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

Kajal B, Harvey J, Alowami S - Diagn Pathol (2013)

Bottom Line: As this entity has been associated with numerous other clinical entities, diagnosis often remains an exclusion process.Polarization failed to reveal any foreign body.Association with rosacea was other findings.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of pathology and molecular medicine, McMaster University, 1280 Main street west, Hamilton, Ontario L8N 3Z5, Canada. babita.kajal@medportal.ca.

ABSTRACT

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

Show MeSH

Related in: MedlinePlus

Dermal edema, peri-lymphatic infiltrate and noncaseating granuloma.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4225747&req=5

Figure 1: Dermal edema, peri-lymphatic infiltrate and noncaseating granuloma.

Mentions: A 51-year-old male presented with clinical symptoms of bilateral heavy lids, predominantly on left side of one-year duration. No itchiness, no redness was found. Working clinical diagnosis of allergy, facial rosacea, or blepharocalasis was made. 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. Family history was remarkable for colon cancer, but colonoscopy was normal for patient. No other significant medical history given in chart. In our department we received a left eye lesion skin biopsy, measuring 0.3x0.3x0.3 cm, submitted in one block. Microscopic sections revealed unremarkable epidermis with mild degree of hyperkeratosis. Underlying dermis showed edema, perivascular lymphocytic infiltrate and well defined perilymphatic non- necrotizing granulomas. Descriptive diagnosis of “edema with mild chronic inflammation and focal perilymphatic granulomas” was given (Figures 1, 2, 3). Fungal, bacterial and mycobacterium stains were negative. Polarization failed to reveal foreign body. The diagnosis of MRS was suggested based upon chronic eyelids swelling and deep plicated tongue (Figures 4 and 5). The serology was negative for c ANCA, p ANCA, angiotensin converting enzyme, and cyclic citrullinated peptide antibody. Patient was started on steroid therapy and doxycycline, patient responded excellent for both eyelid swelling and rosacea.


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

Kajal B, Harvey J, Alowami S - Diagn Pathol (2013)

Dermal edema, peri-lymphatic infiltrate and noncaseating granuloma.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4225747&req=5

Figure 1: Dermal edema, peri-lymphatic infiltrate and noncaseating granuloma.
Mentions: A 51-year-old male presented with clinical symptoms of bilateral heavy lids, predominantly on left side of one-year duration. No itchiness, no redness was found. Working clinical diagnosis of allergy, facial rosacea, or blepharocalasis was made. 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. Family history was remarkable for colon cancer, but colonoscopy was normal for patient. No other significant medical history given in chart. In our department we received a left eye lesion skin biopsy, measuring 0.3x0.3x0.3 cm, submitted in one block. Microscopic sections revealed unremarkable epidermis with mild degree of hyperkeratosis. Underlying dermis showed edema, perivascular lymphocytic infiltrate and well defined perilymphatic non- necrotizing granulomas. Descriptive diagnosis of “edema with mild chronic inflammation and focal perilymphatic granulomas” was given (Figures 1, 2, 3). Fungal, bacterial and mycobacterium stains were negative. Polarization failed to reveal foreign body. The diagnosis of MRS was suggested based upon chronic eyelids swelling and deep plicated tongue (Figures 4 and 5). The serology was negative for c ANCA, p ANCA, angiotensin converting enzyme, and cyclic citrullinated peptide antibody. Patient was started on steroid therapy and doxycycline, patient responded excellent for both eyelid swelling and rosacea.

Bottom Line: As this entity has been associated with numerous other clinical entities, diagnosis often remains an exclusion process.Polarization failed to reveal any foreign body.Association with rosacea was other findings.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of pathology and molecular medicine, McMaster University, 1280 Main street west, Hamilton, Ontario L8N 3Z5, Canada. babita.kajal@medportal.ca.

ABSTRACT

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

Show MeSH
Related in: MedlinePlus