1/4. melkersson-rosenthal syndrome: clinical, pathologic, and therapeutic considerations.melkersson-rosenthal syndrome (MRS) has been described as a rare disease. Localized swelling (usually of the lips) with noncaseating epithelioid granuloma is necessary for diagnosis. We report 11 patients with biopsy-proven MRS. Mean age was 28 years (range 12-47 years). Mean duration of lesions was 4.4 years. lip biopsy confirmed typical sarcoid-like granuloma. Five additional patients with typical symptoms are awaiting or refused lip biopsy. Clinically all patients presented with localized swelling of the lips. Treatment with clofazimine (100 mg 4 times weekly for 3-11 months) was associated with complete remission in 5/10 patients and clinical improvement in 3/10 patients with moderate constant swelling. Two patients (one severe and one moderate swelling) failed to respond to treatment. Rebiopsy in four patients confirmed improvement with disappearance or decreased granulomatous inflammation in 3/4. Complete spontaneous remission was also seen in three untreated patients. We conclude MRS is not uncommon, clofazimine may be effective treatment in moderate lesions and spontaneous remissions can occur.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/4. Treatment and follow-up of persistent granulomatous cheilitis with intralesional steroid and metronidazole.Granulomatous cheilitis (GC) is a chronic edema which frequently affects the upper lip due to granulomatous inflammation. Its etiology is currently unknown. This rare disease is generally accompanied by melkersson-rosenthal syndrome (MRS), characterized by scrotal tongue, orofacial edema and facial paralysis. However, it is also known to develop only with orofacial edema. Granulomatous cheilitis is a difficult disease to treat because of recurrences. There are contradictory reports about the results of treatment without surgical intervention and the rates of recurrence. Our case was a 57-year-old female patient who was characterized by orofacial edema only. The edema and erythema had persisted for 1 year before admission. In the present case, application of intralesional corticosteroid treatment as a total of three injections over 3 consecutive months (one injection per month) and the accompanying metronidazole treatment brought about successful results. No recurrence was observed in the follow-up.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/4. melkersson-rosenthal syndrome in a patient with sero-negative oligoarthritis.The melkersson-rosenthal syndrome is a rare disease consisting of orofacial swellings, facial nerve palsy and plicated tongue. The etiology is unknown. On histopathological examination a characteristic granulomatous inflammation is found. A case of this syndrome in a young white female with seronegative oligoarthritis is described and the possible relationship between the two diseases is discussed on the basis of the literature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/4. melkersson-rosenthal syndrome: a case report.The melkersson-rosenthal syndrome (MRS) is a rare disease which includes a triad of orofacial swelling, recurrent facial nerve paralysis and plicated tongue. MRS can still be diagnosed when any two of the conditions are present. Because of its rarity, it is usually ignored and misdiagnosed. Herein, a diabetic female patient with this syndrome, previously diagnosed as Bell's palsy, is described. This case report is presented to encourage awareness of this entity among medical practitioners. In addition, the MRS literature is reviewed with respect to incidence, etiology, clinical features, pathology, differential diagnosis and treatment.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |