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Macrocheilitis
Last reviewed: 04.07.2025
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Macrocheilitis (Miescher's granulomatous cheilitis) is the leading symptom of Melkersson-Rosenthal syndrome (Rossolimo-Melkersson-Rosenthal). The disease is characterized by a combination of macrocheilitis, folded tongue and facial nerve paralysis. Macrocheilitis has a chronic course with alternating periods of exacerbation and remission. Over time, all symptoms of the disease can become persistent. The duration of the disease can be up to 4-20 years.
ICD-10 code
Q18.6 Macrocheilitis.
Reasons
The etiology of the disease is not completely clear, but infectious-allergic genesis is considered, developing against the background of hereditary predisposition. Macrocheilitis is accompanied by sensitization of the body to antigens of staphylococcus or herpes virus, which is confirmed by allergic tests with microbial allergens. This is a relatively rare disease. Macrocheilitis occurs more often in young men.
Symptoms of macrocheilitis
Asymptomatic course is possible, but more often macrocheilitis is characterized by distension, numbness in the area of the lips and tongue. Acute onset is noted - within a few hours, one or both lips swell, the edge of the lip turns out like a proboscis, the lip increases in size by 3-4 times, which makes it difficult to eat and speak. Usually, the thickening of the lip is expressed unevenly (more in the center of the upper lip). The color of the lips may not change or acquire a stagnant red color. The consistency of the lip tissue is soft or densely elastic. Peeling is possible on the red border of the lip.
The swelling of the lip can persist for a month or more, sometimes the process resolves, but then macrocheilitis recurs again. The swelling can gradually spread to the cheeks, tongue, nose, one or both halves of the face.
When the process spreads to the tongue, it thickens, its mobility becomes difficult, areas of uneven protrusion or lobulation appear, more pronounced in the anterior and middle parts of the tongue, and taste sensitivity is impaired.
Facial nerve paralysis is the second symptom of Melkersson-Rosenthal syndrome, it may precede the appearance of other symptoms (macrocheilitis and folded tongue), and neurological pain in the face or headaches are possible before the development of paralysis. The paralysis is unilateral (accompanied by smoothing of the nasolabial fold, drooping of the corner of the mouth, widening of the palpebral fissure) and has a tendency to relapse. In the innervation zone of the affected facial nerve, motor function and sensitivity may be partially preserved.
The third symptom of the disease is a folded tongue. Macrocheilitis is characterized by the presence of deep grooves on the surface of the tongue, giving it a bumpy appearance. This symptom of macrocheilitis is not noted in all patients.
How to recognize macrocheilitis?
Diagnosis of Melkersson-Rosenthal syndrome does not cause difficulties when all the components of the symptoms are clinically manifested.
In case of isolated symptom of macrocheilia, diagnosis is based on pathomorphological examination. Histological picture is characterized by edema and granulomatous inflammation of tuberculoid, sarcoid or lymphonodular-plasmatic type.
Differential diagnostics
Macrocheilitis is differentiated from Quincke's edema, erysipelas, and hemangioma.
Quincke's edema is not long-lasting and disappears completely and quickly when taking antihistamines.
In chronic erysipelas, elephantiasis of the 176 region may develop, which is clinically manifested by their significant increase. However, the course of erysipelas is accompanied by exacerbations, with an increase in body temperature and inflammatory phenomena, which is not typical for Melkersson-Rosenthal syndrome.
Hemangioma is observed from birth or early childhood.
[ Treatment
Treatment of macrocheilitis is long-term and includes:
- sanitation of foci of chronic infection;
- prescribing antibiotics when microbial allergies are detected (macrolides);
- prescription of antiviral drugs (long-term) if sensitivity to the herpes virus is detected (acyclovir);
- prescription of antithyroid drugs (loratadine, desloratadine, etc.);
- prescribing immunomodulators (likopid, poludan, galanit).
What is the prognosis for macrocheilitis?
The prognosis is favorable.