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Melkersson-rosenthal syndrome (melkersson idiopathic fibroedema; miescher cheilitis granulomatosis) 7


Melkersson-Rosenthal Syndrome (Melkersson Idiopathic Fibroedema; Miescher Cheilitis
Granulomatosis) 626f52g 626f52g 626f52g 626f52g 626f52g 626f52g 7

General: Occurrence in childhood or youth; possible etiologies include viral infection, tuberculosis, sarcoidosis, and allergic reactions (all affecting parasympathetic cells in geniculate ganglia); facial palsy resembles Bell palsy; possible localization of this disorder to the gene at 9p11 has been reported.

Ocular: Lagophthalmos; lid edema; lacrimation secondary to the 'crocodile tear' phenomenon from aberrant seventh nerve regeneration; exposure keratitis and corneal ulcers; corneal opacities.

Clinical: Chronic edema of face and lips; peripheral facial palsy (may be bilateral), which may precede edema by weeks to years; furrowed tongue; granulomatous cheilitis and glossitis; lingua plicata.

Cohen HA, et al. Melkersson-Rosenthal syndrome. Cutis 1994; 54:327-328.

Hallet JW, Mitchell B. Melkersson-Rosenthal Syndrome. Am J Ophthalmol 1968; 65:542.

Paton D. The Melkersson-Rosenthal Syndrome. Am J Ophthalmol 1965; 59:705.

Rosenthal C. Klinischer bio1ogischer Beitrag zur Konstitutionspathologie: Gemeinsames Aufreten von (rezidivierender familiarer) Facialislahmung, Angioneurotischem Gesichtsoderm und Lingua Plicata in Arthritismus-Familien. Z Neurol Psychiatr 1931; 475:500.

Smeets E, et al. Melkersson-Rosenthal syndrome and de novo autosomal t(9;21)(P 11;p 11) translocation. Clin Genet 1994; 45:323-324.




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