Diagnosis of oesophageal mucormycosis managed with medical therapy alone.

Mucormycosis is an invasive mould that can cause aggressive infection, particularly in immunocompromised patients. Though oesophageal mucormycosis is relatively rare, it remains an elusive and devastating manifestation of this disease. The management is also challenging, due to surgical morbidity and contraindications such as thrombocytopenia in immunocompromised hosts. In this report, we present the case of a 60-year-old Lebanese man with newly diagnosed acute myeloid leukaemia who developed oesophageal mucormycosis after induction chemotherapy with idarubicin/cytarabine (7+3). The diagnosis was made when the patient developed febrile neutropenia and odynophagia. CT scan of the chest revealed a thickened oesophagus. Oesophagogastroduodenoscopy with biopsy, histopathology and PCR were performed, resulting in the diagnosis of Rhizopus microsporus The patient was successfully treated with liposomal amphotericin B and salvage posaconazole therapy without surgical intervention. We reviewed the clinical characteristics of the six published oesophageal mucormycosis reports from the literature.

Immunoglobulin G4-Related Disease Manifesting as Isolated, Typical, and Nontypical Gastroesophageal Lesion: A Research of Literature Review.

BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune inflammatory and fibrotic condition. The disease is characterized by tissue infiltration with dense lymphoplasmacytes and IgG4-positive plasma cells. SUMMARY: The aim of this study was to provide gastroenterologists with novel insights into evaluating the gastroesophageal involvement with IgG4-RD or mimickers of this condition and to give special attention to clinicopathological features. A literature review was performed using the PubMed database. A total of 39 studies presenting cases in the form of isolated, typical, and nontypical gastroesophageal involvement with IgG4-RD published between 2010 and 2018 were included. These studies were thoroughly reviewed for symptoms, lesion location, lesion type, lesion size, immune-histopathology, associated diseases, treatment, and follow-up. Of the 39 studies reviewed, 9 were esophageal IgG4-RD lesions, isolated esophageal IgG4-RD 66.66% (6/9), a typical form of esophageal IgG4-RD 11.11% (1/9), and nontypical form esophageal IgG4-RD 22.22% (2/9). The 30 gastric IgG4-RD that include isolated gastric IgG4-RD 46.66% (14/30), typical gastric IgG4-RD 40% (12/30), and nontypical gastric IgG4-RD 13.33% (4/30). The majority of lesions were inflammatory tumors, ulceration, nodular lesions, chronic gastritis, and malignant lesions. Key Messages: IgG4-RD may be manifested by isolated, typical and nontypical forms of gastroesophageal lesions and should be taken into consideration in the differential diagnosis. Corticosteroids may be the sole diagnostic treatment for this condition.

Oesophageal pemphigoid: a rare cause of dysphagia.

Pemphigus vulgaris (PV) is a rare autoimmune bullous disease which affects the skin and mucous membranes. Oesophageal involvement is rare and has previously been limited to case reports and case series. A recent large case series of 477 PV patients showed that 26/477 (5.4%) had symptomatic oesophageal involvement. We present the case of a 54-year-old Somalian lady with a 10-year history of cutaneous PV, currently in remission, who developed dysphagia and odynophagia and was subsequently found to have oesophageal PV involvement with multiple flaccid bullae which were positive for anti-DSG3 antibodies on in-direct immunofluorescence. She had her treatment switched from azathioprine to mycophenolate and prednisolone, leading to resolution of her symptoms.

Structured Diagnostic Approach and Risk Assessment in Mucous Membrane Pemphigoid with Oesophageal Involvement.

