eMedicine Specialties > Dermatology > Diseases of the Oral Mucosa
Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome)
Updated: Oct 5, 2009
Introduction
Background
Granulomatous cheilitis is a chronic swelling of the lip due to granulomatous inflammation. Miescher cheilitis is the term used when the granulomatous changes are confined to the lip. Miescher cheilitis is generally regarded as a monosymptomatic form of the Melkersson-Rosenthal syndrome, although the possibility remains that these may be 2 separate diseases. Melkersson-Rosenthal syndrome is the term used when cheilitis occurs with facial palsy and plicated tongue.
Melkersson-Rosenthal syndrome is occasionally a manifestation of Crohn disease1,2,3,4,5 or orofacial granulomatosis (OFG).
Pathophysiology
In granulomatous cheilitis, normal lip architecture is eventually altered by the presence of lymphoedema and noncaseating granulomas in the lamina propria. T H 1 immunocytes produce interleukin 12 and RANTES/MIP-1alpha and granulomas. Expression of protease-activated receptor 1 and 2 occurs in OFG. HLA typing may show HLA-A2 or HLA-A11.6
Frequency
International
The frequency is unknown; granulomatous cheilitis is rare.
Mortality/Morbidity
Morbidity related to granulomatous cheilitis depends on whether underlying organic disease, such as Crohn disease or sarcoidosis,7 is present.
Race
No racial predilection is recognized for granulomatous cheilitis.
Sex
No sexual predilection is known for granulomatous cheilitis.
Age
Onset of granulomatous cheilitis usually occurs in young adult life.
Clinical
History
Cheilitis granulomatosa is episodic with nontender swelling and enlargement of one or both lips. Occasionally, similar swellings involve other areas, including the periocular region.
- The first episode of edema typically subsides completely in hours or days. After recurrent attacks, swelling may persist and slowly increase in degree, eventually becoming permanent. Recurrences can range from days to years.
- Attacks sometimes are accompanied by fever and mild constitutional symptoms (eg, headache, visual disturbance).
- Cranial nerve palsies may be associated. Melkersson-Rosenthal syndrome is the association with facial nerve palsy.8,9
Physical
The earliest manifestation of granulomatous cheilitis is sudden diffuse or occasionally nodular swellings of the lip or the face involving (in decreasing order of frequency) the upper lip, the lower lip, and one or both cheeks. The forehead, the eyelids, or one side of the scalp may be involved (less common). The upper lip is involved slightly more often than the lower lip, and it may feel soft, firm, or nodular on palpation.
- Once chronicity is established, the enlarged lip appears cracked and fissured with reddish brown discoloration and scaling. The fissured lip becomes painful and eventually acquires the consistency of firm rubber.
- Swelling may regress very slowly after some years.
- Regional lymph nodes are enlarged (usually minimally) in 50% of patients.
- A fissured or plicated tongue is seen in 20-40% of patients.
- Its presence from birth (in some patients) may indicate a genetic susceptibility.
- Patients may lose the sense of taste and have decreased salivary gland secretion.
- Facial palsy of the lower motor-neuron type occurs in about 30% of patients.
- Facial palsy may precede attacks of edema by months or years, but it more commonly develops later.
- Facial palsy is intermittent at first, but it may become permanent.
- It can be unilateral or bilateral, partial or complete.
- Other cranial nerves (eg, olfactory, auditory, glossopharyngeal, hypoglossal) are occasionally affected.
- Central nervous system involvement has been reported, but the significance of resulting symptoms is easily overlooked because they are very variable (sometimes simulating multiple sclerosis but often with a poorly defined association of psychiatric and neurologic features).
- Autonomic disturbances may occur.
Causes
The cause of granulomatous cheilitis is unknown.10 A genetic predisposition may exist in Melkersson-Rosenthal syndrome; siblings have been affected, and a plicated tongue may be present in otherwise unaffected relatives. Crohn disease, sarcoidosis, and orofacial granulomatosis may present in a similar clinical fashion, and with identical histologic findings. Dietary or other antigens are the most common identified cause of orofacial granulomatosis (OFG).11,12 Contact antigens are sometimes implicated.13 OFG may result from reactions to some foods or medicaments, such as cinnamon aldehyde and benzoates.14
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Overview: Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome) |
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| Treatment & Medication: Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome) |
| Follow-up: Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome) |
| Multimedia: Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome) |
| References |
| Further Reading |
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References
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Further Reading
National Institute of Neurological Disorders and Stroke -NINDS Melkersson-Rosenthal Syndrome Information Page
Keywords
granulomatous cheilitis, Miescher-Melkersson-Rosenthal syndrome, orofacial granulomatosis, Miescher cheilitis, Melkersson-Rosenthal syndrome, Rossolimo-Melkersson-Rosenthal syndrome, swelling of the lip, Crohn disease, Crohn's disease
Overview: Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome)