scholarly journals A Diagnostically Challenging Case of Febrile Ulceronecrotic Mucha-Habermann Disease in an Adult Female Successfully Treated with Methotrexate and Cyclosporine

2021 ◽  
pp. 12-17
Author(s):  
Tracy Ngo ◽  
Claudia Hossain ◽  
Jason Cohen ◽  
Beth McLellan ◽  
Rachel Blasiak ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Acute Onset ◽  
Methotrexate Therapy ◽  
Diagnostic Challenge ◽  
Fatal Disease ◽  
Pityriasis Lichenoides ◽  
Cyclosporine Therapy ◽  
Intravenous Vitamin ◽  
Variable Presentation ◽  
Prompt Diagnosis

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by intermittent pyrexia, acute onset of generalized ulceronecrotic lesions, and histopathology suggestive of PLEVA. Prompt diagnosis and treatment are necessary to halt the progression of this potentially fatal disease; however, the widely variable presentation of FUMHD in addition to its rarity poses a diagnostic challenge. We report the case of a previously healthy 43-year-old woman who presented to the emergency department with 1 month of generalized rash and intermittent fevers. Her only reported new exposure were elective intravenous vitamin infusions received at a medi-spa 1 week prior to onset of lesions. Initial evaluations were inconclusive, and confluent ulceronecrotic, hemorrhagic lesions appeared on approximately 90% of her body despite steroid, antibiotic, and cyclosporine therapy. Repeat histopathology was consistent with PLEVA, and in the context of her clinical presentation she was diagnosed with FUMHD. The patient rapidly attained remission with methotrexate therapy but sustained residual scarring.

scholarly journals Intraventricular neurocysticercosis: 18 consecutive patients and review of the literature

2002 ◽  
Vol 12 (6) ◽  
pp. 1-7 ◽  
Author(s):  
Albert C. Cuetter ◽  
Russell J. Andrews
Keyword(s):  
Clinical Presentation ◽  
Case Series ◽  
Surgical Removal ◽  
Review Of The Literature ◽  
Resonance Imaging ◽  
Fatal Disease ◽  
Effective Manner ◽  
Noncommunicating Hydrocephalus ◽  
The Brain ◽  
Prompt Diagnosis

Object The prognosis for intraventricular neurocysticercosis (IVNCC) is poorer than that for parenchymal NCC, making prompt diagnosis and treatment especially important. Although active, viable intraventricular cysts produce no reaction in the host; they can cause noncommunicating hydrocephalus, the onset of which is frequently abrupt. With the death of the larva comes a local granulomatous ependymitis, generalized ventriculitis, and meningoencephalitis. The authors report on 18 patients with IVNCC (accrued from a larger case series of 62 patients with NCC treated over an 11-year period), detailing clinical presentation, neuroimaging findings, treatment, and outcome. Methods All patients presented with hydrocephalus and/or meningitis. The most valuable diagnostic tests were magnetic resonance imaging of the brain and EITB of serum and/or cerebrospinal fluid. Treatment included albenda-zole and steroid therapy in all cases, and when necessary in cases requiring urgent or emergency ventriculostomy and/or surgical removal of the obstructing cyst (followed by shunt placement if indicated). Conclusions An extensive review of the literature on IVNCC has been prepared, with the goal of providing the reader with the information necessary to diagnose and treat this complex and potentially fatal disease in a timely and effective manner.

scholarly journals Haemophagocytic lymphohistiocytosis in pregnancy: a pertinent case during the COVID-19 pandemic

2021 ◽  
Vol 14 (9) ◽  
pp. e244716
Author(s):  
Jacqueline Potts ◽  
Brittney Gaudet ◽  
Danielle Durie ◽  
Joanne Quinones
Keyword(s):  
Caesarean Delivery ◽  
Tertiary Care ◽  
Care Facility ◽  
Febrile Illness ◽  
Pregnant Patient ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Diagnostic Challenge ◽  
Fatal Disease ◽  
Tertiary Care Facility ◽  
Prompt Diagnosis

