Esophageal Involvement as a First Clinical Presentation in a Patient with Newly Diagnosed Pemphigus Vulgaris and a Review of Literature

2013 ◽  
Vol 108 ◽  
pp. S187-S188 ◽  
Author(s):  
Kalaiyarasi Kaliyaperumal ◽  
Charles Vu
Keyword(s):  
Clinical Presentation ◽  
Pemphigus Vulgaris ◽  
Newly Diagnosed ◽  
Review Of Literature ◽  
Esophageal Involvement

scholarly journals Efficient implementation of the ‘non-biopsy approach’ for the diagnosis of childhood celiac disease in the Netherlands: a national prospective evaluation 2010–2013

2021 ◽  
Author(s):  
Caroline R. Meijer ◽  
Joachim J. Schweizer ◽  
Anne Peeters ◽  
Hein Putter ◽  
M. Luisa Mearin
Keyword(s):  
Celiac Disease ◽  
The Netherlands ◽  
Clinical Presentation ◽  
Paediatric Population ◽  
Hla Typing ◽  
Newly Diagnosed ◽  
Prospective Evaluation ◽  
Espghan Guidelines ◽  
Intestinal Symptoms ◽  
Nationwide Implementation

AbstractThe aim of this study was (1) to prospectively evaluate the nationwide implementation of the ESPGHAN-guidelines for the diagnosis of celiac disease (CD), (2) to investigate the incidence and clinical presentation of diagnosed childhood CD (0–14 years) in the Netherlands, and (3) to compare the findings with national survey data from 1975 to 1990 and 1993 to 2000 using the same approach. From 2010 to 2013, all practicing paediatricians were invited to report new celiac diagnoses to the Dutch Pediatric Surveillance Unit. Data were collected via questionnaires. A total of 1107 children with newly diagnosed CD were reported (mean age, 5.8 years; range, 10 months–14.9 years; 60.5% female). After the introduction of the non-biopsy approach in 2012, 75% of the diagnoses were made according to the guideline with a significant decrease of 46.3% in biopsies. The use of EMA and HLA-typing significantly increased with 25.8% and 62.1%, respectively. The overall incidence rate of childhood CD was 8.8-fold higher than in 1975–1990 and 2.0-fold higher than in 1993–2000. During the study period, the prevalence of diagnosed CD was 0.14%, far below 0.7% of CD identified via screening in the general Dutch paediatric population. Clinical presentation has shifted towards less severe and extra-intestinal symptoms.Conclusion: ESPGHAN guidelines for CD diagnosis in children were effectively and rapidly implemented in the Netherlands. Incidence of diagnosed CD among children is still significantly rising with a continuous changing clinical presentation. Despite the increasing incidence of diagnoses, significant underdiagnosis still remains. What is Known:• Since 2000 the incidence of diagnosed childhood CD in the Netherlands has shown a steady rise.• The rise in incidence has been accompanied by a changing clinical presentation at diagnosis. What is New:• The ESPGHAN guidelines 2012 for CD diagnosis were effectively and rapidly implemented in the Netherlands.• The incidence of diagnosed childhood CD in the Netherlands has continued to rise significantly during the reported period.

scholarly journals Temporal gliosarcoma: case report and review of literature

2015 ◽  
Vol 22 (1) ◽  
pp. 112-116
Author(s):  
Amit Agrawal ◽  
Vissa Shanthi ◽  
Baddukonda Appala Ramakrishna ◽  
Kuppili Venkata Murali Mohan
Keyword(s):  
Case Report ◽  
Median Survival ◽  
Glial Cells ◽  
Primary Tumor ◽  
Clinical Presentation ◽  
Spindle Cell ◽  
Review Of Literature ◽  
Future Studies ◽  
Aggressive Tumor ◽  
The Brain

Abstract First characterized by Stroebe, the gliosarcomas are highly malignant and rare primary tumor of the brain composed of neoplastic glial cells in association with spindle cell sarcomatous elements (biphasic tissue patterns). In spite of being recognized as two different pathologies studies have not shown any significant differences between gliosarcoma and glioblastoma with regard to age, sex, size, clinical presentation, and median survival. In summary, gliosarcoma is an aggressive tumor with a propensity to recur and re-grow with poor outcome. Future studies are needed to understand the true pathology of these biphasic tumors.

scholarly journals Pemphigus Vulgaris in the Mouth and Esophageal Mucosa

2018 ◽  
Vol 12 (2) ◽  
pp. 260-265 ◽  
Author(s):  
Angelo Gualberto de Macedo ◽  
Erika Ruback Bertges ◽  
Luiz Carlos Bertges ◽  
Renata Alvim Mendes ◽  
Thais Abranches Bueno Sabino Bertges ◽  
...  
Keyword(s):  
Oral Cavity ◽  
Medical Care ◽  
Pemphigus Vulgaris ◽  
Esophageal Mucosa ◽  
Digestive Endoscopy ◽  
Initial Manifestation ◽  
Esophageal Involvement ◽  
Mucous Membranes ◽  
Upper Digestive Endoscopy

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. We draw attention to UDE as important for the diagnosis, and it should be indicated in patients with vesiculobullous lesions of the mouth, especially if there are esophageal complaints.

scholarly journals Esophageal Pemphigus Vulgaris: A Rare Etiology of Upper Gastrointestinal Hemorrhage

