scholarly journals Angiolymphoid Hyperplasia of the External Auditory Canal; Reconstruction of a Complex Defect

2021 ◽  
pp. 014556132110167
Author(s):  
Carlos Eduardo Torres Fuentes ◽  
Paula Alejandra López González
Keyword(s):  
External Auditory Canal ◽  
Inflammatory Process ◽  
Clinical Presentation ◽  
Unknown Etiology ◽  
Complex Defect ◽  
Angiolymphoid Hyperplasia

Angiolymphoid hyperplasia (AH) was first described by Wells and Whimster in 1969 as a benign vasoproliferative pathology with a varied infiltrate of eosinophils, lymphocytes, and plasmatic cells. Clinical presentation has been described in the literature as small red-bluish nodules, less than 3 cm in diameter that can bleed in 25% of the cases and be pruritic and painful in 37% and 20% of the cases, respectively. Particularly, AH can appear in the ear; nevertheless, other regions have been affected, including the scalp, lips, tongue, orbit, muscle, and bone. Most of these cases have occurred in adults with an unknown etiology; however, an inflammatory process has been associated in approximately 20% with eosinophilia. No malignancy has been reported.

scholarly journals Erythromelanosis Follicularis Faciei et Colli: A Case Report in a Caucasian Male and Brief Review of the Literature

2020 ◽  
Vol 12 (3) ◽  
pp. 231-235
Author(s):  
Carl Maximilian Thielmann ◽  
Wiebke Sondermann
Keyword(s):  
Case Report ◽  
Family History ◽  
Clinical Presentation ◽  
Rare Condition ◽  
Unknown Etiology ◽  
Review Of The Literature ◽  
Caucasian Male

Erythromelanosis follicularis faciei et colli, a rare condition of unknown etiology, was first described by Kitamura et al. from Japan in 1960. It is characterized by a triad consisting of well-demarcated erythema, hyperpigmentation, and follicular papules. We report the case of a 50-year-old Caucasian male, who had asymptomatic symmetrical facial lesions since the age of 42. His family history was unremarkable. Published erythromelanosis follicularis faciei et colli cases of the last 10 years are summarized in this report to demonstrate the variability and differences in the clinical presentation of this uncommon diagnosis.

Sarcoidosis Presenting as Nasal Obstruction

1993 ◽  
Vol 7 (3) ◽  
pp. 133-138 ◽  
Author(s):  
Mark J. Shikowitz ◽  
Aijaz Alvi
Keyword(s):  
Respiratory Tract ◽  
Nasal Obstruction ◽  
Clinical Presentation ◽  
Multiple Organ ◽  
Diagnostic Workup ◽  
Unknown Etiology ◽  
Granulomatous Disease ◽  
Upper Respiratory Tract ◽  
Important Symptom ◽  
Upper Respiratory

Sarcoidosis is a chronic systemic granulomatous disease of unknown etiology. The histology of this disease was first reported by Boeck in 1899. Since that time many reports of multiple organ involvement have been published. Symptoms relating to the upper respiratory tract are not uncommon. Nasal obstruction as the presenting and primary complaint in sarcoidosis however is a rare but important symptom as it may lead to the diagnosis of this treatable disease. We present three patients whose initial complaint of progressive nasal obstruction heralded the diagnosis of sarcoidosis upon further workup. The clinical presentation, diagnostic workup, histology, and management of nasal sarcoidosis are discussed.

Potential use of biomarkers for the clinical evaluation of sarcoidosis

2021 ◽  
pp. jim-2020-001659
Author(s):  
Amir Mousapasandi ◽  
Cristan Herbert ◽  
Paul Thomas
Keyword(s):  
Clinical Evaluation ◽  
Clinical Presentation ◽  
Current Knowledge ◽  
Epithelioid Cell ◽  
Pulmonary Sarcoidosis ◽  
Biological Molecules ◽  
Unknown Etiology ◽  
Chronic Antigenic Stimulation ◽  
Host Genetic ◽  
Potential Use

Sarcoidosis is a systemic granulomatous disease of unknown etiology and pathogenesis with a heterogeneous clinical presentation. In the appropriate clinical and radiological context and with the exclusion of other diagnoses, the disease is characterized by the pathological presence of non-caseating epithelioid cell granulomas. Sarcoidosis is postulated to be a multifactorial disease caused by chronic antigenic stimulation. The immunopathogenesis of sarcoidosis encompasses a complex interaction between the host, genetic factors and postulated environmental and infectious triggers, which result in granuloma development.The exact pathogenesis of the disease has yet to be elucidated, but some of the inflammatory pathways that play a key role in disease progression and outcomes are becoming apparent, and these may form the logical basis for selecting potential biomarkers.Biomarkers are biological molecules that are altered pathologically. To date, there exists no single reliable biomarker for the evaluation of sarcoidosis, either diagnostically or prognostically but new candidates are emerging. A diagnosis of sarcoidosis ideally requires a biopsy confirming non-caseating granulomas, but the likelihood of progression that requires intervention remains unpredictable. These challenging aspects could be potentially resolved by incorporating biomarkers into clinical practice for both diagnosis and monitoring disease activity.This review outlines the current knowledge on sarcoidosis with an emphasis on pulmonary sarcoidosis, and delineates the understanding surrounding the implication of biomarkers for the clinical evaluation of sarcoidosis.

