eMedicine Specialties > Dermatology > Diseases of the Oral Mucosa

Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome)

Author: Crispian Scully, MD, PhD, DSc, FRCPath, MRCS, CBE, MDS, FDSRCS, FDSRCPS, FFDRCSI, FDSRCSE, FMedSci, FHEA, FUCL, DChD, DMed(HC),, Professor, Director of Special Projects, Eastman Dental Institute for Oral Health Care Sciences; Professor, Special Needs Dentistry, University College; Professor, Oral Medicine, Pathology and Microbiology, University of London
Contributor Information and Disclosures

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

More on Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome)

Overview: Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome)
Differential Diagnoses & Workup: Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome)
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

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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

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Contributor Information and Disclosures

Author

Crispian Scully, MD, PhD, DSc, FRCPath, MRCS, CBE, MDS, FDSRCS, FDSRCPS, FFDRCSI, FDSRCSE, FMedSci, FHEA, FUCL, DChD, DMed(HC),, Professor, Director of Special Projects, Eastman Dental Institute for Oral Health Care Sciences; Professor, Special Needs Dentistry, University College; Professor, Oral Medicine, Pathology and Microbiology, University of London
Crispian Scully, MD, PhD, DSc, FRCPath, MRCS, CBE, MDS, FDSRCS, FDSRCPS, FFDRCSI, FDSRCSE, FMedSci, FHEA, FUCL, DChD, DMed(HC), is a member of the following medical societies: Academy of Medical Science, British Dental Association, British Society for Oral Medicine, International Association for Dental Research, and Royal Society of Medicine
Disclosure: Nothing to disclose.

Medical Editor

David P Fivenson, MD, Associate Director, St Joseph Mercy Hospital Dermatology Program, Ann Arbor, Michigan
David P Fivenson, MD is a member of the following medical societies: American Academy of Dermatology, Medical Dermatology Society, Michigan Dermatological Society, Michigan State Medical Society, Photomedicine Society, Society for Investigative Dermatology, and Wound Healing Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey
Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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