Is Diagnosis of Melkersson Rosenthal Syndrome Missing?

Yıl 2014, Cilt: 4 Sayı: 2 EK, 100 - 47, 10.09.2014

Seval Dogruk Kacar Pınar Özuğuz , Merve Terzili Çiğdem Tokyol

Öz

Melkersson Rosenthal Syndrome (MRS) is a neuromucocutaneous granulomatous disease characterized by recurrent facial paralysis, orofacial oedema and fissured tongue. The etiopathogenesis is not known yet, but genetic susceptibility, immunocompromised individual, infectious agents, food intolerance, stress are thought to be responsible. Classic triad is seldomly observed. Generally, monosymptomatic or oligosymptomatic findings are seen. The diagnosis of disease is with clinical findings, and histopathology with non-caseous granulomatous cheilitis further supports the diagnosis. We herein report a patient who previously diagnosed and treated as angioedema and cellulitis in different centers, finally diagnosed as MRS with the classic triad; scrotal tongue, recurrent facial paralysis and orofacial edema; and want to call attention to inadvertent physical examination can miss the diagnosis of MRS.

 

Anahtar Kelimeler

Melkersson-Rosenthal syndrome, angioedema, facial paralysis

Melkersson Rosenthal Sendromu Tanısı Atlanıyor Mu?

Öz

Melkersson Rosenthal sendromu (MRS), tekrarlayan fasiyal paralizi, orofasiyal ödem ve fissürlü dil ile karakterize nöromukokutanöz granülomatoz bir hastalıktır. Etyopatogenezi tam olarak bilinmemekle beraber, genetik yatkınlık, immünyetmezlik, enfeksiyöz etkenler, besin intoleransı, stres gibi birçok faktör suçlanmıştır. Klasik triadın görülmesi nadirdir. Genellikle monosemptomatik veya oligosemptomatik tutulum izlenir. Hastalığın tanısı klinik bulgularla konulmakta olup, histopatolojik incelemede non-kazeifiye granülomatöz keilit varlığı tanıyı destekler. Burada farklı merkezlerde anjiyoödem ve selülit tanılarıyla izlenip tedavi alan, skrotal dil, tekrarlayan fasiyal paralizi ve orofasyal ödem triadı ile MRS tanısı konulan hastanın dikkatli fizik inceleme yapılmadığında, MRS tanısının atlanabileceğine dikkat çekilmek istenmiştir.

Anahtar Kelimeler

Melkersson Rosenthal sendromu; anjiyoödem; fasyal paralizi

Kaynakça

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