Therapeutic response to thalidomide in Melkersson-Rosenthal syndrome: a case report.
BACKGROUND
Melkersson-Rosenthal syndrome (MRS) is a rare disorder of unknown etiology characterized by a triad of symptoms: recurrent orofacial swelling, relapsing facial palsy. and a fissured tongue. A differential diagnosis must be made with other granulomatous diseases, such as sarcoidosis and oral Crohn disease; however, the histologic findings of noncaseating, sarcoidal granulomas support the diagnosis of MRS.


RESULTS
Many therapeutic modalities have been described for this disease. In this case report, we present a patient with MRS that was treated with thalidomide because of the identification of tumor necrosis factor a in the lesion by immunohistochemical analysis. This is the first reported detection of tumor necrosis factor a in an MRS lesion, as well the first reported use of thalidomide to treat this clinical condition.
Medeiros
Manoel
M
Immunology Service, Hospital Universitario Federal University of Bahia, Salvador, Brazil. alergo@zaz.com.br
Araujo
Maria Ilma
MI
Guimarães
Newton Sales
NS
Freitas
Luiz Antonio Rodrigues
LA
Silva
Tania Maria Correia
TM
Carvalho
Edgar Marcelino
EM
D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000627:therapeutic use; D001706:Biopsy; D003907:Dexamethasone / Q000008:administration & dosage / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D008046:Lip / Q000737:chemistry / Q000473:pathology; D008297:Male; D008556:Melkersson-Rosenthal Syndrome / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology; D012074:Remission Induction; D013792:Thalidomide / Q000008:administration & dosage / Q000627:therapeutic use*; D014409:Tumor Necrosis Factor-alpha / Q000032:analysis / Q000037:antagonists & inhibitors*
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D014409:Tumor Necrosis Factor-alpha; D013792:Thalidomide; D003907:Dexamethasone