BACKGROUND: Melkersson-Rosenthal syndrome may manifest as the classical triad 
(orofacial edema, facial nerve palsy and stable lingua plicata) but 
monosymptomatic manifestations or combinations of typical symptoms are not 
infrequent. The available therapeutic options provide only limited success or 
temporary benefit.
CASE REPORT: A 20-year-old man presented with a 7-month history of recurrent 
episodes of swelling of the upper lip without pain, burning or local pruritus. 
No causative factors, such as food, drugs or latex, or physical, chemical or 
emotional conditions could be identified. The patient had been treated with oral 
antihistamines and corticosteroids with no clinical improvement. Physical 
examination showed firm edema without fovea, limited to the central area of the 
upper lip without epidermal changes or symptoms on palpation. The patient had a 
previous history of facial palsy 6 years previously and recurrent episodes of 
herpes simplex labialis. Skin prick tests with inhalant aeroallergens, food, 
latex and Anisakis allergens were negative. Laboratory investigation revealed 
normal complete blood count, erythrocyte sedimentation rate, thyroid hormones, 
biochemistry, complement components (C3, C4 and C1-esterase inhibitor) and CH50, 
rheumatoid factor, antinuclear antibodies, immune complexes, protein 
electrophoresis and immunoglobulins. Thorax and paranasal sinus radiographs were 
clear. Biopsy of the involved area of the lip showed edema with lymphocytic and 
plasma cell infiltration and mononuclear perivascular infiltrates without 
granulomas, suggesting initial granulomatous cheilitis. Because the patient 
showed lack of response and/or poor tolerance to prior treatments (deflazacort, 
clofazimine and metronidazole), intralesional triamcinolone injections were 
administered with satisfactory response from the first session.
CONCLUSIONS: Response to available treatments for Melkersson-Rosenthal syndrome 
is highly variable. In the present case, intralesional triamcinolone injections 
were effective.