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Angelo Gualberto de Macedo, Erika Ruback Bertges, Luiz Carlos Bertges, Renata Alvim Mendes, Thais Abranches Bueno Sabino Bertges, Klaus Ruback Bertges, Fernando Monteiro Aarestrup
Pemphigus vulgaris (PV) is a chronic autoimmune mucocutaneous disease. In most cases, the initial manifestation occurs in the mouth as multiple ulcerations preceded by blisters that rupture and later spread to other mucous membranes and the skin. Esophageal impairment is rare. We report a case of PV with esophageal involvement in a 53-year-old woman who sought medical care, complaining of diffuse painful lesions in the oral cavity for approximately 1 month, with no improvement with nystatin. Upper digestive endoscopy (UDE) was performed with findings of vesiculobullous lesions in the proximal and middle esophagus, which were biopsied and for which histopathology confirmed PV in the esophageal mucosa...
May 2018: Case Reports in Gastroenterology
Ameni Jerbi, Hend Hachicha, Sawsan Feki, Emna Bahloul, Khadija Sellami, Olfa Abida, Slim Charfi, Amina Bouzid, Tahia Sellami Boudawara, Hamida Turki, Abderrahmen Masmoudi, Hatem Masmoudi
BACKGROUND: Pemphigus herpetiformis (PH) is a rare subtype of pemphigus combining clinical features of dermatitis herpetiformis and the immunopathologic characteristics of pemphigus. We aimed to analyze the epidemiological, clinical, and immunological presentation and management of the disease in a cohort of south Tunisian patients with a long-term follow-up. METHODS: We included all patients with confirmed PH followed from January 1987 to December 2015 in the dermatology department...
July 16, 2018: International Journal of Dermatology
Marta Sar-Pomian, Marek Konop, Kamila Gala, Lidia Rudnicka, Malgorzata Olszewska
Introduction: Scalp involvement in the course of pemphigus is observed in 16-60% of patients. Aim: To determine the prognostic significance of scalp involvement in pemphigus vulgaris and pemphigus foliaceus. Material and methods: A total of 75 patients (46 with pemphigus vulgaris, 29 with pemphigus foliaceus) were included into this prospective study. The following clinical data were analyzed: Pemphigus Disease Area Index, time to complete clinical remission and duration of complete clinical remission...
June 2018: Postȩpy Dermatologii i Alergologii
Ece Altun, Savaş Yaylı, Leyla Baykal Selçuk, Deniz Aksu Arıca, Sevgi Bahadır
Pemphigus is an autoimmune disease characterized by intraepithelial bullae and erosions in the skin and mucosa. We aimed to evaluate the clinical and demographic characteristics of pemphigus vulgaris (PV) patients who presented to our Department. Patients who presented to our Department between May 2013 and May 2014, were examined dermatologically and diagnosed with PV based on clinical, histological and direct immunofluorescent findings. Name, family name, and gender of the patients, their complaint at presentation, onset time and location of the lesions, the number of lesions, systemic treatments received by patients and patients' medication histories were recorded...
June 2018: Acta Dermatovenerologica Croatica: ADC
Ayşe Öktem, Yıldız Hayran, Pınar İncel Uysal, Ahmet Uğur Atılan, Başak Yalçın
According to the "desmoglein compensation theory," anti-Dsg1 and anti-Dsg3 profiles are crucial for the clinical outcome of pemphigus vulgaris. However, recent studies have highlighted several cases with an incompatibility between the antibody profile and clinical manifestation. Data of 37 patients who had been diagnosed pemphigus vulgaris in our Department between January 2014-June 2016 were retrieved from our clinical database. Patients with ABSIS skin involvement scores, oral mucosa extent and severity scores, anti-Dsg1 and Dsg3 antibody profile were included in this retrospective study...
June 2018: Acta Dermatovenerologica Croatica: ADC
Maryam Koopai, Hossein Mortazavi, Alireza Khatami, Zohreh Khodashenas
Anti-desmoglein (anti-Dsg) ELISA and indirect immunofluorescence (IIF) are used for the diagnosis of pemphigus vulgaris (PV). The value of salivary ELISA, serum ELISA, and IIF in the diagnosis of PV, and the correlation of salivary anti-Dsg1 and anti-Dsg3 ELISA with serum ELISA, serum and salivary IIF titers, and disease severity in patients with PV were evaluated. Fifty newly diagnosed patients with PV were enrolled in the study. Demographic data and disease-severity scores were recorded for each patient. Anti-Dsg1 and anti-Dsg3 ELISA and IIF were performed on both serum and salivary samples...
