Melkersson-Rosenthal syndrome - New clinicopathologic findings in 4 cases

Objective: To define the clinicopathologic features of eye-lid 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 patie nts had insidious, painless, nonpitting eyelid edema. Three patients had un ilateral edema; one had bilateral, asymmetric involvement. Ipsilateral lip edema was present in 1 case. Computed tomography demonstrated periorbital h eterogeneous thickening that corresponded to the microscopic finding of sca ttered granulomas. All 4 patients demonstrated epithelioid granulomas insid e and adjacent to dilated lymphatic vessels. Polymerase chain reaction test ing was negative for herpes simplex virus. Conclusions: Isolated eyelid swelling that mimics thyroid-associated ophtha lmopathy may occur in MRS. Computed tomography may be useful in the diagnos is. Biopsy should be performed in all cases of unexplained nonpitting eyeli d edema. In the eyelid, MRS is characterized histopathologically by a granu lomatous lymphangitis, a finding that seems to be unique to this condition.