Haemophagocytic lymphohistiocytosis (HLH) is a rare, often fatal disease, and presents a diagnostic challenge in the pregnant patient. This challenge is particularly relevant during the current COVID-19 pandemic. We present a case of HLH in a pregnant woman presenting with fever predating the COVID-19 pandemic. A 33-year-old, gravida 2, para 1 at 27 weeks’ gestation presented with fever, transaminitis, thrombocytopenia and elevated ferritin. After treatment according to the HLH-94 protocol, caesarean delivery and weeks of intensive care, the patient recovered fully. With prompt diagnosis and a multispecialty team at our tertiary care facility, she and her baby overcame a dire prognosis. HLH should be considered in pregnant patients presenting with a febrile illness. Particularly in cases of severe COVID-19, secondary HLH must be considered as an associated diagnosis.

scholarly journals Tuberculosis in Otorhinolaryngology: Clinical Presentation and Diagnostic Challenges

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Rajiv C. Michael ◽  
Joy S. Michael
Keyword(s):  
Mycobacterium Tuberculosis ◽  
Pulmonary Tuberculosis ◽  
Clinical Presentation ◽  
Extrapulmonary Tuberculosis ◽  
Common Type ◽  
The Body ◽  
Diagnostic Challenge ◽  
Cervical Adenitis ◽  
Important Form

Tuberculosis affects all tissues of the body, although some more commonly than the others. Pulmonary tuberculosis is the most common type of tuberculosis accounting for approximately 80% of the tuberculosis cases. Tuberculosis of the otorhinolaryngeal region is one of the rarer forms of extrapulmonary tuberculosis but still poses a significant clinical and diagnostic challenge. Over three years, only five out of 121 patients suspected to have tuberculosis of the otorhinolaryngeal region (cervical adenitis excluded) hadMycobacterium tuberculosisculture-proven disease. Additional 7 had histology-proven tuberculosis. Only one patient had concomitant sputum-positive pulmonary tuberculosis. We look at the various clinical and laboratory aspects of tuberculosis of the otorhinolaryngeal region that would help to diagnose this uncommon but important form of extrapulmonary tuberculosis.

Compression neuropathies of the forearm: anatomy, clinical features and management

2021 ◽  
pp. 1-10
Author(s):  
Alexander Scarborough ◽  
Robert J MacFarlane ◽  
Michail Klontzas ◽  
Rui Zhou ◽  
Mohammad Waseem
Keyword(s):  
Peripheral Nerve ◽  
Brachial Plexus ◽  
Upper Limb ◽  
Peripheral Nerves ◽  
Clinical Presentation ◽  
Permanent Damage ◽  
Peripheral Nerve Lesions ◽  
Clinical Presentations ◽  
Wide Range ◽  
Prompt Diagnosis

The upper limb consists of four major parts: a girdle formed by the clavicle and scapula, the arm, the forearm and the hand. Peripheral nerve lesions of the upper limb are divided into lesions of the brachial plexus or the nerves arising from it. Lesions of the nerves arising from the brachial plexus are further divided into upper (proximal) or lower (distal) lesions based on their location. Peripheral nerves in the forearm can be compressed in various locations and by a wide range of pathologies. A thorough understanding of the anatomy and clinical presentations of these compression neuropathies can lead to prompt diagnosis and management, preventing possible permanent damage. This article discusses the aetiology, anatomy, clinical presentation and surgical management of compressive neuropathies of the upper limb.

scholarly journals Inguinal ulcerated sebaceous carcinoma: an unusual presentation

2013 ◽  
Vol 88 (6 suppl 1) ◽  
pp. 48-51 ◽  
Author(s):  
David Oschilewski Lucares ◽  
Fred Bernardes Filho ◽  
Hernando Vega ◽  
Bernard Kawa Kac ◽  
Maria Rita Pereira ◽  
...  
Keyword(s):  
Male Patient ◽  
Clinical Manifestation ◽  
Clinical Presentation ◽  
Sebaceous Carcinoma ◽  
The Body ◽  
Skin Tumor ◽  
Diagnostic Challenge ◽  
Elderly Male ◽  
Genital Ulcers ◽  
Clinical Description

Sebaceous carcinoma is a rare and aggressive skin tumor. It can be located in any area of the body, the most commonly involved area being the periorbital region. It does not entail a typical clinical presentation, which explains the often late diagnosis. The aim of this report is to outline the rarity of the disease and its atypical clinical description, since to this day, inguinal ulcers with clinical manifestation have not been reported. We present and discuss a case of sebaceous carcinoma with an unusual clinical presentation, in an elderly male patient. The precise approach to genital ulcers, as shown in this case, is a diagnostic challenge that requires a great deal of effort on the part of the clinician.