scholarly journals Clinical Presentation of Congenital Cholesteatoma of the External Auditory Canal in Goldenhar Syndrome: A Case Series

2019 ◽  
Vol 5 (2) ◽  
pp. 44 ◽  
Author(s):  
Sarah Elizabeth Hodge ◽  
April Amber Peterson ◽  
Brendan Powers O’Connell ◽  
Amelia Fischer Drake
Keyword(s):  
External Auditory Canal ◽  
Clinical Presentation ◽  
Case Series ◽  
Goldenhar Syndrome ◽  
Congenital Cholesteatoma

scholarly journals Method for the prevention of inflammatory processes in the bone part of the external auditory canal accompanied by the growth of connective tissue

2020 ◽  
Vol 19 (4) ◽  
pp. 21-26
Author(s):  
I. A. Anikin ◽  
◽  
S. A. Eremin ◽  
A. E. Shinkareva ◽  
◽  
...  
Keyword(s):  
Hearing Loss ◽  
Connective Tissue ◽  
Granulation Tissue ◽  
Satisfactory Result ◽  
External Auditory Canal ◽  
Inflammatory Process ◽  
Local Therapy ◽  
Scar Tissue ◽  
Inflammatory Processes ◽  
Bone Section

Otosurgery plays an important role in the rehabilitation of patients with hearing loss. Any operation can be accompanied by a long inflammatory process with excessive development of connective tissue, forming atresia or stenosis of the external auditory canal. Formed scars in the external auditory canal reduce the effectiveness of treatment and can lead to reoperation. In the treatment of scars, the use of injections of a suspension of prolonged steroids directly into the developing connective tissue is effective. We have developed a method of injecting drugs into the scar tissue of the bone section of the external auditory canal, which allows the use of such therapy in patients after otosurgery. 12 people were treated with the proposed technique. All patients had an inflammatory process in the external auditory canal with undesirable growth of connective tissue. Previous local therapy did not bring a satisfactory result. Patients received injections of a suspension of steroids into developing scar tissue. The treatment period was 1-8 weeks, for which 1 to 4 sessions of injections were performed. As a result of treatment according to the developed method, in all cases a cessation of the pathological inflammatory process was observed. Timely therapy made it possible to completely eliminate the excess granulation tissue (100%), to stop the growth of the scar, or even to cause a significant decrease (33%). The developed method allows to use of a small dose of a substance (0,1–0,5 ml) for injection to achieve the desired therapeutic effect.

scholarly journals Kikuchi-Fujimoto Disease: A Study Of 67 Cases From Coastal South India 

2020 ◽  
Author(s):  
Basavaprabhu Achappa ◽  
Nipuni Chamathka Herath ◽  
Jyoti Ramanath Kini ◽  
Ramesh Holla ◽  
Bodhi Sebastian ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Histopathological Examination ◽  
Cervical Lymphadenopathy ◽  
Symptomatic Treatment ◽  
Lymph Node Biopsy ◽  
Unknown Etiology ◽  
Extensive Literature ◽  
Cervical Lymphadenitis ◽  
Benign Condition ◽  
The Mean

Abstract Background Kikuchi-Fujimoto disease is a rare, benign condition, of unknown etiology, presenting as cervical lymphadenitis, usually tender and maybe associated with systemic symptoms. Despite the extensive literature on this disease, it continues to be misdiagnosed owing to its misleading clinical presentation. Results The mean age in our study was 26.9 (±11.3) years with a female majority of 65.7%. The clinical presentation of most cases was, tender swelling over the side of the neck ( n =50, 74.6%). On local examination, the mean length and width were 2.3 (±1.0) cm and 2.2 (±0.7) cm respectively. Histopathological examination revealed that most patients presented in the proliferative stage ( n =40, 59.7%). In 83.6% of the patients lymphadenopathy resolved in less than 2 months. Follow up of these patients over 9 months revealed that a vast majority of the patients underwent full recovery with symptomatic treatment ( n =42, 62.7%). Conclusions The disease is prevalent in young, female patients of Asian descent and often presents as cervical lymphadenopathy. Early diagnosis with excisional lymph node biopsy is crucial to avoid unnecessary investigations and treatment. Treatment is only symptomatic unless complicated, where steroid therapy is considered. Kikuchi’s disease has an excellent prognosis with almost no risk of fatality.

