Melkersson's syndrome

Orofacial granulomatosis (OFG)

• granulomatous cheilitis is the most frequent sign of OFG -a disorder under which also encompasses sarcoidosis, Crohn's disease, atypical tuberculosis, Anderson-Fabry disease, possibly some allergic reactions, Melkersson-Rosenthal syndrome (MRS) and cheilitis granulomatosa (Miescher's cheilitis)
• this condition was first described by Miescher (1) in 1945
• histologically defined as a noncaseating, epithelioid, granulomatous inflammatory disorder (2, 3)
• presents with chronic swelling of the oral or facial tissues due to granulomatous inflammation

MRS is additionally characterised by

• facial paralysis
• plicated (furrowed) tongue
• in MRS facial nerve palsy may be manifest several years before the furrowed tongue and lip swelling

Note that incidence of OFG or MRS, is similar to that of Crohn's Disease (reported to peak between 15 and 25 years of age). Some have suggested a common aetiology for OFG and Crohn's Disease - these conditions very occasionally occur simultaneously in the same individual. There is a necessity for evaluation of patients for Crohn's Disease and other aetiologies once the diagnosis of OFG is made.

Reference:

1. Miescher G. Über die essentielle granulomatöse Makrocheilie (granulomatöse Cheilitis). Dermatologica (Basel) 1945;91:57 64.
2. Hornstein OP. Über die Pathogenese des sogenannten Melkersson-Rosenthal Syndrom (einschlielich der Cheilitis granulomatosa Miescher). Arch Klin Exp Dermatol 1961;212:570 605.
3. De Aloe G, Rubegni P, Mazzatenta C, Fimiami M. Complete Melkersson-Rosenthal syndrome in a patient with Crohn's disease. Dermatology 1997;195:182.
4. Hornstein OP, Stosiek N, Schönberger A, Meisel-Stosiek M. Klassifikation und klinische Variationsbreite des Melkersson-Rosenthal Syndroms. Zentralbl Hautkr 1987;62:1453 1475.
5. Pediatr Dermatol. 1999 Jan-Feb;16(1):39-42.
6. Dermatology. 2003;206(3):273-8.
7. Oral Dis. 1997 Sep;3(3):188-92.