Melkersson-rosenthal syndrome G51.2

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 13.03.2022

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orofacial granulomatosis; Orofacial granulomatous

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Melkersson, 1928; Rosenthal, 1931

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Chronic, systemic, granulomatous inflammation with the classic symptom triad: Cheilitis granulomatosa, facial paresis, lingua plicata. The full picture of the syndrome is rare, more frequent are differently pronounced minus variants. That of E. Melkersson and C. Rosenthal is now increasingly referred to under the broader term"orofacial granulomatosis" and more generally defined as "recurrent uni- or bilateral orofacial swelling with cranial nerve dysfunction or paresis".

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The incidence of Melkersson-Rosenthal syndrome (MRS) in adults is reported to be 1:1250 (Ziem et al. 2000). In children, the full-blown MRS is rare.

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Unknown, discussed are: constitutional anomaly, hereditary or acquired disorder of the autonomic defense system, inflammatory, possibly infectious allergic reactions to different antigens.

Familial occurrence is reported in about 30% of patients (Feng et al. 2014). An autosomal dominant mode of inheritance is discussed. Possibly, the responsible gene is located on chromosome 9p11.

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The average age at manifestation is 14.1 years (Feng et al 2014).

Clinical features
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The fully developed syndrome is only observed in a minority of patients in larger groups. Mono- and oligosymptomatic cases are the rule (>80%); they are also found as initial indicative manifestations.

Typical is a variable course of symptoms with circumscribed facial swelling, mostly lip swelling (initially recurrent, later persistent, usually unilateral) and facial nerve palsy. The changes are functionally and cosmetically disturbing for the patient, sometimes even painful.

  • Circumscribed facial swelling:
  • Facial nerve palsy (in 19.4% of cases): Mostly unilateral, in 25% of cases bilateral, always of the peripheral type.
  • Lingua plicata in 54% of cases (normal population 13%). Assignment to cardinal symptoms is abandoned in recent years, has only indicator function.
  • Minor symptoms: Due to their frequent and constant occurrence, they are of great importance in the diagnosis. They are found with decreasing frequency:
    • Crohn's disease (6.8%)
    • Migraine headaches (5.7%)
    • Paresthesia in the swelling area
    • Disturbances of tear secretion
    • Redness
    • Itching
    • Nausea/nausea
    • secretion disorders of the salivary glands or nasal mucosa
    • Swindle
    • Tingling
    • facial sweating
    • Acroparesthesia
    • Heat sensation
    • Globus feeling in the throat
    • acoustic sensations.
    • systemic lupus erythematosus (2.3%)

Differential diagnosis
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Erysipelas recidivans: Characteristic history with the early erysipelas tetrad (fever, redness, painful swelling, acute lymphadenopathy). Laboratory: signs of inflammation.

Herpes simplex recidivans: Typical interval-like course with signs of evident herpes simplex on the skin.

Furuncle of the upper lip: Acute course with circumscribed dolent swelling. The tip of the abscess is often purulent.

Macrocheilia of other cause: No variable course. Mostly evident underlying diseases such as exfoliative cheilitides etc.

Facial paresis of other cause: not combined with other partial symptoms of Melkersson-Rosenthal syndrome.

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The treatment is difficult and often unsuccessful.

External therapy
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The use of glucocorticoids intralesionally usually shows only temporary success and can be useful at the beginning of a relapse. This is done by intradermal injection of triamcinolone acetonide crystal suspension (e.g. Volon A diluted 1:1 with local anesthetics such as scandicain) once a month.

Internal therapy
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There are no approved therapies for this disease pattern so far. Unfortunately, there is a lack of reliable study results due to the rarity of the disease.

  • Improvements can be achieved with clofazimine (e.g. Lamprene). Early use in particular improves the chances of success, initially 100 mg/day p.o. for 10 days, then alternating 100 mg 3-4 times/week for 6 months, repeated after a 3-month break if necessary. If there is no success, the dosage can be doubled.
  • Alternative (better therapy modality): Fumaric acid ester (e.g. Fumaderm®) in the usual dosage. There are own personal experiences about this therapy approach as well as some published casuistics (G. Hauck 2017). Note: This therapy should be planned over a period of >12 months!
  • Alternative: Permanent intake of non-steroidal anti-inflammatory drugs such as ibuprofen (e.g. Ibuprofen Heumann 400-800 mg/day).
  • Alternative: Systemic glucocorticoids are often recommended, but in our experience show only moderate success. Prednisolone (e.g. Decortin H) initially 40-60 mg/day p.o. for 2-4 weeks, then slow dose reduction.
  • Alternative: Omalizumab (Nettis E et al. 2018).
  • Other methods: Multiple therapeutic modalities with chloroquine, diuretics, DADPS, acetylsalicylic acid, nicotinic acid amide, and others have been described with dubious success.

Operative therapie
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In the stage of persistent swellings, the operative reduction of the lip (lip reduction plastic surgery) is often the only possibility to achieve a permanent improvement.

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Thrustworthy, chronic, years-long, possibly life-long course. Quoad vitam good, quoad sanationem bad. Spontaneous remission possible.

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  2. Feng S et al (2014) Melkersson-Rosenthal syndrome: a retrospective study of 44 patients. Acta Otolaryngol 134:977-981
  3. Hauck G (2017) Fumaric acid ester therapy for Melkersson-Rosenthal syndrome. Act Dermatol 43: 459-460
  4. Hornstein OP et al. (1987) Classification and clinical variation of Melkersson-Rosenthal syndrome (MRS). Z Hautkr 62: 1453-1475
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  9. Nettis E et al(2018) A favorable response to omalizumab in a patient with cheilitis granulomatosa.
    J Allergy Clin Immunol Pract 6:1425-1426.
  10. Rosenthal C (1931) Clinical hereditary biological contribution to constitutional pathology. Common occurrence of recurrent familial facial paralysis, angioneurotic facial edema and lingua plicata in families with arthritis. Z Total Neurol Psychiatry 131: 475-501
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  14. Shapiro M et al (2003) Melkersson-Rosenthal syndrome in the periocular area: a review of the literature and case report. Ann Plast Surgery 50: 644-648
  15. van De Scheur MR et al (2003) Orofacial granulomatosis in a patient with Crohn's disease. J Am Acad Dermatol 49: 952-954
  16. van der Waal RI et al (2001) Cheilitis granulomatosa. J Eur Acad Dermatol Venereol. 15: 519-523
  17. Volz A et al (2010) Operative lip reduction plasty for therapy-resistant Cheilitis granulomatosa. JDDG 8: 303-305
  18. Ziem PE et al(2000) Melkersson-Rosenthal syndrome in childhood: a challenge in differential diagnosis and treatment. Br J Dermatol 143: 860-863
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Last updated on: 13.03.2022