CLINICAL ANALYSIS OF DESQUAMATIVE GINGIVITIS RELATED ORAL MUCOCUTANEOUS DISEASES

Objectives: Several mucocutaneous diseases are responsible for desquamative gingivitis (DG) and diagnostic delays are common in these diseases due to non-specific clinical oral presentations. The present study aimed to analyze the clinical features of DG-related mucocutaneous diseases with oral manifestations and compare the findings with previous reports. Materials and Methods: Thirty newly diagnosed patients with oral mucocutaneous disease in the Dermatology and Venereology Department of Akdeniz University Medical School, between January 2018 and January 2020, and referred to the Periodontology Department for periodontal treatment were evaluated retrospectively. Patients demographic data (age and gender), medical comorbidities, primary lesion site (oral or skin), and clinical features of gingival and oral lesions (location and symptoms) were examined. Results: There were 18 oral lichen planus (OLP), 8 pemphigus vulgaris (PV), and 4 mucous membrane pemphigoid (MMP) patients with oral involvements. The mean age of the patients was 50.03 ± 9.71 years with a female predominance (male to female ratio 1:1.7). All patients suffered from chronic burning sensation and oral pain. DG was detected in all three mucocutaneous diseases but OLP was the main disease associated with DG (60%). However other oral mucosal lesions and extraoral involvements were more frequent in PV patients. Conclusions: According to these results, OLP, MMP, and PV are the mucocutaneous diseases responsible for DG, and OLP is the most common cause of DG. Clinicans should be familiar with the clinical presentations and signs (patient complaints) of DG-related mucocutaneous disease for early diagnosis, proper treatment, and quality of life of the patient

Successful Treatment of a Bullous Pemphigoid Patient with Rituximab Who Was Refractory to Corticosteroid and Omalizumab Treatments

Omalizumab is a humanized monoclonal antibody which is an FDA-approved treatment of severe allergic asthma and inhibits IgE binding to FcεRI. According to increasing evidence of IgE inhibition, omalizumab was suggested as a therapeutic approach for bullous pemphigoid (BP). Rituximab has been reported to be effective in various autoimmune diseases, including autoimmune bullous dermatoses. A specific protocol for the use of rituximab to treat BP patients is not yet available. There are only small case series and case reports about the efficacy and safety of rituximab in BP. Here we present a young BP patient who responded well to rituximab therapy and was refractory to conventional and omalizumab therapies although he had elevated IgE levels and eosinophilia. Our case supports the knowledge about the effectiveness and safety of rituximab not only in pemphigus but also in BP. On the other hand, although it did not work in our case, omalizumab may be a potentially effective agent in some carefully selected patients with certain subtypes of BP

PUVA Induced Bullous Pemphigoid in a Patient with Mycosis Fungoides

Background. Bullous pemphigoid is an autoimmune subepidermal blistering skin disease in which autoantibodies are directed against components of the basement membrane. The disease primarily affects the elderly people and in most of the patients inducing factors cannot be identified. Herein, we report a case of BP that occurred in a patient who was receiving PUVA therapy for the treatment of mycosis fungoides. Main Observation. A 26-year-old woman with mycosis fungoides developed blisters while receiving PUVA therapy. On physical examination tense bullae on the normal skin, remnants of blisters, and erosions were observed on her breasts, the chest wall, and the upper abdomen. Histopathological investigations revealed subepidermal blisters with eosinophilic infiltration and in direct immunofluorescence examination linear deposition of IgG along the basement membrane zone was observed. The diagnosis of bullous pemphigoid was also confirmed by ELISA and BIOCHIP mosaic-based indirect immunofluorescence test. Conclusions. PUVA therapy is an extremely rare physical factor capable of inducing bullous pemphigoid. So the development of blistering lesions during PUVA therapy may be suggestive sign of a bullous disease such as bullous pemphigoid and it should be excluded with proper clinical and laboratory approaches immediately after withdrawal of PUVA therapy

Türkiye’deki Pemfigus Hastalarının CTLA-4 +49 A/G, -318 C/T, -1661 A/G, CT60 A/G Gen Polimorfizmleri

