Author/Editor     Balkovec, Valerija; Gabrič-Zirkelbach, Maja
Title     Sindrom Melkersson-Rosenthal
Translated title     Melkersson-Rosenthal syndrome
Type     članek
Source     Zdrav Vestn
Vol. and No.     Letnik 73, št. 12
Publication year     2004
Volume     str. 907-9
Language     slo
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 year old male patient with grartulomatous 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. Pull confirmation of the diagnosis of SMR requires a histological examination.
Summary     Izhodišča. Granulomatozni heilitis je redek vzrok za kronično oteklino ustnic. Če granulomatozno vnetje prizadene samo ustnice, govorimo o cheilitis granulomatosa Miescher (CG), ki lahko nastopa samostojno, večina pa ga ima za monosimptomatsko obliko sindroma Melkersson-Rosenthal (SMR). V bolezensko triado sodijo oteklina ustnice, pareza facialisa in razbrazdan jezik. Etiologija bolezni je nejasna, kot možni vzrok navajajo virusne, bakterijske okužbe in Crohnovo bolezen. Bolnik in metode. Prispevek opisuje 59-letnega bolnika zgranulomatoznim vnetjem ustnice. Zaradi otekline ustnice in lica so bili opravljeni alergološki epikutani in prick testi. Vsi testi so bili negativni. Biopsija prizadetega tkiva je pokazala granulomatozno celično vnetje v dermisu. Nekatere sistemske granulomatozne bolezni, kot npr. sarkoidoza in tuberkuloza, so bile izključene. Rezultati. Po zdravljenu s sulfonyldianilinom se je oteklina ustnic zmanjšala. Občasno še pride do otekline lica, ki se delno odzove na antihistaminike v parenteralni obliki. Zaključki. Pri ireverzibilni oteklini ustnic, brade in lic moramo med drugim pomisliti tudi na SMR. V začetku pride do regresije otekline v nekaj urah ali dneh, kar je značilno za angioedem. Po obdobju pojavljanja oteklin v nepravilnih presledkih sčasoma postanejo trajne. Dokončno diagnozo potrdimo s histološkim izvidom.
Descriptors     MELKERSSON-ROSENTHAL SYNDROME
DAPSONE
MIDDLE AGE