An extraordinary form of the Melkersson-Rosenthal syndrome successfully treated with the tumour necrosis factor-α blocker adalimumab.
Melkersson-Rosenthal syndrome (MRS) is a rare granulomatous inflammatory disease characterised by the triad of orofacial oedema, facial nerve palsy and furrowed tongue. We describe the case of a 29-year-old patient suffering from an oligosymptomatic form of the disease with orofacial oedema, cobblestone pattern on the buccal mucosa and swelling of the tongue, accompanied by intermittent fatigue, influenza-like symptoms, intermittent tinnitus and acute hearing loss. An increase of several autoimmune-associated antibodies was also detected. Treatment with prednisolone, azathioprine or methotrexate failed to adequately control all symptoms in the long term. In the absence of a specific and well-established therapy for MRS, treatment with adalimumab was administered. Under adalimumab, total remission of all symptoms was achieved, indicating that tumour necrosis factor-α blockers are a promising therapeutic option for patients with Melkersson-Rosenthal syndrome.
Department of Internal Medicine, Krankenhaus Sachsenhausen, Frankfurt/Main, Germany.
Juergen
J
Stein
Institute of Legal Medicine, J W von Goethe University Frankfurt, Frankfurt/Main, Germany.
Alexander
A
Paulke
Department of Periodontology, Centre for Dental, Oral and Maxillofacial Medicine (Carolinum), J W von Goethe University Frankfurt, Frankfurt/Main, Germany.
Beate
B
Schacher
Department of Rheumatology, Liebig-University Giessen, Giessen, Germany.
Matthias
M
Noehte
D000068879:Adalimumab; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D008556:Melkersson-Rosenthal Syndrome / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors*
D000893:Anti-Inflammatory Agents; D061067:Antibodies, Monoclonal, Humanized; D014409:Tumor Necrosis Factor-alpha; D000068879:Adalimumab