Melkersson-rosenthal syndrome G51.2

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

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Synonym(s)

orofacial granulomatosis; Orofacial granulomatous

History
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Melkersson, 1928; Rosenthal, 1931

Definition
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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".

Occurrence/Epidemiology
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The incidence of MRS in adults is reported to be 1:1250 (Ziem et al. 2000). In children the full picture of MRS is rare.

Etiopathogenesis
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Unknown, to be discussed: constitutional abnormality, hereditary or acquired disturbance of the autonomous defence system, inflammatory, possibly infection-allergic reactions to different antigens.

In about 30% of patients a familial occurrence is reported (Feng et al. 2014). An autosomal dominant inheritance is being discussed? Possibly the responsible gene is located on the chromosome 9p11.

Manifestation
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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 early erysipelas tetrade (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.
  • Upper lip furuncle: Acute course with circumscribed dolent swelling. Often purulent abscess tip.
  • Macrochelia of other cause: No variable course. Mostly evident underlying diseases like exfoliative chelitis and others.
  • Facial nerve palsy of other cause: not combined with other partial symptoms of Melkersson-Rosenthal syndrome.

Therapy
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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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So far, there are no approved therapies for this clinical picture.

  • Improvements can be achieved with clofazimine (e.g. Lamprene). Early use in particular improves the chances of success, initially 100 mg/day p.o. over 10 days, then alternating 100 mg 3-4 times/week over 6 months, possibly repeated after a 3-month break. In the absence of success the dosage can be doubled.
  • Alternatively (better therapy modality): Fumaric acid ester (e.g. Fumaderm®) in the usual dosage. This therapy approach has been the subject of personal experience as well as several published cases (G. Hauck 2017). Remark: This therapy should be planned over a period of >12 months!
  • Alternative: Continuous 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 they show only moderate success. Prednisolone (e.g. Decortin H) initially 40-60 mg/day p.o. over 2-4 weeks, followed by a slow dose reduction.
  • Alternatively: Omalizumab (Nettis E et al. 2018)
  • Other procedures: Various therapy attempts with chloroquine, diuretics, DADPS, acetylsalicylic acid, nicotinic acid amide and others are described with doubtful 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.

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

Literature
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  1. Elias MK et al (2013) The Melkersson-Rosenthal syndrome: a retrospective study of biopsied cases. J Neurol 260:138-143
  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
  5. Lüscher E (1949) syndrome from Melkersson-Rosenthal. Switzerland Med Weekly 79: 1-3
  6. Melkersson E (1928) One case of recurrent social security benefits in connection with the epidemic of epidemic oedema. Hygiea, Stockholm 90: 737-741
  7. Miescher G (1956) Cheilitis and pareitis granulomatosa without facial paresis in the presence of a scrotal lingua. Dermatologica 112: 536
  8. Neuhofer J, Fritsch P (1984) Cheilitis granulomatosa (Melkersson-Rosenthal syndrome): treatment with clofazimine. dermatologist 35: 459-463
  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
  11. Stosiek N et al (1991) Melkersson-Rosenthal syndrome - Analysis of the course of 73 patients (1968-1990). Z Hautkr 66: 18-24
  12. Sussman GL et al (1992) Melkersson-Rosenthal syndrome: clinical, pathologic, and therapeutic considerations. Ann Allergy 69: 187-194
  13. Tausch I et al (1992) experiences with clofazimine therapy of Melkersson-Rosenthal syndrome. Dermatologist 43: 194-198
  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
  19. Zimmer WM et al (1992) Orofacial manifestations of Melkersson-Rosenthal syndrome. A study of 42 patients and review of 220 cases from the literature. Oral Surg Oral Med Oral Catholic 74: 610-619

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Last updated on: 29.10.2020