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Melkerrson-Rosenthal syndrome is a disease with a triad of clinical features: unilateral facial palsy; orofacial swelling; and a fissured tongue. It is a subset of orofacial granulomatosis. The authors present a case report to increase awareness of the syndrome among general dental practitioners, who may be the first to encounter its manifestations. The management and outcomes of this case are discussed. National and international guidance is lacking owing to the rarity of the condition.
CPD/Clinical Relevance: Early recognition of manifestations of the syndrome is important for timely diagnosis, appropriate referral, and multidisciplinary management to prevent complications.
Article
Melkersson–Rosenthal syndrome (MRS) is an uncommon neuro-mucocutaneous disorder with an unknown cause. It can begin at any age, from early childhood to late adulthood, and is primarily diagnosed through the clinical identification of a triad of symptoms: orofacial swelling; recurrent facial paralysis; and a fissured tongue.1,2,3
Melkersson first observed a female patient with lip oedema and intermittent facial palsy in 1928. Rosenthal subsequently noted the presence of a fissured tongue in these patients in 1931, and the condition was termed Melkerrson–Rosenthal syndrome.4
Oligosymptomatic or monosymptomatic presentations of this syndrome are more common than the classic triad, which occurs in up to 25% of patients.2,3,4,5
The most frequent monosymptomatic presentation of MRS is recurrent lip swelling, known as Miescher's syndrome or Miescher's cheilitis granulomatosa. Histological characteristics of this condition include lymphomonocytic infiltration, non-caseating epithelioid granulomas, multinucleated Langhans-type giant cells and fibrosis.3,6
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