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

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

Melkersson-Rosenthal sindrom

“…7 The diagnosis is made based on the clinical features and by excluding other autoimmune and infectious diseases with similar symptoms and, in cases that are still not clear, it can include a biopsy and pathohistolic analysis of the affected tissue and the genetic analysis. 8 MRS has a chronic course accompanied by the relapses and remissions, with occasional spontaneous recovery, aided by certain symptomatic procedures. 9 Since the etiology is not known enough, the therapy in cludes a local and/or systemic use of corticosteroids, analgesics, in rare cases imunosupressives and antibiotics, as well as the physical treatment.…”

Melkersson-Rosenthal Syndrome: Case Report

“…Ödemin tekrarlayıcı ve kronik doğası, geçici olan anjiyonörotik ödemden ayrılır. 1,3,5,7,[11][12][13] MRS'nin klasik triadının üçüncü bulgusu olan fissürlü dil (plika linguata), diğer bulgulara göre daha az sıklıkta ve olguların %30-40'ında görül-mektedir. Klinik ataklar sırasında dilde ağrı ve acı hissi ile fissürlerin oluştuğu belirtilmektedir.…”

“…1,5,7,12,18 Chron hastalığı, sarkoidoz, granülomatozis keilitis ve MRS'de yüzdeki ödemli dokuda histolojik olarak aynı granülomatöz görü-nümün olması, bunların aynı hastalığın farklı şe-killeri olabileceğini, immün sistemin bu patogenezde rol oynadığını düşündürmektedir. Bu nedenle MRS tanısı alan hastalar Chron hastalığı ve sarkoidoz gelişimi açısından takip edilmelidir.…”

Orbital inflammatory disease management

“…Treatment for MRS leading to eyelid edema typically involves corticosteroids, and resolution of edema has been reported with both intralesional and systemic administration [225][226][227][228][229], although improvement is often short-lived given the relapsing and remitting nature of the disease.…”

“…Within the orbit and adnexa, MRS usually presents with upper and lower eyelid edema [224][225][226][227][228][229].…”