Oesophageal involvement in mucous membrane pemphigoid is considered rare, but it may be underdiagnosed. To assess the incidence of oesophageal involvement in a group of patients with newly diagnosed mucous membrane pemphigoid we retrospectively analysed the medical records of 30 consecutive patients with mucous membrane pemphigoid diagnosed between 2006 and 2016 at the Department of Dermatology, University Hospital Würzburg. Twenty-one patients (70%) reported symptoms indicative of oesophageal mucous membrane pemphigoid. Twelve patients (40%) underwent oesophagogastroduodenoscopy, and oesophageal pathology compatible with mucous membrane pemphigoid was endoscopically found in 9 cases (30%). In all patients indirect and direct immunofluorescence were performed. Patients with and without oesophageal involvement did not differ with regard to the results of indirect immunofluorescence on salt-split human skin and monkey oesophagus. Study results demonstrate the necessity of a standardized diagnostic work-up, including adequate tissue samples for direct immunofluorescence, to prevent underdiagnosis of oesophageal mucous membrane pemphigoid.

Mixed connective tissue disease-enigma variations?

In 1972, Sharp et al. described a new autoimmune rheumatic disease that they called MCTD, characterized by overlapping features of SSc, SLE, PM/DM, high levels of anti-U1snRNP and low steroid requirements with good prognosis. MCTD was proposed as a distinct disease. However, soon after the original description, questions about the existence of such a syndrome as well as disputes over the features initially described began to surface. The conundrum of whether MCTD is a distinct disease entity remains controversial. We undertook a literature review, focusing on the articles reporting new data about MCTD published in the last decade, to determine whether any new observations help to answer the conundrum of MCTD. After reviewing recent data, we question whether the term MCTD is appropriately retained, preferring to use the term undifferentiated autoimmune rheumatic disease.

Rare cause of odynophagia: Giant esophageal ulcer.

Gastrointestinal complications are a frequent cause of morbidity after transplantation and may affect up to 40% of kidney transplant recipients. Here we report a rare case of idiopathic giant esophageal ulcer in a kidney transplant recipient. A 37-year-old female presented with a one-week history of odynophagia and weight loss. Upon admission, the patient presented cold sores, and a quantitative cytomegalovirus polymerase chain reaction was positive (10(5) copies/mL). An upper endoscopy demonstrated the presence of a giant ulcer. Serological test and tissue biopsies were unable to demonstrate an infectious origin of the ulcer. Immunosuppression was reduced and everolimus was introduced. An empirical i.v. therapy with acyclovir was started, resulting in a dramatic improvement in symptoms and complete healing of the ulcer. Only two cases of idiopathic giant esophageal ulcer in kidney transplant recipients have been reported in the literature; in both cases, steroid therapy was successful without recurrence of symptoms or endoscopic findings. However, this report suggests that correction of immune imbalance is mandatory to treat such a rare complication.

Risk factors for AIDS-defining illnesses among a population of poorly adherent people living with HIV/AIDS in Atlanta, Georgia.

In order to achieve the programmatic goals established in the National HIV/AIDS Strategy, virologic suppression remains the most important outcome within the HIV care continuum for individuals receiving antiretroviral therapy (ART). Therefore, clinicians have dedicated substantial resources to improve adherence and clinic retention for individuals on ART; however, these efforts should be focused first on those most at risk of morbidity and mortality related to AIDS. Our study aimed to characterize the factors that are associated with AIDS-defining illnesses (ADIs) amongst people living with HIV (PLHIV) who are poorly adherent or retained in care in order to identify those at highest risk of poor clinical outcomes. We recruited 99 adult PLHIV with a history of poor adherence to ART, poor clinic attendance, or unsuppressed viral load (VL) from the Infectious Disease Program (IDP) of the Grady Health System in Atlanta, Georgia between January and May 2011 to participate in a survey investigating the acceptability of a financial incentive for improving adherence. Clinical outcomes including the number of ADI episodes in the last five years, VLs, and CD4 counts were abstracted from medical records. Associations between survey items and number of ADIs were performed using chi-square analysis. In our study, 36.4% of participants had ≥1 ADI in the last five years. The most common ADIs were Pneumocystis jirovecii pneumonia, recurrent bacterial pneumonia, and esophageal candidiasis. Age <42.5 years (OR 2.52, 95% CI = 1.08-5.86), male gender (OR 3.51, 95% CI = 1.08-11.34), CD4 nadir <200 cells/µL (OR 11.92, 95% CI = 1.51-94.15), unemployment (OR 3.54, 95% CI = 1.20-10.40), and travel time to clinic <30 minutes (OR 2.80, 95% CI = 1.20-6.52) were all significantly associated with a history of ≥1 ADI in the last five years. Awareness of factors associated with ADIs may help clinicians identify which poorly adherent PLHIV are at highest risk of HIV-related morbidity.

