MELKERSSON-ROSENTHAL SYNDROME
Abstract
Background. Granulomatous cheilitis is a rare chronic swelling of the lip due to granulomatous inflammation. Miescher cheilitis is the term used when the granulomatous changes are confined to the lip. Miescher cheilitis is generally regarded as a monosymptomatic form of the Melkersson-Rosenthal syndrome (SMR). SMR is characterized by a triad, including orofacial swelling, facial palsy and lingua plicata. Aetiology of disease is unknown. Virus infection, several other infectious conditions, allergic reactions and Crohn’s disease may be involved in the aetiology of SMR.
Patients and methods. This paper describes a case of 59-yearold male patient with granulomatous cheilitis. Because of swelling of the lip and the cheek allergic patch and prick tests were performed. All tests were negative. Skin biopsy of the affected tissue showed granulomas inflammatory cell infiltration in the dermis. Other systemic granulomatous diseases such a sarcoidosis and tuberculosis were excluded.
Results. A systemic therapy with sulphonyldianilyne 50 mg daily was started. After one year a swelling is less visible.
Conclusions. SMR needs to be recognized by the clinician with persistent swelling of the lip, chin and cheeks.Their duration is short at first, subsiding completely in a few hours or days, and thus simulating angio-oedema. After recurrent attacks at irregular intervals, the oedema becomes persistent. Full confirmation of the diagnosis of SMR requires a histological examination.
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References
Rook, Wilkinson, Ebling. Textbook of dermatology. 5th ed. Oxford: Blackwell scientific publication, 1992: 2767–8.
Muelleger RR, Weger W, Zoechling N et al. Granulomatous cheilitis and Borrelia burgdorferi. Arch Dermatol 2000; 136: 1502–6.
Querings K, Wagner A, Tilgen W. Unterlippenschwellung bei einem 15jährigem Patienten. Hautarzt 2003; 54: 869–70.
Ziem PE, Pfrommer C, Goerdt S, Orfanos CE, Blume-Peytavi U. MelkerssonRosenthal syndrome in childhood: a challenge in differential diagnosis and treatment. Br J Dermatol 2000; 143: 860–3.
Braun-Falco O, Plewig G, Wolff HH, Burgdorf WHC. Dermatology. 2nd ed. Berlin, Heidelberg, New York: Springer Verlag, 1996: 450–0.
International Centers for Excellence in Dentistry, and Eastman Dental Institute for Oral Health Care Sciences, University of London. Cheilitis granulomatosa Miescher-Melkersson-Rosenthal syndrome. http://www.emedicine.com/derm/topic72.htm,marec 2004
Braun-Falco O, Plewig G, Wolff HH, Burgdorf WHC. Dermatology. 2nd ed. Berlin, Heidelberg, New York: Springer Verlag, 1996: 1168–70.
Chen C, Selva D, James C, Huilgol SC. Chronic periorbital swelling in an elderly man. Arch Dermatol 2003; 139: 1075–80.
Neuhofer J, Fritsch P. Cheilitis granulomatosa (Melkersson-Rosenthal-Syndrom): Behandlung mit Clofazimin. Hautarzt 1984; 35: 459–63.
Tausch I, Sönichsen N. Erfahrungen mit der Clofazimin – Therapie des Melkersson-Rosenthal-Syndroms. Hautarzt 1992; 43: 194–8.
Thomas P, Wälchner M, Ghoreschi K, Röcken M. Successful treatment of granulomatous cheilitis with thalidomide. Arch Dermatol 2003; 139: 136–7.
Iwao F, Sawamura D, Yokota K, Shimizu H. Granulomatous blepharitis successfully treated with tranilast. Br J Dermatol 2003; 149: 222–4.
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