An extraordinary form of the Melkersson-Rosenthal syndrome successfully treated with the tumour necrosis factor-α blocker adalimumab.

Abstract

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.

• Authors: Juergen Stein, Alexander Paulke, Beate Schacher, Matthias Noehte
• Journal: BMJ case reports (BMJ Case Rep) Vol. 2014 (May 14 2014) ISSN: 1757-790X [Electronic] England
• PMID: 24827666 (Publication Type: Case Reports, Journal Article)
• Copyright: 2014 BMJ Publishing Group Ltd.

Chemical References

• Anti-Inflammatory Agents
• Antibodies, Monoclonal, Humanized
• Tumor Necrosis Factor-alpha
• Adalimumab

Topics

• Adalimumab
• Adult
• Anti-Inflammatory Agents (therapeutic use)
• Antibodies, Monoclonal, Humanized (therapeutic use)
• Humans
• Male
• Melkersson-Rosenthal Syndrome (complications, drug therapy, pathology)
• Tumor Necrosis Factor-alpha (antagonists & inhibitors)