June 2018: Acta Dermatovenerologica Croatica: ADC
Matteo Saccucci, Gabriele Di Carlo, Maurizio Bossù, Francesca Giovarruscio, Alessandro Salucci, Antonella Polimeni
Oral signs are frequently the first manifestation of autoimmune diseases. For this reason, dentists play an important role in the detection of emerging autoimmune pathologies. Indeed, an early diagnosis can play a decisive role in improving the quality of treatment strategies as well as quality of life. This can be obtained thanks to specific knowledge of oral manifestations of autoimmune diseases. This review is aimed at describing oral presentations, diagnosis, and treatment strategies for systemic lupus erythematosus, Sjögren syndrome, pemphigus vulgaris, mucous membrane pemphigoid, and Behcet disease...
2018: Journal of Immunology Research
Mark Yulis, Dennis H M Kusters, Asma Nusrat
The single pass, transmembrane proteins of the cadherin family have been appreciated as important proteins that regulate intercellular adhesion. In addition to this critical function, cadherins contribute to important signalling events that control cellular homeostasis. Many examples exist of classical, desmosomal, and atypical cadherins participating in the regulation of signalling events that control homeostatic functions in cells. Much of the work on cadherin mediated signalling focuses on classical cadherins or on specific disease states such as pemphigus vulgaris...
July 2, 2018: Journal of Physiology
K Coyner, K Tater, M Rishniw
OBJECTIVES: To characterise the signalment, clinical presentation and therapy of pemphigus foliaceus in cats treated by primary care practitioners. MATERIALS AND METHODS: Retrospective evaluation of patient and treatment factors with the following case outcomes: remission (achieving remission, time to remission), treatment-related adverse effects (number, severity) and final disease outcome (medical control, cure, death or euthanasia). RESULTS: Data were obtained from 48 cats with biopsy-confirmed pemphigus foliaceus managed by practitioners from six countries and 47 hospitals...
July 1, 2018: Journal of Small Animal Practice
Enke Baldini, Teresa Odorisio, Chiara Tuccilli, Severino Persechino, Salvatore Sorrenti, Antonio Catania, Daniele Pironi, Giovanni Carbotta, Laura Giacomelli, Stefano Arcieri, Massimo Vergine, Massimo Monti, Salvatore Ulisse
The skin is the largest organ of the body, at the boundary with the outside environment. Primarily, it provides a physical and chemical barrier against external insults, but it can act also as immune organ because it contains a whole host of immune-competent cells of both the innate and the adaptive immune systems, which cooperate in eliminating invading pathogens following tissue injury. On the other hand, improper skin immune responses lead to autoimmune skin diseases (AISD), such as pemphigus, bullous pemphigoid, vitiligo, and alopecia...
June 15, 2018: Reviews in Endocrine & Metabolic Disorders
Wei-Shen Chen, Michael T Tetzlaff, Hafeez Diwan, Richard Jahan-Tigh, Adi Diab, Kelly Nelson, Isabella C Glitza, Genevieve J Kaunitz, Daniel Johnson, Carlos Torres-Cabala, Omar Pacha, Janis M Taube, Kudakwashe Maloney, Victor G Prieto, Jonathan L Curry
Checkpoint inhibitors (CPIs) restore the function of effector immunocytes to target and destroy cancer cells. Immune-related adverse events (irAEs) are a consequence of immune reactivation, with unpredictable inflammatory response, loss of self-tolerance, and development of autoimmunity. Adverse events from CPIs that present as dermatologic toxicities have diverse clinical and histopathologic features. CPI-associated dermatologic toxicities may exhibit histopathologic features of lichenoid dermatitis, bullous pemphigoid, and granulomatous/sarcoid-like reactions...
June 25, 2018: Journal of Cutaneous Pathology
R Rodriguez-Lojo, M M Otero-Rivas, T Usero Bárcena, I Castiñeiras Mato
No abstract text is available yet for this article.
June 21, 2018: Actas Dermo-sifiliográficas
Swastika Suvirya, Sucheta Pathania, Kiran Preet Malhotra, Parul Verma
Cicatricial pemphigoid (CP) is a chronic, autoimmune, subepidermal blistering disease with predominant mucosal involvement. In this article, we report a young patient with mucosal and extensive cutaneous involvement in the form of large erosions mimicking those of pemphigus vulgaris thus leading to diagnostic dilemma. We were unable to find any other previous reports with such extensive cutaneous erosions mimicking those of pemphigus vulgaris. Laminin 5 was the antigen found on knockout substrate testing. Antiepiligrin CP is a distinct subtype of CP with antibodies against laminin 5...
