Melkersson-Rosenthal Syndrome

Year 1956, Volume: 15 Issue: 1-2, 5 - 12, 12.12.1956

Lütfü Tat

https://izlik.org/JA55WJ39SC

Abstract

A 32-year-old male presented with a two-year history of recurrent swelling of the face, lips, and tongue. Tongue swelling caused speech difficulty; lips were cyanotic and firm-elastic. Buccal mucosa showed lobulation (bucca lobata), gingival edema, and plicated appearance of the hard and soft palate. The tongue demonstrated marked fissures (lingua plicata) and edema. No facial nerve palsy was detected. Biopsy of the lip and tongue revealed edema, acanthosis, dense lymphocytic and epithelioid cell infiltrates, and Langhans-type giant cells. Clinical and histological findings were consistent with Melkersson–Rosenthal syndrome. Although the etiology is unclear, it is thought to be related to granulomatous cheilitis. Cortisone is considered the most effective treatment, but was unavailable; partial improvement was achieved with streptomycin, isonicotinic acid hydrazide, and antihistamines.

Keywords

Melkersson–Rosenthal syndrome , Lingua plicata , Macrocheilia

Melkersson - Rosenthal Syndrom'u

https://izlik.org/JA55WJ39SC

Abstract

32 yaşındaki erkek hasta, iki yıldır tekrarlayan yüz, dudak ve dil şişlikleri ile başvurdu. Dil şişmesi konuşma güçlüğüne yol açıyor, dudaklar siyanoze ve elastik-sert kıvamda bulunuyordu. Yanak mukozasında lobülasyon (bucca lobata), diş etlerinde ödem, sert ve yumuşak damakta plica görünümü, dilde belirgin fissür (lingua plicata) ve ödem vardı. N. facialis felci saptanmadı. Biyopsi, dudakta ve dilde ödem, akantoz, yoğun lenfosit ve epiteloid hücre infiltrasyonu, Langhans tipi dev hücreler gösterdi. Klinik ve histolojik bulgular Melkersson–Rosenthal sendromu ile uyumluydu. Etiyoloji kesin olmamakla birlikte granülomatöz cheilitis ile ilişkili olduğu düşünülmekte, tedavide kortizonun en etkili seçenek olduğu ancak bu olguda uygulanamadığı belirtilmiştir. Streptomisin, izonikotinik asit hidrazid ve antihistaminiklerle kısmi düzelme gözlenmiştir.

Keywords

Melkersson–Rosenthal sendromu , Lingua plicata , Makrokheil

References

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