Melkersson-Rosenthal syndrome with isolated unilateral eyelid edema: an immunopathologic study.
Lymphedema is caused by defective drainage of the lymphatic system. In Melkersson-Rosenthal syndrome, involvement is predominantly of the lumens with blockage of lymphatic channels by histiocytic-epithelioid cell clusters accompanied by dermal granulomas and lymphocytes. It is a localized, painless, nonitching, and nonpitting form of lymphedema. Besides the eyelids, the disease can cause lip edema, facial palsy, and/or fissured tongue. It is rare and has received little attention in the ophthalmic literature, either in its complete triadic form, or more frequently, in its monosymptomatic forms. Pathogenesis is not well understood, and there is no effective therapy. The authors describe a case of Melkesson-Rosenthal syndrome in a 45-year-old Hispanic man with isolated unilateral upper eyelid edema. Histopathological and immunohistochemical evaluations of an eyelid biopsy specimen revealed intravascular and extravascular clusters of histiocytic-epithelioid cells that were CD68/163-positive. Variable numbers of mostly T-lymphocytes were found in the epidermis, dermis, and orbicularis muscle and by virtue of the associated granulomas established the diagnosis of Melkersson-Rosenthal syndrome. CD4 helper and CD8 suppressor T-lymphocytes were equally represented. CD20 B-lymphocytes were exceedingly sparse. Conspicuous CD1a-positive Langerhans' cells were present in the epidermis, sometimes formed subepithelial loose aggregates and were also incorporated in the granulomas. The differential diagnosis includes the far more common condition of acne rosacea. Management of Melkersson-Rosenthal syndrome, and of angioedema in general, is reviewed.
*Department of Ophthalmology, David G. Cogan Laboratory of Ophthalmic Pathology, †Department of Ophthalmology, Eye Plastic, Orbital, and Cosmetic Surgery Service, Massachusetts Eye and Ear Infirmary; and ‡Harvard Medical School, Boston, Massachusetts, U.S.A.
Xi
X
Chen
Frederick A
FA
Jakobiec
Prashant
P
Yadav
Xiang Q
XQ
Werdich
Aaron
A
Fay
D018949:Antigens, CD1 / Q000378:metabolism; D018951:Antigens, CD20 / Q000378:metabolism; D018414:CD8-Positive T-Lymphocytes / Q000473:pathology; D004487:Edema / Q000276:immunology / Q000473:pathology*; D005141:Eyelid Diseases / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007801:Langerhans Cells / Q000378:metabolism / Q000473:pathology; D008297:Male; D008556:Melkersson-Rosenthal Syndrome / Q000276:immunology / Q000473:pathology*; D008875:Middle Aged; D006377:T-Lymphocytes, Helper-Inducer / Q000473:pathology
D018949:Antigens, CD1; D018951:Antigens, CD20; C419393:CD1a antigen