1/102. Granulomatous cheilitis and Crohn's disease in a 3-year-old boy. Granulomatous cheilitis and Crohn's disease are disorders of unknown etiology. There are case reports describing their coincidence and pointing out the necessity of ruling out systemic disorders once the diagnosis of granulomatous cheilitis is made. However, such reports are few and the causal association of both diseases is controversial in the literature. We report the youngest patient so far, a 3-year-old boy, who had granulomatous cheilitis and Crohn's disease simultaneously. This coincidence so early in life strongly suggests that both represent manifestations of the same disease. ( info) |
2/102. Histopathology and electron and immunofluorescence microscopy of gingivitis granulomatosa associated with glossitis and cheilitis in a case of Anderson-fabry disease. A 17-year-old white boy with signs, symptoms, and family history of angiokeratoma corporis diffusum universale, Anderson-fabry disease (AFD), developed recurrent and then persistent swelling of both lips, erythematous hyperplastic gingivae, and a pebbled tongue. Positive blood findings were raised serum IgE, decreased T-cell level, and increased B-cell level. Histopathology of the gingiva showed noncaseating granulomas with multinucleate giant cells containing Schaumann bodies and large plasma-cell infiltrates in which immunofluorescence demonstrated immune globulins of several classes. Electron microscopy and histochemistry demonstrated ceramide in the vasculature. No glycolipid was found in the macrophages or giant cells of the granulomas which, in contrast, resembled sarcoid reactions. plasma cells with Russell bodies and immune reaction-induced degranulation of mast cells were also identified. The pathogenesis of the oral findings possibly relates to altered immune reactivity associated with damage to the microvasculature analogous to that in melkersson-rosenthal syndrome. ( info) |
| A 30-year-old woman with a 10-year history of recurrent bloody diarrhea and documented colitis of the descending colon, consistent with Crohn's disease, presented with an exacerbation of her gastrointestinal disease and an 18-month history of recurrent facial and genital swelling. Her course evolved to include severe ear pain, dysphagia and colonic dysmotility. She was diagnosed with melkersson-rosenthal syndrome and treated with multiple agents. The neurological aspects of her presentation are highlighted, and the melkersson-rosenthal syndrome is reviewed. ( info) |
| melkersson-rosenthal syndrome is classically described as a triad of orofacial swelling, facial palsy, and fissured tongue. More often this syndrome presents in its oligosymptomatic forms. melkersson-rosenthal syndrome may not be as rare as suspected but rather a syndrome that often goes undiagnosed. Presented is the case of a mentally challenged man who was eventually diagnosed with melkersson-rosenthal syndrome only after being misdiagnosed and incorrectly treated for an odontogenic infection for more than a decade. ( info) |
5/102. An 8-year-old girl with unilateral facial and ear pain and isolated frontal headaches. An 8 1/2-year-old with chronic but fluctuating unilateral facial pain, earache, frontal headache and facial swelling is presented. Her journey through the health care system provides an instructional lesson for all who deal with patients with unusual or difficult to recognize conditions. ( info) |
6/102. melkersson-rosenthal syndrome in childhood: successful management with combination steroid and minocycline therapy. The melkersson-rosenthal syndrome consists of a triad of recurrent lip and/or face swelling, fissured tongue, and intermittent facial palsy. Onset of the symptoms may occur during childhood, and treatment of the condition is difficult. We describe two children with melkersson-rosenthal syndrome in whom combination treatment with prednisone and minocycline proved effective and well tolerated. ( info) |
| An oligosymptomatic manifestation of the melkersson-rosenthal syndrome is described. The importance of serial sections of the tissue specimen is emphasized. Satisfactory postoperative results are obtained. ( info) |
| OBJECTIVE: To define the clinicopathologic features of eyelid involvement in melkersson-rosenthal syndrome (MRS). methods: Four patients with eyelid edema consistent with MRS were evaluated clinically, including diagnostic imaging in 2 patients. Eyelid tissue from these patients was examined by light microscopy and immunohistochemistry. polymerase chain reaction for herpes simplex virus was performed in 1 case. RESULTS: The 3 men and 1 woman ranged in age from 33 to 74 years. All patients had insidious, painless, nonpitting eyelid edema. Three patients had unilateral edema; one had bilateral, asymmetric involvement. Ipsilateral lip edema was present in 1 case. Computed tomography demonstrated periorbital heterogeneous thickening that corresponded to the microscopic finding of scattered granulomas. All 4 patients demonstrated epithelioid granulomas inside and adjacent to dilated lymphatic vessels. polymerase chain reaction testing was negative for herpes simplex virus. CONCLUSIONS: Isolated eyelid swelling that mimics thyroid-associated ophthalmopathy may occur in MRS. Computed tomography may be useful in the diagnosis. biopsy should be performed in all cases of unexplained nonpitting eyelid edema. In the eyelid, MRS is characterized histopathologically by a granulomatous lymphangitis, a finding that seems to be unique to this condition. ( info) |
9/102. Orofacial edema: a diagnostic and therapeutic challenge for the clinician. BACKGROUND AND CONCLUSION: A case of a 41-year-old patient with a 5-year history of chronic recurrent angioedema, refractory to conservative treatment is presented. The results of the case report suggest that in differential diagnosis of angioedema, in addition to usual causes, the allergist-immunologist needs to consider melkersson-rosenthal syndrome, which can present with a variety of symptom-combinations of the classic triad. The distinguishing characteristics of the melkersson-rosenthal syndrome are its refractoriness to the usual anti-inflammatory therapy and the need to consider corrective cosmetic surgery, which may benefit some patients. ( info) |
| melkersson-rosenthal syndrome is a rare disorder consisting of the triad of persistent or recurrent orofacial edema, relapsing facial paralysis and fissured tongue. It is far more uncommon to find the complete triad since it generally presents in oligosymptomatic forms. We present three cases of the melkersson-rosenthal syndrome with the classic triad of symptoms and discuss the etiology and the clinical and electromyography findings of this syndrome. ( info) |