口腔病理科 On-Line KMU Student Bulletin
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原文題目(出處)： Oral Lesions: The Clue to Diagnosis of Pemphigus
Vulgaris(Hindawi Publishing Corporation Case Reports in Dentistry Volume 2015)
原文作者姓名： Diana Kuriachan, Rakesh Suresh, Mahija Janardhanan, and Vindhya Savithri
通訊作者學校： Amrita School of Dentistry,India 報告者姓名(組別)： Intern F 組 胡瑋凡
報告日期： 105.01.05 內文：
Introduction
1. most common variant of Pemphigus group
2. autoimmune diseases characterized by cutaneous or mucosal blistering 3. 40-50 years old
4. blisters and painful erosion
5. buccal mucosa, lips, and soft palate
6. Diagnosis:clinical manifestations and biopsy.
Case Report of Pemphigus Vulgaris at Multiple Intraoral Sites, with No Involvement of Skin
Age: 55 years old Gender: male
Chief complaint: Painful non-healing ulcers on the left buccal mucosa and left posterolateral border of tongue four months ago
Present Illness:
1. burning sensation at both sites for the past six months
2. blister which appeared and burst rapidly on the buccal mucosa, after which ulcerations appeared on both the sites
Oral Examination:
1. 2 cm × 2 cm ovoid shallow ulcer
with sloping margins along the line of occlusion of 35 to 37 on the left buccal mucosa
口腔病理科 On-Line KMU Student Bulletin
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2. 1 cm × 1 cm ovoid ulcer with yellow crusted surface on the left postero-lateral border of the tongue
→Suspect: Pemphigus, Pemphigoid, Bullous Lichen Planus
Histopathologic Report:1. ulcerated stratified squamous epithelium exhibiting suprabasal split
2. round acantholytic (Tzanck) cells with hyperchromatic nuclei within the split 3. basal cells attached to the underlying connective tissue, below the split
4. dense inflammatory cell infiltrate consisting mainly of plasma cells in the connective tissue
Direct Immunofluorescence Study:
Deposits of IgG and C3 (complement) in a fish-net pattern along the spinous intercellular zone
→Diagnosis confirmed: Pemphigus Vulgaris
Discussion:
1. Intraepithelial blister formation
- blisters in the epithelium where the patients IgG autoantibodies produced in response to triggering factors target two structured proteins of desmosomes : Desmogleins 1 and 3
- new Pemphigus antigen Desmoglein 4 and other non-Desmoglein antigens like human 𝛼-9-acetylcholine receptor that regulates keratinocyte adhesion and keratinocyte annexin like molecules binding acetylcholine termed pemphaxin and catenin
- thin separation at the desmosomal region triggers the acantholysis and suprabasal spilt
口腔病理科 On-Line KMU Student Bulletin
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2. Epidemiology - 40~50 years old - Female
- Initial oral manifestation (more than 50%) - Duration of oral lesion: 3 months~1 years
3. Treatment
- Oral, intralesional, topical corticosteroids - Corticosteroids along with adjuvants
(methotrexate, cyclophosphamide)
- Cholinergic agonists : reverse the acantholysis
4. Our Case
- Oral lesion (blister, and then ulceration) : thin roof of the blister ruptures easily and forms an erosion or ulcer in the area
- Pain and burning sensation : most common symptoms - Multiple intraoral sites : as many reports showed
- Buccal mucosa and tongue : buccal mucosa more common - Treatment :
(i) 100 mg dexamethasone for 3 days with 500 mg of cyclophosphamide, two more cycles of this regime at intervals of 4 weeks each
(ii)30 mg Wysolone tablets during the interim 4-week period
題號 題目
1 下列有關天疱瘡（pemphigus）的敘述，何項錯誤？
(A)與 Nikolsky sign 無關
(B)和表皮細胞間的 desmosomes 有關
(C)和表皮基底層細胞間的 hemidesmosome 無關 (D)組織學變化常見表皮層呈現不同程度的水泡
答案(A) 出處：Oral and Maxillofacial Pathology, third edition
題號 題目
2 下列有關尋常性天疱瘡（pemphigus vulgaris）的敘述，何項錯誤？
(A) 為自體免疫性疾病
(B) 不會藉由垂直感染給胎兒
(C) 使用類固醇治療時需量高時間短(high dose, short course)
(D) 嘴唇上的潰瘍較容易結痂
答案(B) 出處：Oral and Maxillofacial Pathology, third edition
