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[Melkersson-Rosenthal syndrome].

F Cousin1, P Grezard, F Berard

  • 1Service de Dermatologie, Hôpital de l'Antiquaille, Lyon.

Annales De Medecine Interne
|February 18, 1999
PubMed
Summary

Melkersson-Rosenthal syndrome is a rare disorder featuring orofacial swelling, facial palsy, and a plicated tongue. Diagnosis involves clinical signs, with histopathology showing granulomas and lymphangitis.

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Area of Science:

  • Dermatology
  • Neurology
  • Pathology

Context:

  • Melkersson-Rosenthal syndrome (MRS) is a rare neurological disorder.
  • Complete MRS presents with orofacial swelling, peripheral facial palsy, and a plicated tongue.
  • Incomplete forms are common and diagnosed using established clinical features.

Purpose:

  • To describe the clinical and histopathological features of Melkersson-Rosenthal syndrome.
  • To differentiate between complete and incomplete forms of the syndrome.
  • To discuss potential etiologies and treatment options for MRS.

Summary:

  • MRS is characterized by recurrent orofacial swelling, peripheral facial palsy, and a plicated tongue.
  • Histopathology reveals non-caseating epithelioid cell granulomas and obliterant epithelioid lymphangitis.
  • Miescher's granulomatous macrocheilitis is considered a monosymptomatic presentation of MRS.

Impact:

  • Enhances understanding of a rare condition, aiding in diagnosis and management.
  • Highlights the importance of histopathological findings in confirming the diagnosis.
  • Suggests a potential link with Crohn's disease, warranting further investigation.

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