Proton pump inhibitor responsive esophageal eosinophilia, a distinct disease entity?

Recent studies have suggested the existence of a patient population with esophageal eosinophilia that responds to proton pump inhibitor therapy. These patients are being referred to as having proton pump inhibitor responsive esophageal eosinophilia (PPI-REE), which is currently classified as a distinct and separate disease entity from both gastroesophageal reflux disease (GERD) and eosinophilic esophagitis (EoE). The therapeutic effect of proton pump inhibitor (PPI) on PPI-REE is thought to act directly at the level of the esophageal mucosa with an anti-inflammatory capacity, and completely independent of gastric acid suppression. The purpose of this manuscript is to review the mechanistic data of the proposed immune modulation/anti-inflammatory role of the PPI at the esophageal mucosa, and the existence of PPI-REE as a distinct disease entity from GERD and EoE.

[Esophageal aspergillosis in a patient with acute myelogenous leukemia and febrile neutropenia]. / Aspergilosis esofágica en una paciente con leucemia mieloide aguda y neutropenia febril.

Aspergillosis usually compromises the respiratory system, but can also affect others. We report a 46 yo female with acute myeloid leukemia, developed febrile neutropenia and dysphagia. Endoscopy revealed esophageal cytomegalovirus-like ulcers, but biopsies showed Aspergillus spp. It's important to consider aspergillosis in the differential diagnosis of esophageal lesions in high-risk patients.

Occupational egg allergy in an embryological research facility.

BACKGROUND: Hen and quail eggs are commonly used in embryological research. While immunoglobin E (IgE)-associated allergy to hens' egg proteins is recognized in employees in the food industry, there are no previous reports from workers in embryological research. Two newly identified cases prompted us to examine the extent of this problem in a university laboratory. AIMS: To determine the prevalence and determinants of sensitization to egg among a group of workers in an embryological research laboratory. METHODS: Following the identification of egg sensitization in two research workers, we surveyed 116 employees from a single embryology research laboratory in 2005. Sensitization to egg was assessed by skin prick tests and/or specific IgE measurement and examined in relation to a number of potential determinants, including the extent of appropriate control measures. RESULTS: Four employees were sensitized to egg, each with digestive symptoms of egg allergy. All had worked directly with eggs, giving a prevalence of specific sensitization in that group of 6.1% (95% confidence interval (CI) [1.7%, 14.8%]). There was a statistically significant trend for those sensitized to report a shorter duration of exposure to eggs and less frequent use of hygiene control measures to reduce exposure. CONCLUSIONS: Sensitization to eggs among those occupationally exposed to egg protein in research work occurs more commonly than in the general population. The presence of digestive symptoms after eating eggs may be indicative of sensitization to egg and should be incorporated into health evaluation of exposed workers.

[A case of esophageal actinomycosis in a patient with normal immunity].

Actinomycosis is a chronic suppurative disease and caused by Actinomycosis species, principally Actinomyces israelii, which are part of the normal inhabitant on the mucous membrane of the oropharynx, gastrointestinal tract, and urogenital tract. It usually affects cervicofacial, thoracic and abdominal tissue. Cervicofacial type has the highest percentage of occurrence with 50%. Actinomycosis frequently occurs following dental extraction, jaw surgery, chronic infection or poor oral hygiene. It may also be considered as an opportunistic infection in immunocompromised patients such as malignancy, human immunodeficiency virus infection, diabetes mellitus, steroid usage or alcoholism. But, actinomycosis rarely occurs in adults with normal immunity and rare in the esophagus. We report an unusual case of esophageal actinomycosis which was developed in a patient with normal immunity and improved by therapy with intravenous penicillin G followed oral amoxicillin, and we also reviewed the associated literature.