scholarly journals Role of viruses in biliary atresia: news from mice and men

2018 ◽  
Vol 3 (2) ◽  
pp. 101-106 ◽  
Author(s):  
Claus Petersen ◽  
Omid Madadi-Sanjani
Keyword(s):  
Biliary Atresia ◽  
Inflammatory Process ◽  
Biliary Tree ◽  
Unknown Etiology ◽  
Symptomatic Therapy ◽  
Kasai Procedure ◽  
Current State ◽  
State Of Research ◽  
Oriented Approach

AbstractBiliary atresia (BA) is still an enigmatic disease of unknown etiology and cryptic pathomechanism. Despite the fact that BA is rated among rare diseases, it represents the most frequent indication for pediatric liver transplantation. Although every effort is made to elucidate the origin of the ongoing deterioration of liver function, no breakthrough has so far been achieved, which switches the surgical but symptomatic therapy to a cause-oriented approach. The nowadays leading hypothesis focuses on hepatotropic virus as a triggering agent for an autoimmunological self-limiting inflammatory process along the entire biliary tree. The present review highlights the current state of research on the factor “viruses in biliary atresia” in both patients undergoing the Kasai procedure and the virus-induced BA mouse model.

scholarly journals Eosinophilic Enterocolitis Diagnosed by Means of Upper Endoscopy and Colonoscopy with Random Biopsies Treated with Budenoside: A Case Report and Review of the Literature

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Ghulamullah Shahzad ◽  
Duane Moise ◽  
Seth Lipka ◽  
Kaleem Rizvon ◽  
Paul J. Mustacchia
Keyword(s):  
Inflammatory Process ◽  
Colonic Mucosa ◽  
Clinical Presentation ◽  
Age Group ◽  
Pediatric Age ◽  
Peripheral Eosinophilia ◽  
Review Of The Literature ◽  
Unique Case ◽  
Protein Losing Enteropathy ◽  
Pediatric Age Group

Intense infiltration of gastrointestinal and colonic mucosa with eosinophils or acidophilic gastroenteritis (EG) is a relatively uncommon picture for a pathologist endoscopist especially outside the pediatric age group and is highly suggestive of an ongoing chronic inflammatory process. Existing literature projected a hypothetical association with allergy but the exact pathophysiology is still unknown. Association with malabsorption, protein losing enteropathy, and refractory ulcers with gastrointestinal bleeding makes the clinical presentation more complicated. We present a unique case of diarrhea and abdominal pain in the clinical presentation with associated peripheral eosinophilia, asthma, and gastroesophageal reflux disease (GERD). The patient's symptoms abated after initiation of budesonide.

Atypical Presentation of Congenital Yellow Nail Syndrome in a 2-Year-Old Female

2013 ◽  
Vol 17 (1) ◽  
pp. 66-68 ◽  
Author(s):  
Michael Cecchini ◽  
Joseph Doumit ◽  
Nordau Kanigsberg
Keyword(s):  
Rare Case ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Positive Family History ◽  
Clinical Manifestations ◽  
Clinical Entity ◽  
Unknown Etiology ◽  
Close Monitoring ◽  
Yellow Nail Syndrome ◽  
Atypical Clinical Presentation

Background: Yellow nail syndrome (YNS) is a rare clinical entity of unknown etiology that is characterized by a triad of yellow nails, respiratory manifestations, and lymphedema. The condition appears in the mid- to later years of life and only rarely in childhood. We describe a rare case of YNS with an atypical clinical presentation consisting of only yellow and dystrophic nails in a 2- year-old female since birth. Objective: A case of congenital YNS with only dystrophic and yellow nails is reported. Methods and Results: A 2-year-old female presented with yellow nails since birth. There was no positive family history. Physical examination revealed 20 thickened, dystrophic, yellow nails with onycholysis. There was no evidence of respiratory manifestations or lymphedema. Conclusion: Although rare, YNS can present as a congenital clinical entity and persist after birth. Pediatric patients with YNS show different clinical manifestations than the classic adult patient. The presence of yellow and dystrophic nails in the absence of respiratory and lymphatic manifestations may be the only sign of pathology and warrants close monitoring as progression to more serious complications can occur.

scholarly journals Langerhans cell histiocytosis of liver

2020 ◽  
Vol 8 (8) ◽  
pp. 3079
Author(s):  
Swapna Samudrala ◽  
Anuradha Sekaran ◽  
Bharat Patodiya ◽  
Nageshwar Reddy Duvuru
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Survival Rates ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Hepatic Involvement ◽  
Organ Systems ◽  
Impact On Survival ◽  
Considerable Impact ◽  
Single Organ

Langerhans cell histiocytosis (LCH) is a rare disorder of unknown etiology caused by proliferation of Langerhans cells. It can involve single organ system to multi organ systems and clinical presentation is variable depending on the organ involved and have different prognosis. LCH is common in children when compared to adults. Hepatic involvement in adults is relatively rare. Liver involvement has considerable impact on survival rates. Histopathology and immunohistochemistry provide the definitive diagnosis. Authors report a case of Langerhans cell histiocytosis in a young adult with hepatic involvement.

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