Melkersson-Rosenthal syndrome (MRS) is a rare neuromucocutaneous syndrome marked 
by the triad of recurrent nonpitting orofacial edema, fissured dorsal tongue 
(lingua plicata), and lower motoneuron facial paralysis. Large case series 
including treatment are limited. A retrospective records review was performed 
for the diagnoses Melkersson-Rosenthal syndrome, granulomatous cheilitis, and 
orofacial granulomatosis, confirmed by noncaseating granulomas on biopsy, at the 
Mayo Clinic in Rochester, Minnesota (1979-2009). There were 72 patients [51 
women (71 %), mean age at presentation 39 years (range 8-79)] identified with 
facial edema with noncaseating granulomas on skin biopsy. Lingua plicata 
occurred in 34 cases (47 %, 95 % confidence interval 35.3-59.3 %). Unilateral or 
partial facial nerve palsy occurred in 14 cases (19.4, 95 % confidence interval 
11.4-30.8 %). Comorbidities among those with facial edema included periodontal 
disease (n = 10, 14 %), history of allergic disease (n = 10, 14 %), Crohn's 
Disease (n = 6, 8 %), migraine headaches (n = 5, 7 %), and systemic lupus 
erythematosus (n = 2, 3 %). There were no patients who had low C1q or C4 levels 
among those who were tested. Overall, the full triad canonical of 
Melkersson-Rosenthal syndrome was observed in nine patients (seven female, 
median age at symptomatic presentation 35 years (range 10-74 years), 13 %, (95 % 
confidence interval 6.2-22.9 %) with a median time from first symptoms to 
diagnosis of 4 years (range 1-35). The medication treatments attempted in the 
nine patients with the full triad of symptoms included non-steroidal 
anti-inflammatory drugs, oral and intra-lesional steroids, metronidazole, 
dapsone, acyclovir, methotrexate, and thalidomide with no consistent treatment 
responses. The Melkersson-Rosenthal syndrome may present over the course of most 
of the lifespan and may require several years of observation to be diagnosed. 
Neurologists who observe a combination of facial edema and facial palsy in a 
patient should consider the diagnosis of MRS and proceed to a diagnostic skin 
biopsy and a trial of steroid treatment for their patient.