June 21, 2018: BMJ Case Reports
Oscar Thabouillot, Julien Le Coz, Nicolas-Charles Roche
A seven-month-old girl was referred to the emergency department (ED) after a general practitioner suspected Steven-Johnson syndrome. Actually, the diagnosis of bullous pemphigoid (BP) was made based on biopsies; BP is a rare, autoimmune skin disease involving the presence of blisters known as bullae. The child was efficiently treated with topical steroids. This case shows the importance of the ED physician's prior knowledge of BP so that a differential diagnosis with other autoimmune diseases (dermatosis, pemphigus) can be made...
June 22, 2018: Prehospital and Disaster Medicine
Reem Akel, Ghina Fakhri, Rana Salem, Fouad Boulos, Khaled Habib, Arafat Tfayli
Background: Follicular dendritic cell sarcoma (FDCS) is an extremely rare malignant tumor caused by proliferation of antigen-presenting follicular dendritic cells. The tumor most commonly arises in lymph nodes, with fewer than 30% of cases originating in extranodal sites. Case Presentation: We report the case of a 39-year-old previously healthy male patient who presented with paraneoplastic pemphigus in the setting of a large intraperitoneal mass with no associated lymphadenopathy...
May 2018: Case Reports in Oncology
Elisabeth Schlögl, Mariya Y Radeva, Franziska Vielmuth, Camilla Schinner, Jens Waschke, Volker Spindler
Pemphigus vulgaris (PV) is a potentially lethal autoimmune disease characterized by blister formation of the skin and mucous membranes and is caused by autoantibodies against desmoglein (Dsg) 1 and Dsg3. Dsg1 and Dsg3 are linked to keratin filaments in desmosomes, adhering junctions abundant in tissues exposed to high levels of mechanical stress. The binding of the autoantibodies leads to internalization of Dsg3 and a collapse of the keratin cytoskeleton-yet, the relevance and interdependence of these changes for loss of cell-cell adhesion and blistering is poorly understood...
2018: Frontiers in Immunology
Kyle T Amber, Manuel Valdebran, Sergei A Grando
Pemphigus vulgaris (PV) is a potentially life-threatening mucocutaneous autoimmune blistering disease. Patients develop non-healing erosions and blisters due to cell-cell detachment of keratinocytes (acantholysis), with subsequent suprabasal intraepidermal splitting. Identified almost 30 years ago, desmoglein-3 (Dsg3), a Ca2+ -dependent cell adhesion molecule belonging to the cadherin family, has been considered the "primary" autoantigen in PV. Proteomic studies have identified numerous autoantibodies in patients with PV that have known roles in the physiology and cell adhesion of keratinocytes...
2018: Frontiers in Immunology
Nicholas A Ross, Miriam L Vega, Matthew S Keller
Dermatology was consulted in the care of a 58-year-old man with a history of paranoid schizophrenia, neuroleptic malignant syndrome, a positive purified protein derivative test, and a lack of bathing for approximately 4 years who had been admitted to the hospital because of thick, crusted lesions over an increasing portion of his body. Admitted involuntarily, he was disinterested in the history, physical examination, and diagnostic testing. Comorbid schizophrenia presented a unique challenge because he was unable to participate in his care effectively...
2018: Skinmed
Khalaf Kridin, Mogher Khamaisi, Doron Comaneshter, Erez Batat, Arnon D Cohen
There is a little consensus regarding the association of pemphigus with autoimmune thyroid diseases. While this association had been confirmed by some observational studies, others had refuted it. We aimed to study the association between pemphigus and Hashimoto's thyroiditis, Grave's disease, and thyroid cancer using a large-scale real-life computerized database. A cross-sectional study was performed to compare pemphigus patients with age-, sex-, and ethnicity-matched control subjects regarding the prevalence of overt thyroid diseases...
2018: Frontiers in Medicine
B Tamási, V Brodszky, M Péntek, L Gulácsi, K Hajdu, M Sárdy, A Szegedi, Z Bata-Csörgő, Á Kinyó, F Rencz
BACKGROUND: No studies have employed the EuroQoL (EQ-5D) questionnaire to assess health-related quality of life (HRQoL) in pemphigus patients, to date. OBJECTIVES: To evaluate HRQoL of pemphigus patients by the EQ-5D and to analyse the convergent and known-groups validity of the EQ-5D in this patient population. METHODS: Between 2014 and 2017, a multicentre cross-sectional study was carried out. Outcome measures included the five-level EQ-5D (EQ-5D-5L), Dermatology Life Quality Index (DLQI), Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and an average pain intensity visual analogue scale (VAS) for the past three months...
June 13, 2018: British Journal of Dermatology
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