Is Diagnosis of Melkersson Rosenthal Syndrome Missing?

Year 2014, Volume: 4 Issue: 2 EK, 100 - 47, 10.09.2014

Seval Dogruk Kacar Pınar Özuğuz , Merve Terzili Çiğdem Tokyol

Abstract

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.

 

Keywords

Melkersson-Rosenthal syndrome, angioedema, facial paralysis

Melkersson Rosenthal Sendromu Tanısı Atlanıyor Mu?

Abstract

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.

Keywords

Melkersson Rosenthal sendromu; anjiyoödem; fasyal paralizi

References

• Ziem PE, Pfrommer C, Goerdt S et al. Melkersson-Rosenthal Syndrome in childhood: a challenge in differential diagnosis and treatment. Br J Dermatol 2000;143:860-863
• Rogers RS 3rd. Granulomatous cheilitis, Melkersson-Rosenthal Syndrome and orofacial granulomatosis. Arch Dermatol 2000;136:1557-8
• Shaphiro M, Peters S, Spinelli HM. Melkersson-Rosenthal Syndrome in the periocular area: a review of the literature and case report. Ann Plast Surg 2003;50:644-8
• Camacho-Alonso F, Bermejo-Fenoll A, Lopez-Jornet P. Meiescher’s cheilitis granulomatosa. A presentation of five cases. Med Oral Patol Oral Cir Bucal 2004;9:427-429
• Sciubba JJ, Said-Al-Naief N. Orofacial granulomatosis: Presentation, Pathology and Management of 13 cases. J Oral Pathol Med 2003;32:576-585.
• Stein S, Mancini AJ. Melkersson-Rosenthal Syndrome in childhood: Successful management with combination steroid and minocycline therapy. J Am Acad Dermatol 1999;41:746-748
• Litvyakova LI, Bellanti JA. Orofacial edema. A diagnostic and therapeutic challenge for the Clinician. Ann Allergy Asthma Immunol 2000;84:188-192.