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Recurrent facial palsy and fissured tongue

      Keywords

      1. Case description

      A 28-year old Chinese man presented with right facial paralysis for 8 days. He felt that for first 3-days, his right face was swollen that subsided after starting oral valaciclovir and prednisolone by his general practitioner. He did not have any pain or swelling of the parotid gland and denied any ear symptom. He had developed right facial paralysis 7-years ago and left facial paralysis 2-years ago, which recovered considerably within a month on both occasions. He did not have any cardiovascular risk factors or immunocompromised state. Examination revealed right lower motor neuron facial paralysis (figure-1A) and a fissured tongue (Fig. 1B). Rest of the clinical examination was unremarkable. With facial physiotherapy, he achieved complete recovery in 3 weeks. What is your diagnosis for this patient?
      Fig. 1
      Fig. 1Clinical presentation in a patient with Melkersson-Rosenthal syndrome. Panel A shows right facial paralysis. Fissured tongue is shown in panel B.

      2. Discussion section

      He was diagnosed as Melkersson–Rosenthal syndrome (MRS), which is a rare neuro-mucocutaneous disease of unknown etiology that presents with orofacial swelling, facial palsy and fissured tongue [
      • Elias M.K.
      • Mateen F.J.
      • Weiler C.R.
      The Melkersson–Rosenthal syndrome: a retrospective study of biopsied cases.
      ]. Onset of this disease is more frequent in young adults, between the second and the third decades of life [
      • Ziem P.E.
      • Pfrommer C.
      • Goerdt S.
      • Orfanos C.E.
      • Blume- Peytavi U.
      Melkersson–Rosenthal syndrome in childhood: a challenge in differential diagnosis and treatment.
      ]. These symptoms may occur individually or simultaneously [
      • Dodi I.
      • Verri R.
      • Brevi B.
      • et al.
      A monosymptomatic Melkersson–Rosenthal syndrome in an 8-year old boy.
      ]. Diagnosis is usually made on clinical presentation. There are no specific biomarkers. However, the diagnosis may be confirmed by histologic findings of lymphomonocytic infiltration, non-caseating epithelioid cell granulomas, multinucleate Langerhans-type giant cells and fibrosis on lip biopsy. The differential diagnosis of MRS includes granulomatous disorders such as Bell's palsy, sarcoidosis, Crohn's disease, Wegener's vasculitis and amyloidosis [
      • Elias M.K.
      • Mateen F.J.
      • Weiler C.R.
      The Melkersson–Rosenthal syndrome: a retrospective study of biopsied cases.
      ,
      • Ziem P.E.
      • Pfrommer C.
      • Goerdt S.
      • Orfanos C.E.
      • Blume- Peytavi U.
      Melkersson–Rosenthal syndrome in childhood: a challenge in differential diagnosis and treatment.
      ]. Isolated swelling of the face or lip is often the most common initial finding and may mimic hereditary or acquired angioedema, a disorder caused by histamine or bradykinin-mediated plasma-leakage affecting subcutaneous and/or submucosal tissue[
      • Cicardi M.
      • Aberer W.
      • Banerji A.
      • et al.
      Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group.
      ].
      There is no definitive therapy for MRS and recurrences are frequent [
      • Elias M.K.
      • Mateen F.J.
      • Weiler C.R.
      The Melkersson–Rosenthal syndrome: a retrospective study of biopsied cases.
      ]. Non-steroidal anti-inflammatory drugs, antihistamines, methotrexate as well as corticosteroids are used with variable results. Occasional patient may require corrective surgical approach if significant facial deformations occur.

      Declaration of Competing Interest

      None declared. I disclose no actual or potential conflict of interest including any financial, personal or other relationships with other people or organizations within last three years.

      References

        • Elias M.K.
        • Mateen F.J.
        • Weiler C.R.
        The Melkersson–Rosenthal syndrome: a retrospective study of biopsied cases.
        J Neurol. 2013; 260: 138-143
        • Ziem P.E.
        • Pfrommer C.
        • Goerdt S.
        • Orfanos C.E.
        • Blume- Peytavi U.
        Melkersson–Rosenthal syndrome in childhood: a challenge in differential diagnosis and treatment.
        Br J Dermatol. 2000; 143: 860-863
        • Dodi I.
        • Verri R.
        • Brevi B.
        • et al.
        A monosymptomatic Melkersson–Rosenthal syndrome in an 8-year old boy.
        Acta Biomed. 2006; 77: 20-23
        • Cicardi M.
        • Aberer W.
        • Banerji A.
        • et al.
        Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group.
        Allergy. 2014; 69: 602-616