• 沒有找到結果。

Melkersson-Rosenthal syndrome (MRS) is a rare, noncaseating

N/A
N/A
Info
下載
Protected

Academic year: 2022

Share "Melkersson-Rosenthal syndrome (MRS) is a rare, noncaseating "

Copied!
3
0
0

加載中.... (立即查看全文)

立即下載 ( 3 頁 )

全文

(1)

口腔病理科 On-Line KMU Student Bulletin

- 1 -

原文題目(出處)： Melkersson-Rosenthal syndrome revisited as a

misdiagnosed disease. Am J Otolaryngol 2009;30:33-7 原文作者姓名： Ozgursoy OB, Ozgursoy SK, Tulunay O, Kemal O, Akyol

A, Dursun G 通訊作者學校： Ankara University 報告者姓名(組別)： 李羽婕(INTERN A組) 報告日期： 98.1.16

內文：

Introduction:

z

Melkersson-Rosenthal syndrome (MRS) is a rare, noncaseating

granulomatous disease

z Classic triad: orofacial edema, facial paralysis, lingua plicata (scrotal, fissure or furrowed tongue)

z Etiology: unknown

z Possible cause:infectious disease, genetic ,allergy and benign lymphologranulomatosis

z Histopathological feature: lymphoedema, noncaseating epitheloid cell

granulomas, multinucleated Langhans-type giant cell, perivascular mononuclear inflammatory infiltration, and fibrosis

z Propose of study: current diagnosis, Dr.’s attention

Materials and methods

Retrospective review of p’t database

Results

3 patients were found Patient 1 : 44y/o female

clinical feature

Recurrent right peripheric facial paralysis (PFP), House-Brackmann grade 6 Swollen upper and lower lip, furrowed tongue

(2)

口腔病理科 On-Line KMU Student Bulletin

- 2 -

Histiopathology feature (upper lip)

Lymphocytic infiltration, submucosal edema Treatment

Facial nerve decompression via transmastoid approach Medication:1mg/kg methylprednisolone

F/U:

2 month after: PFP regress to HB3

6 month after: syndrome regressed, except HB3 PFP recur on right side 1, 3, 7 years after surgery No recurred during last 3 yr

Patient 2: 32y/o male clinical feature

lower lip edema, fissure tongue, diarrhea, burning sensation in right eye (uveal ectropion, visual loss)

Treatment

Intralesional corticosteroid treatment (triamcinolone,10mg/mL) F/u

Lip edema regression 1 month after injection

Recurrent PFP: 1990, 1991, 2002(right) 1993, 1994(left) No recurred during last 1 year

Patient 3: 21y/o male clinical feature

Upper lip edema, fissure tongue, periorbital edema , hives, rhinitis, conjunctivitis No PFP

Histopathology feature

Non caseified granulomatous morphology, granulomatous cheilitis Treatment

Intralesional corticosteroid injection Regression 3 weeks after injection

Discussion

z Diagnosis is difficult because triad presence only8~18%

z Most dominant manifestation:facial edema, acute, diffuse, painless, mostly upper lip

z Does not respond to antihistamines z Can lead to fibrosis

z Facial paralysis occur months to years before or after facial edema z PFP could be uni or bilateral, partial or complete

z Lingua plicata has been considerate as congenital

z Biopsy exclude Crohn disease and sarcoidosis (類肉瘤症)

z Other finding:trigeminal neuralgia,paresthesias(皮膚異常), ocular palsies(眼部 癱瘓), blepharospasm(眼瞼痙攣) , epiphora(淚漏) keratits(角膜炎), migraine z Treatment:systemic or intralesional corticosteroids

題號 題目

1 下列何者非Melkersson-Rosenthal syndrome的特徵 (A) 臉部水腫(facial edema)

(B) 顏面麻痺癱瘓(facial paralysis) (C) 溝紋舌(fissure tongue)

(D) 扁平苔蘚(lichen planus)

(3)

