irritation.

• After a few episodes of recurrence, the nature of the swelling changes. It acquires a rubbery texture and exhibits small cracks, scales, and minute points of bleeding.

• Lips then acquire a more intense pigmentation. Finally, there will be associated soreness and burning kind of sensations in the lips.

• Apart from the findings in the lips, there are some general features manifested in Miescher’s syndrome. Some of them include the following:

1. Elevated body temperature

2. Irregularities in vision

3. Headache

What Causes Miescher Syndrome?

Researchers are still trying to figure out the culprit behind this rare cutaneous condition. However, there are a great deal of hypotheses that suggest certain reasons for the development of this condition. Some of the relevant hypothetical reasons include the following:

• Genetic Influence: Certain theories put forward a familial connection to the disease pattern though they lack solid pieces of evidence.

• Allergy: Allergic factors are considered in charge of the condition. They propose that the hypersensitivity (allergic) response to certain materials, particularly eatables, is elicited through Miescher’s syndrome. Few materials used in different dental treatment procedures can also evoke similar kinds of responses.

• Infection: Microbial agents like Candida albicans, Saccharomyces cerevisiae, Mycobacterium tuberculosis, and Borrelia burgdorferi are recognized for altering the response of immune cells. It is believed that this altered response precipitates the syndrome.

• Immunologic Alterations: Some form of alterations in the normal functioning of immune cells could reflect in inflammatory diseases.

• Ultraviolet Radiation: Exaggerated reaction to ultraviolet exposure may evoke this kind of inflammatory enlargement.

• Association With Syndromes: Numerous syndromes and diseases can influence bodily functions. Occasionally Miescher’s syndrome is encountered in interrelation with some other disease conditions like Crohn’s disease.

How Is Miescher Syndrome Diagnosed?

Thorough medical history and clinical findings act as the leading lamp for diagnosis. There are no specific tests to detect the syndrome. However, biopsy specimens are collected for analyzing the histopathology of the cells, which often guides to a final diagnosis. If any other syndrome is suspected, diagnostic tests specific to that particular condition are advised to rule out the confusion.

What Are the Treatment Interventions Available for Miescher Syndrome?

There is no specific medicine for Miescher’s syndrome; however, the treatment strategy is largely based on the symptoms and suspected causes that led to the development of the condition. For example, in case of allergy-induced, the potent agent that is responsible for evoking this reaction needs to be identified and keep oneself away from those agents. Routinely advised medicines include the following:

• Steroid Therapy: Corticosteroids are advised frequently to deal with the syndrome. Initially, it is advised in topical form to apply over the affected lips. However, if that is not fruitful, then other means of administration, like local and intralesional injections (into the lesion), are preferred. Very rarely, systemic steroids are recommended.

• Anti-Inflammatory Therapy: Non-steroidal anti-inflammatory drugs are suggested to calm down the inflammatory reaction. Antibiotics with anti-inflammatory effects, like penicillin, are also advised. Dapsone (sulfone antibiotic) is also proposed for resistant cases.

• Immunomodulatory Therapy: Drugs with the potential to modify immune response, like Clofazimine and Tacrolimus, are also found effective.

• Surgical Therapy: Enlargement of the lips creates aesthetic concerns in the patients. If the enlargement progresses to a permanent stage, it significantly hampers the patient's physical appearance. In such cases, surgical correction of the lip size is advised, which is technically known by the term reduction cheiloplasty.

Conclusion

Miescher’s syndrome, though quite rare, can significantly hamper a person's social life. It produces facial deformities and thereby impairs the physical appearance of the person. It is not a completely curable condition. However, surgical therapy aids in regaining physical appearance, enhancing the person's self-confidence.