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Open Access Highly Accessed Case Report

Melkerrson-Rosenthal Syndrome, a rare case report of chronic eyelid swelling

Babita Kajal1*, John Harvey2 and Salem Alowami1

Author Affiliations

1 Department of pathology and molecular medicine, McMaster University, 1280 Main street west, Hamilton, Ontario L8N 3Z5, Canada

2 Department of ophthalmology, McMaster University, 1280 Main street west, Hamilton, Ontario L8N 3Z5, Canada

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Diagnostic Pathology 2013, 8:188  doi:10.1186/1746-1596-8-188

Published: 13 November 2013

Abstract

Melkerrson-Rosenthal syndrome is a rare disorder of unknown etiology. The classical triad of recurrent facial paralysis, swelling of the face, lips and deep furrowed tongue (Lingua Plicata) is seen in very few cases, majority of the patients often present with one or two symptoms only, which often leads to misdiagnosis and mismanagement. Clinically these symptoms vary from days to years, which further delay the definitive diagnosis and symptoms may eventually become permanent. The cause of this entity is not very well understood, but thought to be attributable to multiple entities including genetic and Infectitious. As this entity has been associated with numerous other clinical entities, diagnosis often remains an exclusion process. Methods: A middle age male with a chronic history of heavy eyelids with skin indurations predominately around left eye was presented to ophthalmology clinic. Physical examination revealed a deep furrowed tongue. The skin biopsy from left eyelid revealed a non-specific granulomatous lesion. The clinical correlation of facial swelling and deep plicated tongue prompted the differential of MRS Results: Histologically a non-specific granulomatous lesion was seen in dermis. As a rule, other causes of granulomatous diseases were ruled out especially Crohn’s disease and sarcoidosis. Polarization failed to reveal any foreign body. Conclusions: The finding of granulomatous lesion and clinical picture led to the definite diagnosis of Melkerrson-Rosenthal syndrome. Association with rosacea was other findings. Clinically his sign and symptoms are under control and no occurrence of symptoms has been noted so far.

The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1647494495993706 webcite

Keywords:
Melkerrson Rosenthal syndrome; Granulomatous disease