Background and Objective: Melkersson Rosenthal syndrome (MRS) is a rare disorder
of unknown etiology and comprises the triad: orofacial edema, recurrent facial paralysis and lingua
plicata. In the current literature confusing heterogeneity exists, mixing together the historically
grown terms cheilitis granulomatosa or granulomatous cheilitis, Melkersson Rosenthal syndrome
and the umbrella term Orofacial Granulomatosis (OFG).
Methods: We provide a systematic review comprising all three disease entities of orofacial granulomatosis
using the computerized database “Pubmed Medline” entering the keywords “orofacial
granulomatosis” (141 references), “Melkersson-Rosenthal syndrome” (207 references), “granulomatous
cheilitis” or “cheilitis granulomatosa” (102 references) back to 1956. Full-text journals and
case studies were included, and data synthesis was performed individually.
Results: Etiology remains unclear for all three disease entities. Etiological relatedness to chronic
inflammatory bowel disease is under discussion and effectiveness was found for different treatments,
e.g. local triamcinolone injections, antibiotics, surgical interventions, TNF alpha blockers or
exclusive enteral nutrition. No randomized controlled trial concerning the therapy of orofacial
granulomatosis was found. As a consequence, the therapeutic conclusion is drawn mainly from
small case series, thus limiting the evidence of therapeutic interventions.
Conclusion: OFG with the sub-entities MRS and cheilitis granulomatosa is an etiological obscure
disease process with various possible therapeutic interventions potentially alleviating the disease
course but to broaden treatment knowledge further study in randomized controlled trials is needed.