scholarly journals Renal Leiomyosarcoma: A Diagnostic Challenge

2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Jose R. Valery ◽  
Winston Tan ◽  
Cherise Cortese
Keyword(s):  
Renal Mass ◽  
Early Stage ◽  
Flank Pain ◽  
Bone Lesions ◽  
Brush Cytology ◽  
Diagnostic Challenge ◽  
Male Smoker ◽  
Initial Biopsy ◽  
Pathologic Analysis ◽  
Prompt Diagnosis

Renal leiomyosarcoma is a very rare tumor that clinically and radiographically mimics more common renal malignancies. The infrequency of the condition makes it very difficult to diagnose. A 70-year-old male smoker presented with months of hematuria, right-sided flank pain, and weight loss. Imaging revealed a 3.8-centimeter renal mass that had characteristics similar to renal cell carcinoma. Initial biopsy of the mass was negative for malignancy. Two months later, subsequent imaging revealed what appeared to be metastatic bone lesions. Again, a biopsy of one of the lesions was negative for malignancy. Subsequent ureteral pyeloscopy, ureteroscopic renal pelvis biopsy, and brush cytology were negative for malignancy as well. The decision was made to perform nephrectomy for the removal of the mass. Pathologic analysis revealed renal leiomyosarcoma. This case illustrates the difficulty in diagnosing renal leiomyosarcoma. Repeated pathologic sampling was negative because of the tumor heterogeneity. Prompt diagnosis and treatment are very significant as surgical resection at an early stage offers the best prognosis.

scholarly journals Conservative management of eosinophilic enteritis presenting with acute abdominal syndrome

2013 ◽  
pp. 124-127
Author(s):  
Marco Bassi ◽  
Gelorma Belmonte ◽  
Carmelo Luigiano ◽  
Paola Billi ◽  
Angela Salerno ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Relevant Literature ◽  
Inflammatory Cells ◽  
Therapeutic Strategies ◽  
Acute Onset ◽  
Peripheral Eosinophilia ◽  
Diffuse Infiltration ◽  
Appropriate Therapy ◽  
Specific Symptoms

Eosinophilic enteritis, an increasing recognized condition, is rare and often presents with non-specific symptoms. We report a case of a 46-year old female who presented with acute onset abdominal pain and nausea associated with ascites, small bowel thickening and peripheral eosinophilia. Diagnosis was confirmed by biopsies taken at esophagogastroduodenoscopy demonstrating diffuse infiltration by inflammatory cells, mainly eosinophils. Appropriate therapy was instituted. The patient recovered well and was symptom-free at 1-month follow up. In this report, we discuss the clinical presentation and the diagnostic criteria of the eosinophilic enteritis, and examine the pathophysiological theories and therapeutic strategies. The relevant literature on eosinophilic enteritis is summarized.

scholarly journals Clinical and angioarchitectural characteristics of spinal vascular malformations of the cervical spine

2020 ◽  
Vol 32 (5) ◽  
pp. 755-762 ◽  
Author(s):  
Waleed Brinjikji ◽  
Elisa Colombo ◽  
Giuseppe Lanzino
Keyword(s):  
Cervical Spine ◽  
Lower Extremity ◽  
Clinical Presentation ◽  
Vascular Malformations ◽  
Case Series ◽  
Acute Onset ◽  
Lower Extremity Weakness ◽  
Arteriovenous Fistulas ◽  
Spinal Vascular Malformations ◽  
Interventional Neuroradiologist

