Melkersson-Rosenthal syndrome

Melkersson-Rosenthal syndrome (MRS) is a rare neuromucocutaneous disorder characterized by a classical triad of symptoms: recurrent orofacial edema (particularly swelling of the lips), relapsing peripheral facial nerve palsy (Bell's palsy), and fissured tongue (lingua plicata). The condition was first described by Melkersson in 1928 and further characterized by Rosenthal in 1931. In practice, the complete triad is present in only a minority of patients; many individuals present with oligosymptomatic forms, with recurrent lip swelling being the most common and often the earliest manifestation. The condition primarily affects the nervous system and the orofacial soft tissues. Granulomatous cheilitis (Miescher cheilitis) — persistent, non-tender swelling of one or both lips due to granulomatous inflammation — is considered a monosymptomatic variant of MRS. The syndrome typically begins in childhood or young adulthood, with episodes that may recur at irregular intervals. The facial palsy can be unilateral or bilateral and may become permanent after repeated episodes. Additional features can include migraine-like headaches, cranial nerve involvement beyond the facial nerve, and occasionally other neurological symptoms. Histopathologically, affected tissues often show non-caseating epithelioid granulomas, perivascular lymphocytic infiltration, and edema. The exact etiology remains unclear, though genetic predisposition, immune dysregulation, and possible infectious triggers have been proposed. Treatment of Melkersson-Rosenthal syndrome is largely symptomatic and can be challenging. Corticosteroids (systemic or intralesional) are the most commonly used first-line therapy to reduce inflammation and swelling. Other immunosuppressive or immunomodulatory agents such as methotrexate, clofazimine, dapsone, thalidomide, and anti-TNF biologics have been used with variable success in refractory cases. Surgical reduction (cheiloplasty) may be considered for persistent, disfiguring lip swelling that does not respond to medical therapy. Facial nerve palsy episodes are managed similarly to idiopathic Bell's palsy, with corticosteroids and supportive care. There is currently no cure, and the clinical course tends to be chronic and relapsing.

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