口腔病理科 On-Line KMU Student Bulletin

- 3 -

答案 (D )

出處：Oral and maxillofacial pathology 2nd edition,p12,741,294

題號 題目

2 以下對Melkersson-Rosenthal syndrome的敘述何者錯誤

(A) 只會發作一次就終身免疫

(B) 目前以口服或施打抗生素做症狀治療

(C) 最常出現的顏面腫脹部位為上唇

(D) 發病的機制目前尚未明瞭

答案 (A )

出處：Head and neck medicine and surgery 30(2009)33-37 註:House-Brackmann facial nerve grading system

Grade Definition I Normal symmetrical function in all areas

II Slight weakness noticeable only on close inspection Complete eye closure with minimal effort

Slight asymmetry of smile with maximal effort

Synkinesis barely noticeable, contracture, or spasm absent III Obvious weakness, but not disfiguring

May not be able to lift eyebrow

Complete eye closure and strong but asymmetrical mouth movement with maximal effort

Obvious, but not disfiguring synkinesis連帶運動, mass movement or spasm痙攣

IV Obvious disfiguring weakness Inability to lift brow

Incomplete eye closure and asymmetry of mouth with maximal effort Severe synkinesis, mass movement, spasm

V Motion barely perceptible

Incomplete eye closure, slight movement corner mouth Synkinesis, contracture, and spasm usually absent

VI No movement, loss of tone, no synkinesis, contracture緊縮, or spasm 註:sarcoidosis

Multisystem granulomatous disorder

Clinical feature:dyspnea呼吸困難,dry cough, chest pain, fever, malaise,fatique Pulmonary symptoms are most common

Histopathologic feature:non-caseating granulomatous inflammation,giant cell,histiocytes aggregation

參考文獻

立即下載 ( PDF - 3 頁 - 226.92 KB )

相關文件

原文作者姓名原文作者姓名原文作者姓名原文作者姓名：：：：

Sjögren’s syndrome is a chronic, systemic autoimmune disorder involves salivary and lacrimal glands resulting in xerostomia(dry mouth), xerophthalimia (dry

Introduction Cowden syndrome (CS), also called multiple hamartoma syndrome, is a rare autosomal dominant genetic condition. This change is associated with mutations in the

Among the diseases that cause by mouth papillomatous lesions, the main disease that should be included in the differential diagnosis of CS is Heck’s Disease, characterized by

Seven-Year Follow-Up of Peutz-Jeghers Syndrome

Peutz-Jeghers syndrome (PJS) is a rare inherited autosomal dominant disease with an incidence of 1 in 12–30,000 live births, characterized by mucocutaneous pigmentation and

A Case of Lacrimo-Auriculo-Dento-Digital Syndrome with Multiple Congenitally Missing Teeth

Lacrimo-auriculo-dento-digital (LADD) syndrome, also known as Levy-Hollister syndrome, is a rare genetic disorder characterized by anomalies affecting the lacrimal and salivary

文題目

Lacrimo-auriculo-dento-digital (LADD) syndrome, also known as Levy-Hollister syndrome, is a rare genetic disorder characterized by anomalies affecting the lacrimal and

Multiple Dentigerous Cysts as a Rare Presentation of Maroteaux–Lamy Syndrome

Although Maroteaux–Lamy syndrome is not encountered routinely in dental practice, unerupted dentition with dentigerous cyst formation can be rare presentation of the syndrome

Introduction Casereport Abstract Alargedevelopingcomplexodontome

We report a rare case of a large developing complex odontoma in a young girl causing expansion of bone and facial asymmetry with the failure of eruption of several mandibular

Ramon Syndrome‑ A Rare Form of CherubismL. K. Surej Kumar, D. S. Deepa, S. DilnaDepartment of Oral and Maxillofacial Surgery, KIMS Hospital, Thiruvananthapuram, Kerala, India

The association of cherubism with gingival fibromatosis, epilepsy, mental retardation, stunted growth, and hypertrichosis is referred to as a rare case of possible Ramon syndrome