• 대한기관식도과학회지
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• 제10권2호
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• pp.72-75
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• 2004

Pemphigus vulgaris is a rin, chronic intraepidermal bullous disease with potentially fatal outcome. Oral lesions precede skin lesions in at least $70\%$ of cases, and in cutaneous disease, concomitant oral lesions are encountered in $90\%$ of patients. This disorder involve the skin and mucous membranes, especially the oral and pharyngeal mucosa, but may also involve the nasal, oropharyngeal, laryngeal and esophageal mucosa. Oral lesions are initially vesicobullous but rapidly rupture, leaving a painful erosion that shows little tendency to heal. Pemphigus vulgaris affecting the oral mucosa is still diagnosed only after considerable delay, because oral ulceration in common, and clinicians believed the lesions to be caused by more common conditions such as recurrent aphthous stomatitis rather than a rare disorder such as pemphigus vulgaris. The definitive diagnosis of pemphigus vulgaris should be undertaken as early as possible, so that treatment can be started at an earl·y stage. Because of the presence of nonspecific oral ulcer, high degree of suspicion is often required to ultimately make the diagnosis of pemphigus vulgaris and then we report a case of pemphigus vulgaris with a literature review.

• 한방안이비인후피부과학회지
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• 제29권2호
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• pp.112-122
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• 2016

Objectives : The purpose of this report is to know the effect of Korean medical treatments on pemphigus vulgaris with generalized pruritus. Methods : We treated a 44-years-old woman patient with pemphigus vulgaris on the whole body with Korean medicine. After Korean medical treatment for 17 weeks, we measured the extinction of blisters and decrease of pruritus. We recorded pictures of changes on symptoms. Results & Conclusions : The symptoms of pemphigus vulgaris were significantly improved. The blisters and itching were decreased and skin damages were almost recovered. Thus Korean medical treatments are effective on pemphigus vulgaris.

• Archives of Plastic Surgery
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• 제38권5호
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• pp.687-690
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• 2011

Purpose: Pemphigus vulgaris, a rare autoimmune blistering disease of the skin and mucous membranes remains a challenging disease to treat. Management is focused on immunotherapy against autoimmune antibodies that target keratinocyte cell adhesion molecules, and antibiotics preventing secondary infections. There is no established dressing protocol and skin is usually manipulated the least amount possible in order to minimize irritation. The authors suggest that early initiation of aggressive bathing and debridement of skin lesions, with nutritional support, is essential in accelerating resolution. Methods: A 40 year-old male previously diagnosed with pemphigus vulgaris was admitted due to exacerbation of mucocutaneous lesions involving the epidermis and mucosa of the whole body. Steroids, immunosuppressants, intravenous immunoglobulin and antibiotics were administrated, but infection and de-epithelialization progressed, while his general condition deteriorated with a weight loss of over 20 kilograms. The plastic surgery department intervened with daily bathing, debridement of unhealthy debris and non-traumatizing coverage of growing epithelium. Total parenteral nutrition and mobilization with rehabilitation therapy was initiated as early as possible. Results: After bathing, healthy epithelium gradually covered the patient's entire body, while his general condition improved with a corresponding weight gain of 14 kgs. Conclusion: Treatment of pemphigus vulgaris focuses on immunotherapy and infection control. However, an equal amount of attention should be laid on early intervention with daily dressings including bathing and irrigation, nutritional support, and exercise as this accelerates resolution of existing infections, promotes healthy epithelialization and leads to faster recovery.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제31권5호
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• pp.414-418
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• 2009

Pemphigus vulgaris is a chronic autoimmune intraepithelial blistering disease with oral mucosal manifestations that very often precede the skin lesions. The vesicles or bullae are produced by an acantholytic process, detachment of differentiating keratinocytes from one another in the epithelial stratum spino sum or spinous cell layer. The pathogenesis of this disease is initially manifested by IgG(mainly) binding to desmosome(desmoglein 3 or 1) in the intercellular spaces of epithelium. This autoantibody binding caused the release of a plasminogen activator(a proteolytic enzyme) from keratinocytes. This ultimately results in cell to cell separation. The mainstay therapy of pemphigus vulgaris is systemic corticosteroids and immunosuppressive agents to eliminate the pathogenic autoantibodies from circulation. A 41-year old woman presented with a 1.5 year history of oral ulceration. There were no lesions on the skin or other mucosal sites. Histology and immunostaining were consistent with pemphigus vulgaris. Control of oral ulceration and normal oral function were achieved after systemic corticosteroids and immunosuppressive agents were instituted.

