scholarly journals The enigmatic fascia: eosinophilic fasciitis

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Jonathan A. Aun ◽  
Rachel S. Knox ◽  
Jared E. Roberts

Abstract This case report highlights the potentially underrecognized subtype of unilateral eosinophilic fasciitis (EF) in a 28 year old man. With fewer than 300 reported encounters to date, EF is a rare disease that eludes clinicians by presenting as a scleroderma like syndrome. As EF remains a clinical diagnosis, biopsy results may be nonspecific, and the disease can easily be misdiagnosed (or missed entirely) if a full thickness biopsy is not reviewed by a dermatopathologist. The authors also emphasize the importance of internationally accepted diagnostic criteria, of which at least two different sets exist.

Author(s):  
Sawako Hiroi ◽  
Michinori Hamaoka ◽  
Rie Yamamoto ◽  
Yasuhiro Matsugu ◽  
Takashi Nishisaka ◽  
...  

Lymphoepithelial cyst (LEC)is a rare disease. Clear diagnostic criteria have not been established, and the number of cases is expected to increase in the future. In particular, LEC in the pancreatic accessory spleen has not been reported in the past, and this report documents it for the first time.


Author(s):  
Kenneth Rockwood ◽  
Heather Davis ◽  
Chris MacKnight ◽  
Robert Vandorpe ◽  
Serge Gauthier ◽  
...  

Background:The Consortium to Investigate Vascular Impairment of Cognition (CIVIC) is a Canadian, multi-centre, clinic-based prospective cohort study of patients with Vascular Cognitive Impairment (VCI). We report its organization and the impact of diagnostic criteria on the study of VCI.Methods:Nine memory disability clinics enrolled patients and recorded their usual investigations and care. A case report form included all vascular dementia (VaD) individual criteria for each of four sets (National Institute of Neurological Disorders and Stroke (NINDS-AIREN), Alzheimer’s Disease Diagnostic Treatment Centers (ADDTC), the ICD-10 Classification of Mental and Behavioural Disorders (ICD-10), and the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV)) of consensus-based diagnostic criteria and for the Hachinski Ischemia Score (HIS). Investigators, having completed the case report form, were asked to make a clinical judgement about the cognitive diagnosis based on the best available information, including neuroimaging.Results:Of 1,347 patients (mean age 72 years; 56% women), 846 (63%) were diagnosed with dementia and 324 (24%) were diagnosed with VCI. The proportion of patients diagnosed with VaD by the diagnostic criteria was: 23.9% (n=322) by DSM-IV, 10.2% (n=137) by HIS, 4.3% (n=58) by ICD-10, 3.8% (n=51) by ADTCC, and 3.6% (n=48) by NINDS-AIREN. Judged against a clinical diagnosis of VaD, the sensitivity/specificity of each was: DSM-IV (0.77/0.80); HIS (0.41/0.92); ICD-10 (0.29/0.98); ADTCC (0.24/0.98); NINDS-AIREN (0.42/0.995). Compared with a clinical diagnosis of VCI, sensitivities were lower for the diagnostic criteria, reflecting the exclusion of patients who did not have dementia.Conclusions:Consensus-based criteria for VaD omit patients who do not meet dementia criteria that are modeled on Alzheimer’s disease. Even for patients who do, the proportion identified with VaD varies widely. Criteria based on empirical analyses need to be developed and validated.


Toxins ◽  
2021 ◽  
Vol 13 (12) ◽  
pp. 860
Author(s):  
Robertino Dilena ◽  
Mattia Pozzato ◽  
Lucia Baselli ◽  
Giovanna Chidini ◽  
Sergio Barbieri ◽  
...  

Infant botulism is a rare and underdiagnosed disease caused by BoNT-producing clostridia that can temporarily colonize the intestinal lumen of infants less than one year of age. The diagnosis may be challenging because of its rareness, especially in patients showing atypical presentations or concomitant coinfections. In this paper, we report the first infant botulism case associated with Cytomegalovirus coinfection and transient hypogammaglobulinemia and discuss the meaning of these associations in terms of risk factors. Intending to help physicians perform the diagnosis, we also propose a practical clinical and diagnostic criteria checklist based on the revision of the literature.


2021 ◽  
Vol 07 (02) ◽  
pp. e116-e120
Author(s):  
Tatjana Braun ◽  
Amelie De Gregorio ◽  
Lisa Baumann ◽  
Jochen Steinacker ◽  
Wolfgang Janni ◽  
...  

AbstractSplenosis is a rare disease, which is often discovered incidentally years after surgical procedures on the spleen or traumatic splenic lesions. Through injury of the splenic capsule, splenic cells are able to spread and autoimplant in a fashion similar to the process of metastatic cancer. Here we present the case of a 62-year-old female patient with a palpable tumor of the lower abdomen. Her medical history was unremarkable, except for splenectomy after traumatic splenic lesion in her childhood. Clinical examination and diagnostic imaging raised the suspicion of advanced ovarian cancer, which was further substantiated by the typical presentation of adnexal masses and disseminated peritoneal metastases during the following staging laparotomy. Surprisingly, we also found peritoneal implants macroscopically similar to splenic tissue. Microscopic examination of tissue specimens by intrasurgical frozen section confirmed the diagnosis of intra-abdominal splenosis. The patient then underwent cytoreductive surgery with complete resection of all cancer manifestations, sparing the remaining foci of splenosis to avoid further morbidity. This case demonstrates the rare coincidence of intra-abdominal carcinoma and splenosis, which could lead to intraoperative difficulties by misinterpreting benign splenic tissue. Therefore, splenosis should be considered in patients with medical history of splenic lesions and further diagnostic imaging like Tc-99m-tagged heat-damaged RBC scan could be used for presurgical distinguishing between tumor spread in the abdominal cavity and disseminated splenosis. The presented case report should not only raise awareness for the rare disease splenosis, but also emphasize the need to consider the possibility of simultaneous incidence of benign and malignant intra-abdominal lesions, as to our knowledge this is the first published case of simultaneous peritoneal carcinomatosis and splenosis.