OBJECTIVEVascular malformations of the cervical spine are exceedingly rare. To date there have been no large case series describing the clinical presentation and angioarchitectural characteristics of cervical spine vascular malformations. The authors report their institutional case series on cervical spine vascular malformations diagnosed and treated at their institution.METHODSThe authors retrospectively reviewed all patients with spinal vascular malformations from their institution from January 2001 to December 2018. Patients with vascular malformations of the cervical spine were included. Lesions were characterized by their angioarchitectural characteristics by an interventional neuroradiologist and endovascular neurosurgeon. Data were collected on clinical presentation, imaging findings, treatment outcomes, and long-term follow-up. Descriptive statistics are reported.RESULTSOf a total of 213 patients with spinal vascular malformations, 27 (12.7%) had vascular malformations in the cervical spine. The mean patient age was 46.1 ± 21.9 years and 16 (59.3%) were male. The most common presentations were lower-extremity weakness (13 patients, 48.1%), tetraparesis (8 patients, 29.6%), and lower-extremity sensory dysfunction (7 patients, 25.9%). Nine patients (33.3%) presented with hemorrhage. Fifteen patients (55.6%) had modified Rankin Scale scores of 0–2 at the time of diagnosis. Regarding angioarchitectural characteristics, 8 patients (29.6%) had intramedullary arteriovenous malformations (AVMs), 5 (18.5%) had epidural arteriovenous fistulas (AVFs), 4 (14.8%) had paraspinal fistulas, 4 (14.8%) had mixed epidural/intradural fistulas, 3 (11.1%) had perimedullary AVMs, 2 (7.4%) had dural fistulas, and 1 patient (3.7%) had a perimedullary AVF.CONCLUSIONSThis retrospective study of 27 patients with cervical spine vascular malformations is the largest series to date on these lesions. The authors found substantial angioarchitectural heterogeneity with the most common types being intramedullary AVMs followed by epidural AVFs, paraspinal fistulas, and mixed intradural/extradural fistulas. Angioarchitecture dictated the clinical presentation as intradural shunts were more likely to present with hemorrhage and acute onset myelopathy, while dural and extradural shunts presented as either incidental lesions or gradually progressive congestive myelopathy.

scholarly journals Recent advancements in management of gastrointestinal tuberculosis

2018 ◽  
Vol 5 (9) ◽  
pp. 3730
Author(s):  
Basim F. Khan ◽  
Ahmed M. Basha ◽  
Bandar R. Bakhurji ◽  
Bader J. Aldossari ◽  
Abdulaziz S. Alsumaihi ◽  
...  
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Good Accuracy ◽  
Clinical Presentation ◽  
Intestinal Tuberculosis ◽  
Scoring Systems ◽  
Abdominal Tuberculosis ◽  
Diagnostic Challenge ◽  
Important Concern ◽  
Diagnostic Modalities

Abdominal tuberculosis and its protean manifestations still create a worldwide diagnostic challenge for clinicians and remain an important concern in the developing world. Crohn’s disease, which is being increasingly recognized in countries where intestinal tuberculosis is prevalent, needs to be differentiated as the two diseases resemble each other in their clinical presentation, and in their radiological, endoscopic, and histological findings. New diagnostic modalities and scoring systems have facilitated the differentiation of Crohn’s disease from intestinal tuberculosis with good accuracy. Randomized trials have shown 6 months of therapy to be equivalent to longer durations of treatment for patients with abdominal tuberculosis. 

scholarly journals Molecular Diagnosis - An Indispensable Tool to Clinch the Diagnosis in Familial Haemophagocytic Lymphohistiocytosis – A Case Report with Review of Literature

2020 ◽  
Vol 7 (43) ◽  
pp. 2503-2507
Author(s):  
Nisha Bhaskar Meshramm ◽  
Rasika Uday Gadkari ◽  
Fatema Akbar Kamal
Keyword(s):  
Cytotoxic T Cells ◽  
High Morbidity ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Girl Child ◽  
Familial Form ◽  
Confirmatory Tests ◽  
Indispensable Tool ◽  
Familial Haemophagocytic Lymphohistiocytosis ◽  
Variable Presentation ◽  
Prompt Diagnosis

Haemophagocytic Lympho-Histiocytosis (HLH) includes variety of life-endangering conditions caused due to defective immunity and represented as uncontrolled hyperinflammatory response. HLH is mostly instigated by infection. Familial form is due to genetic defects in cytotoxic T cells & natural killer cells. HLH mostly goes unrecognized, and hence has high morbidity and mortality. Prompt diagnosis is critical to ensue curative treatment. Diagnosis of familial Haemophagocytic Lymphohistiocytosis is ellusive due to variable presentation, coexistence with other diseases, & select availability of confirmatory tests. We present a case of a 3-yearold girl child, with organomegaly, fever and pancytopenia diagnosis could not be reached for a long period.

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