• Journal of Oral Medicine and Pain
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• 제48권4호
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• pp.174-180
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• 2023

Pemphigus vulgaris (PV) is a chronic autoimmune bullous disease caused by autoantibodies to proteins in the oral mucosa and skin. It is a rare disease with an annual incidence of 2.059 per million in South Korea. In many patients with PV, oral mucosal lesions precede other lesions elsewhere, and oral lesions can be the only manifestation. Early diagnosis is important because the disease has a high mortality rate if untreated appropriately in the early stages, and rapid treatment initiation is associated with rapid disease control. Oral PV lesions are clinically variable. In this study, we describe oral PV lesions in a 60-year-old woman, a 75-year-old man, and a 60-year-old man presenting with various clinical presentations. Oral PV lesions can affect any part of the oral mucosa, including the buccal mucosa, gingiva, tongue, palate, and free mucosa, and can vary in appearance from desquamative gingivitis, painful ulcers, and erosions to aphthous-like stomatitis. Clinicians should be aware of the difficulty of early diagnosis in PV, particularly when oral lesions are the only manifestation, and should consider many factors, including the patient's age, to make an accurate diagnosis and manage oral lesions to improve the patient's quality of life and avoid delayed diagnosis.

• 대한치과의사협회지
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• 제22권4호통권179호
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• pp.268-268
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• 1984

• Applied Microscopy
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• 제17권2호
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• pp.1-22
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• 1987

Pemphigus vulgar교의 본태(本態)를 알아보기 위한 일환으로 본질병(本疾病)의 병변조직(病變組織)을 전자현미경적(電子顯微鏡的)으로 관찰(觀察)하고 본질병(本疾病)에 관여(關與)하는 세포(細胞)들에 대(對)해 면역전자현미경적(免疫電子顯微鏡的)으로 추구(追究)하였던 바, 그 결과(結果)를 보고(報告)하는 바이다. 견(犬)의 pemphigus vulgaris의 구강(口腔) 및 식도(食道)의 점막층(粘膜層)을 전자현미경적(電子顯微鏡的)으로 관찰(觀察)하였던 바 acantholysis를 일으켜도 desmosome과 기저막(基底膜)은 큰 변화(變化)가 없었으며 세포간(細胞間)은 세포간물질(細胞間物質)의 집적(集積)에 의(依)해 확장(擴張)되고 이들 세포간강물질(細胞間腔物質)은 집괴(集塊) 또는 무구조(無構造)한 물질(物質)로 나타났다. 그리고 기저세포(基底細胞)는 기저막(基底膜)에 단단히 부착(附着)되어 있었고 dendritic cell이 기저세포층(基底細胞層)위로 분포(分布)되어 있었으며, 이들 dendritic cell 중(中)에서는 가끔 여러 형태(形態)의 퇴행성변화(退行性變化)를 볼 수 있었다. Mouse 피부유리상피세포(皮膚遊離上皮細胞)에 있어서 immunogold-labeling 방법(方法)에 의해 dendritic cell을 동정(同定)하는 데에는 post-fixation, pre-embedding immunogold-labeling technique가 좋았으며 15nm와 40nm 크기의 colloid-fold 입자(粒子)로 Langerhans cell과 Thy-1양성(陽性) dendritic cell이 표식(標識)될 수 있었다. 이들 세포(細胞)들은 세포막항원(細胞膜抗元)에 따라 monoclonal antibody의 반응(反應)에 이어 치밀한 colloid-gold 입자(粒子)가 세포막표면(細胞膜表面)을 따라 일정(一定)하게 표식(標識)되었다. 또한 이들 상피세포(上皮細胞)들을 투과전자현미경적(透過電子顯微鏡的)으로 관찰(觀察)하였을 때 초미세구조(超微細構造)가 잘 보존(保存)되었으나 Langer-hans cell내(內)의 Birbeck granule은 유리전(遊離前) 피부상피조직내(皮膚上皮組織內)의 Langerhans cell내(內)의 Birbeck granule에 비(比)해 수적(數的)으로 현저히 감소(減少)되어 있었다. 그러나 Thy-1 양성(陽性) dendritic cell에서 볼 수 있는 dense-core 과립(顆粒)은 별변화(別變化)없이 쉽게 관찰(觀察)될 수 있었다. 조직배양(組織培養)을 한 견(犬)의 keratinocyte에 대(對)해 사람 pemphigus vulgaris의 항체(抗體)로 반응(反應)시킨 후 protein-A gold(15 nm)로 표식(標識)시킨 바 제일 바깥 상층(上層)의 keratinocyte에 있어서 세포막표면(細胞膜表面)을 따라 표식(標識)되어 세포막항원(細胞膜抗元)을 나타내었으며, 이와 같은 소견(所見)으로 미루어 정상피부(正常皮膚) 중층편평상피세포(重層扁平上皮細胞)에서도 동일(同一)한 소견(所見)을 관찰(觀察)할 수 있다고 본다.