2021 ◽  
pp. 239719832110043
Author(s):  
Paulina Śmigielska ◽  
Justyna Czarny ◽  
Jacek Kowalski ◽  
Aleksandra Wilkowska ◽  
Roman J. Nowicki

Eosinophilic fasciitis is a rare connective tissue disease of unknown etiology. Therapeutic options include high-dose corticosteroids and other immunosuppressive drugs. We present a typical eosinophilic fasciitis case, which did not respond to first-line treatment, but improved remarkably after infliximab administration. This report demonstrates that in case of initial treatment failure, infliximab might be a relatively safe and effective way of eosinophilic fasciitis management.


Perfusion ◽  
2021 ◽  
pp. 026765912098677
Author(s):  
Chuntian Li ◽  
Xin Mao ◽  
Xu Zhao ◽  
Guiqing Liu ◽  
Gang Xu ◽  
...  

Middle aortic syndrome (MAS) is a rare disease characterized by distal thoracic aorta or abdominal aorta coarctation, and thoraco-abdominal aortic bypass grafting is an effective treatment for this condition. However, significant trauma is associated with the conventional surgical approach. We report a 26-year-old woman with MAS who presented with hypertension and needed thoraco-abdominal bypass grafting. In this operation, we adopted the endoscopic technique to improve the conventional surgical approach (reduce the incision). This case report shows that it is safe and feasible to use an endoscopic technique to reduce the trauma during this kind of operation, and provides a reference for similar treatments.


2021 ◽  
Vol 42 (Supplement_1) ◽  
pp. S130-S131
Author(s):  
Andrew Khalifa ◽  
Anzar Sarfraz ◽  
Jacob B Avraham ◽  
Ronnie Archie ◽  
Matthew Kaminsky ◽  
...  

Abstract Introduction Electrical injuries represent 0.4–3.2% of admissions to burn units and are responsible for >500 deaths per year in the United States. Approximately half occur in the workplace and are the fourth leading cause of work-related-traumatic death. The extent of injury can be drastically underestimated by total body surface area percentage (TBSA). Along with cutaneous burns, high voltage electrical injuries can lead to necrosis of muscle, bone, nervous tissue, and blood vessels. Aggressive management allows for patient survival, but at significant cost. Newer technologic advances help improve functional outcomes. Methods This case-report was conducted via retrospective chart review of the case presented. Results A 43-year-old male sustained a HVEI (>10, 000 V) after contacting an active wire while working as a linesman for an electric company. He presented after less than 15-minute transport from an outside hospital with full thickness burns and auto-amputation to all fingers on both hands and the distal third of the left hand (Images 1 and 2). There were full thickness circumferential burns to the entire left and right upper extremities with contractures, with the burns extending into the axilla, and chest wall musculature. The patient had 4th degree burns and a large wound to the left shoulder with posterior extension to the scapula, flank and back with approximately 25% TBSA (Image 3). Compartments were tense in both upper extremities. Patient was sedated and intubated to protect the airway and placed on mechanical ventilation. A femoral central line was then placed, and the patient was given pain control, continued fluid resuscitation, and blood products. Dark red colored urine from a foley catheter that was immediately identified as rhabdomyolysis induced myoglobinuria. Labs drawn demonstrated elevated troponin I, CK >40,000. BUN 18, creatinine 1.0, K+ 5.2 and phosphate 5.6. Decision was made immediately for operative intervention with emergent amputation of both upper extremities in the light of rhabdomyolysis secondary to tissue necrosis and oliguria. During the patient’s hospital course, he underwent multiple operations for further debridement with vacuum-assisted closure therapy and skin grafting of sites, as well as targeted muscle reinnervation (TMR) 6 months later at an outside hospital. Conclusions Although HVEI only account for a small percentage of burn admissions, they are associated with greater morbidity than low-voltage injuries. Patients with HVEI often incur multiple injuries, more surgical procedures, have higher rates of complications, and more long term psychological and rehabilitative difficulties. Despite the need for amputation in some of these critically ill patients, options exist that allow for them to obtain long term functional success.


Author(s):  
Juliet E. Brown ◽  
Elise B. Russell ◽  
A. Russell Moore ◽  
Astrid Oscos‐Snowball ◽  
Andrew Stent ◽  
...  

2011 ◽  
Vol 17 (9) ◽  
Author(s):  
Sudip Kumar Ghosh ◽  
Debabrata Bandyopadhyay ◽  
Loknath Ghoshal ◽  
Susmit Haldar
Keyword(s):  

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