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.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1647494495993706
Highlights
Melkerrson Rosenthal syndrome is a rare neurological disorder characteristically shows swelling of face, lips, facial paralysis and deep plicated tongue [1]
Cheilitis granulomatous of Miescher is considered as monosymptomatic form of MRS
Miescher cheilitis may be a monosymptomatic form of the Melkerrson-Rosenthal syndrome, but probably these are two separate entities
Summary
Melkerrson Rosenthal syndrome is a rare neurological disorder characteristically shows swelling of face, lips, facial paralysis and deep plicated tongue [1]. This classical triad is not seen in all of the patients, majority of the patients often show one or two features only. A 3 mm punch biopsy taken from left lid swelling and submitted for histopathological examination. His past medical history include hernia repair, left knee scope for benign cyst, left shoulder repair. The diagnosis of MRS was suggested based upon chronic eyelids swelling and deep plicated tongue (Figures 4 and 5). Patient was started on steroid therapy and doxycycline, patient responded excellent for both eyelid swelling and rosacea
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