• Journal of Periodontal and Implant Science
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• 제30권3호
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• pp.631-639
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• 2000

Desquamative gingivitis is characterized by a diffuse erythema of the free and attached gingiva associated with areas of vesiculation, erosion, and desquamation. Desquamative gingivitis is not a distinct disease entity but represents a reaction pattern of the gingiva to various stimuli. Pemphigus vulgaris, cicatricial pemphigoid, and lichen planus may presents as desquamative gingivitis. We observed 3 patients whose disease was limited to the gingiva, and studied them by light and direct immunofluorescence microscope. We classified them according to clinical, histologic, and immunopathologic observations. Identification of the underlying causes of desquamative gingivitis is of utmost importance and is dependent upon clinical, histologic, and immunologic criteria.

• Journal of Oral Medicine and Pain
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• 제40권4호
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• pp.135-139
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• 2015

Desquamative gingivitis (DG) is a gingival manifestation of systemic mucocutaneous disorders such as mucous membrane pemphigoid, oral lichen planus, and pemphigus vulgaris. The lesion is very painful, so affects the patient's ability to do proper oral hygiene practices. This may be a potential risk factor for long-term periodontal health. However, there is some controversy about the relationship between the existence of DG and periodontal status. Although the correlation between DG-associated diseases and periodontal status is not to be certain, early diagnosis and appropriate treatment including adequate plaque control and removal of local factors is very important for preventing the progression of diseases and destruction of periodontal tissues.

• Journal of Oral Medicine and Pain
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• 제35권4호
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• pp.259-264
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• 2010

만성 궤양성 치은 병소는 편평태선, 양성점막유천포창, 심상성 천포창 등과 같은 다양한 질환에 의해 유발될 수 있으며 코티코스테로이드 약물요법이 주된 치료로 이용된다. 코티코스테로이드를 복용하는 경우 위장장애, 체중증가 등의 부작뿐만 아니라 장기 복용 시 골다공증, 당뇨 및 고혈압 발생 또는 악화, 부신기능 저하, 쿠싱증후군 등의 심각한 부작용이 발생 할 수 있어 병소가 국소 부위에 제한되어 발생하거나 전신증상을 동반하지 않는 경우 스테로이드 약물의 국소 도포가 만성 궤양성 치은 병소의 주된 치료로 이용되고 있다. 하지만 국소 스테로이드 치료를 구강 내에 사용하는 경우 타액 분비, 혀, 입술, 협점막 등의 움직임에 의해 도포한 약제가 소실되어 효과가 감소되고 병소가 넓은 부위에 분포하거나 구강 내 깊숙한 부위에 발생하는 경우 환자 스스로 약물을 도포하기가 용이하지 않으며, 질병에 이환 되지 않은 정상 점막에도 약제가 도포 되는 등의 단점이 있다. 국소 스테로이드 적용 방법의 단점을 극복하고 효과를 최대화함으로써 스테로이드 복용을 최소화할 수 있는 방법으로 스텐트를 이용한 스테로이드 밀폐 요법이 보고된 바 있으나 실제 임상에서 적극적으로 활용되지는 않고 있다. 따라서 만성 궤양성 치은 병소가 발생한 환자에게 스텐트를 이용한 국소스테로이드 치료를 시행하여 양호한 치료 효과를 보인 증례를 통해 그 효용성과 임상적 활용 방안을 알아보고자 한다.