Amaç: Yaşamı tehdit eden bir grup bülleşme gösteren hastalığı ifade eden pemfigus, desmozomlardaki hücreler arası yapışmayı sağlayan ve desmoglein 1 ve/veya 3 isimli proteinlere karşı otoantikorlardan kaynaklanır. Sitotoksik T-lenfosit-ilişkili antijen (CTLA)- 4'ün, bu otoimmün reaksiyonda rol alan T lenfositlerin negatif regülasyonundaki önemi iyi bilinmektedir. Bu moleküle ilişkin gen polimorfizmleri otoimmüniteyi etkilemektedir. CTLA-4 +49 A/G, -318 C/T, -1661 A/G and CT60 A/G gen polimorfizmlerinin Türk popülasyonunda pemfigus yatkınlığına yol açıp açmadığını belirlemeyi amaçladık. Yöntemler: Polimeraz zincir reaksiyonu-restriksiyon fragman uzunluk polimorfizmi metodu yardımıyla 118 pemfigus hastası ve 108 sağlıklı kişinin bu tek nükleotid polimorfizmleri için genotiplerini saptadık. Bulgular: CTLA-4 +49 A/G, -318 C/T, -1661 A/G ve CT60 A/G allel ve genotip dağılımlarının sıklıkları pemfigus hastaları ve sağlıklı kontroller arasında farklı değildi (sırasıyla p=0,643, OR=0,931; p=0,847, OR=1,160; p=0,968, OR=0,975; p=0,173, OR=1,303). Sonuç: Bu polimorfizmlerin Türk popülasyonunda pemfigus yatkınlığı ile ilişkili olmadığı sonucuna vardık. Bu, söz konusu 4 CTLA tek nükleotid polimorfizminin pemfigus yatkınlığında olası rolünün eşzamanlı araştırıldığı ilk çalışmadır. CTLA-4 -1661 A/G gen polimorfizminin pemfigustaki rolü daha önce çalışılmamıştırObjective: Pemphigus, expressing a life-threatening blistering disease, result from autoantibodies against the proteins that mediate intercellular adhesion in desmosomes, namely desmoglein 1 and/or 3. The importance of cytotoxic T lymphocyte-associated antigen (CTLA)-4 in negative regulation of T lymphocytes, which take part in this autoimmune reaction, is well known. Gene polymorphisms regarding this molecule affect autoimmunity. We aimed to determine whether CTLA-4 +49 A/G, -318 C/T, -1661 A/G and CT60 A/G gene polymorphisms cause susceptibility to pemphigus in Turkish population. Methods: We detected genotypes of the single nucleotide polymorphisms for 118 pemphigus patients and 108 healthy individuals with the help of polymerase chain reaction-restriction fragment length polymorphism method. Results: Distribution of the CTLA-4 +49 A/G, -318 C/T, -1661 A/G and CT60 A/G allele and genotype frequencies did not differ between pemphigus patients and healthy controls (p=0.643, OR=0.931; p=0.847, OR=1.160; p=0.968, OR=0.975; p=0.173, OR=1.303, respectively). Conclusion: We concluded that these polymorphisms are not associated with pemphigus susceptibility in Turkish population. This is the first study investigating the possible role of the 4 CTLA single nucleotide polymorphism in pemphigus susceptibility simultaneously. The role of CTLA-4 -1661 A/G gene polymorphism in pemphigus was not studied previousl

Angina bullosa haemorrhagica-a report of two cases

Anjina bülloza hemorajika (ABH) oral mukozada gelişen hemorajik büllerle karakterize selim, akut bir tablodur. Lezyonlar genellikle tekrarlayıcı karekterde olup skar bırakmadan iyileşirler. ABH genellikle orta yaş veya yaşlı hastalarda görülen bir hastalıktır. Oluşumunda birçok faktör sorumlu tutulmaktadır, ancak travmanın temel provake edici faktör olduğu ve uzun süre inhaler steroid kullanımının hastalığı olumsuz yönde etkilediği düşünülmektedir. Biz kronik obstrüktif akciğer hastalığına sahip rekürran oral hemorajik bülleri olan iki hasta sunuyoruz: Hipertansiyonu, aterosklerotik kalp hastalığı ve hiperglisemisi olan 78 yaşında bir erkek hasta ve altı yıldır inhaler steroid kullanan 40 yaşında erkek hasta. Selim olan bu hastalık hava yolunda akut obstrüksiyonla sonuçlanabilmektedir. Bu nedenle dermatologlar ABH’dan haberdar olmalıdır.ABH is a benign phenomenon that is characterized by acute blood-filled blisters of the oral mucosa. The lesions are usually recurrent, that heal without scarring. ABH is predominantly a localized disorder of middle-aged or elderly patients. The cause may be a multifactorial phenomena, but trauma seems to be the major provoking factor and long term use of steroid inhalers has also been implicated in the disease. We report two patients with recurrent oral blood blisters, who have chronic obstructive airway disease. A 78-year-old male patient has also hypertension, atherosclerotic heart disease and hyperglycemia. A 40-year-old male other patient has used steroid inhalers for 6 years. Although this is a benign condition, it may result in acute airway obstruction. Therefore dermatologists should be aware of ABH

Diagnosis and Management of Pemphigus: recommendations by an International Panel of Experts

International audienceSeveral European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management, OBJECTIVE: We now present results from a subsequent Delphi consensus to broaden the generalizability of recommendations