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Jennifer Rose F. Del Castillo ◽  
Muhammad Nadeem Yousaf ◽  
Fizah S. Chaudhary ◽  
Nahar Saleh ◽  
Lawrence Mills
Keyword(s):  
Early Recognition ◽  
Gastrointestinal Symptoms ◽  
Pemphigus Vulgaris ◽  
Diagnostic Endoscopy ◽  
Presenting Symptoms ◽  
Esophageal Involvement ◽  
Asymptomatic Patients ◽  
Mucosal Surfaces ◽  
Early Endoscopy ◽  
Upper Gastrointestinal

Pemphigus vulgaris (PV) is an autoimmune blistering disorder of the skin and mucosal surfaces characterized by acantholysis (loss of adhesion between epidermal cells). Esophageal involvement of PV is an underdiagnosed entity as routine diagnostic endoscopy is not recommended in asymptomatic patients. Dysphagia and odynophagia are common presenting symptoms; however, upper gastrointestinal bleeding (UGIB) associated with esophageal involvement of PV without a history of mucosal blistering is extremely uncommon. We present a case of esophageal involvement of PV associated with active UGIB that was diagnosed on endoscopic evaluation. This case illustrated the importance of early endoscopy to identify the esophageal involvement of PV especially in patients with preexisting disease who present with gastrointestinal symptoms such as dysphagia, odynophagia, and hematemesis. Early recognition of esophageal involvement of PV and initiation of corticosteroid and/or immunosuppressant therapy may improve the outcome of the disease.

Sweet Syndrome and Pemphigus Vulgaris

2012 ◽  
Vol 16 (2) ◽  
pp. 128-130 ◽  
Author(s):  
Ashley O'toole ◽  
Maureen O'malley
Keyword(s):  
Clinical Presentation ◽  
Pemphigus Vulgaris ◽  
Neutrophilic Dermatosis ◽  
Rare Presentation ◽  
Sweet Syndrome ◽  
Autoimmune Blistering Disease ◽  
Mucous Membranes ◽  
Blistering Disease ◽  
Erythematous Plaques ◽  
New Onset

Background: Pemphigus vulgaris is an autoimmune blistering disease of the skin and mucous membranes. Sweet syndrome is an uncommon reactive neutrophilic dermatosis. Objective: This case report describes the rare presentation of a 58-year-old female patient with newly diagnosed pemphigus vulgaris who later presented with erythematous plaques and leukocytosis highly suggestive of Sweet syndrome. Methods: Review of the literature using the PubMed and Medline databases. Results: The clinical presentation of new-onset Sweet syndrome in a patient with pemphigus has been reported in the literature only on one other occasion. Conclusion: The observation of an association between Sweet syndrome and autoimmune diseases, such as pemphigus vulgaris, may ultimately lead to better understanding of the pathophysiology of this disease.

Quadricuspid aortic valve: a case report and review of literature

2021 ◽  
Author(s):  
Usman Ghani Piracha ◽  
Gurukripa N. Kowlgi ◽  
Walter Paulsen ◽  
Mohammad Khalid Mojadidi ◽  
Nimesh Patel
Keyword(s):  
Case Report ◽  
Aortic Valve ◽  
Aortic Valve Replacement ◽  
Functional Status ◽  
Aortic Regurgitation ◽  
Valve Replacement ◽  
Clinical Presentation ◽  
Quadricuspid Aortic Valve ◽  
Review Of Literature ◽  
Cardiac Defect

Quadricuspid aortic valve, a rare congenital cardiac defect, manifests most commonly as aortic regurgitation. Clinical presentation mainly depends on the functional status of the aortic valve, myocardium and associated cardiovascular abnormalities. Aortic valve replacement or repair is usually warranted in the 5th or 6th decade.

Esophagogastroduodenoscopy and Esophageal Involvement in Patients with Pemphigus Vulgaris

Dysphagia ◽  
2019 ◽  
Vol 35 (3) ◽  
pp. 503-508
Author(s):  
Katharine A. Ozeki ◽  
Thomas A. Zikos ◽  
John O. Clarke ◽  
Irene Sonu
Keyword(s):  
Pemphigus Vulgaris ◽  
Esophageal Involvement

Esophagogastroduodenoscopy and Esophageal Involvement in Patients With Pemphigus Vulgaris

2018 ◽  
Vol 113 (Supplement) ◽  
pp. S177
Author(s):  
Katharine Ozeki ◽  
Thomas A. Zikos ◽  
John Clarke ◽  
Irene Sonu
Keyword(s):  
Pemphigus Vulgaris ◽  
Esophageal Involvement

scholarly journals AGGRESSIVE MESENTRIC FIBROMATOSIS: A RARE CASE REPORT AND REVIEW OF LITERATURE

2013 ◽  
Vol 03 (01) ◽  
pp. 79-82
Author(s):  
Rohan Shetty ◽  
Shubha Bhat ◽  
Rajesh Ballal ◽  
Pramod Makannavar ◽  
Anil Kumar K. N.
Keyword(s):  
Case Report ◽  
Male Patient ◽  
Rare Case ◽  
Clinical Presentation ◽  
Aggressive Fibromatosis ◽  
Definitive Diagnosis ◽  
Review Of Literature ◽  
Therapeutic Challenge ◽  
Rare Case Report ◽  
Small Intestinal

AbstractMesentric fibromatosis is a proliferative fibroblastic neoplasm of the small intestinal mesentery with varied clinical presentation. Giant mesentric fibromatosis is uncommon and its rarity poses a diagnostic and therapeutic challenge. This paper presents a recurrent aggressive fibromatosis in a 38 year old male patient, who had initially undergone a laparotomy outside for mass abdomen but only pus was evacuated and definitive diagnosis was not made.

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