bluuebunny/crossref_metadata_2025_split huggingface dataset:
An Atypical Presentation of Pemphigus Vegetans in the Umbilicus
Authors: Jimenez Antonio , Hoyer Paige , Wilkerson Michael 
Published: September 2020

Pemphigus is a chronic, autoimmune bullous disease that affects the skin and mucous membranes. Pemphigus vegetans is a rare variant of pemphigus and presents as oral ulcerations with associated verrucous lesions in intertriginous or flexural areas. A 38-year-old African American woman presented to the clinic with a chief complaint of oral ulcers. She carried a diagnosis of Behcet’s disease and was referred by rheumatology for evaluation of treatment-resistant mucosal ulcerations. At the time of her dermatology visit, she also reported an enlarging umbilical mass that had been present for several months. Further examination of the umbilical lesion identified an exophytic, vegetative mass. Histologic assessment of the lesion identified acanthosis and acantholysis with dermal eosinophils consistent with pemphigus vegetans. A pemphigus antibody panel was done and resulted positive for IgG desmoglein-3 antibodies. The patient was treated with prednisone and rituximab with improvement of her lesions. We present an atypical presentation of pemphigus vegetans involving the umbilicus. This diagnosis should be considered in patients who present with oral erosions and concomitant vegetative lesions, regardless of location or prior diagnoses.
Extensive Tinea Associated with Tofacitinib Therapy Masquerading as New-Onset SCLE
Authors: Buechler Connor R , Kurland Elena , Veenstra Jesse 
Published: September 2020

Rheumatoid arthritis is a chronic autoimmune disorder that often requires treatment with immunomodulatory agents, however, such interventions are not without risk of opportunistic infection. Monitoring for such conditions is critical, whereas recognition and treatment can prove challenging at times, as they may manifest with an atypical presentation or masquerade as another condition entirely. We present here a case of extensive tinea corporis with concomitant tinea capitis masquerading as new-onset connective tissue disorder in a patient being treated for rheumatoid arthritis with the Janus kinase inhibitor tofacitinib.
Facial Papules in Birt-Hogg-Dubé Syndrome: A Growing Spectrum of Pathologic Findings
Authors: Yi Lauren G , Guerra Ricardo , Wu Sam B , Flowers Richard H 
Published: September 2020

Birt-Hogg-Dubé syndrome is rare genodermatosis caused by a mutation in the folliculin gene. It is characterized by pulmonary cysts, renal tumors, and a variety of skin manifestations including trichodiscomas, fibrofolliculomas, less commonly reported angiofibromas, and perifollicular fibromas. These cutaneous lesions have overlapping histopathologic features. Here we report a case of a man presenting with facial papules and nonspecific histology found to have Birt-Hogg-Dubé syndrome and a novel mutation in the folliculin gene. Our aim is to raise awareness of the spectrum of associated cutaneous and pathologic findings in Birt-Hogg-Dubé syndrome, which are not all included in the diagnostic criteria.
Dermatologists' Perceptions and Use of Electronic Health Record Systems
Authors: Katamanin Olivia , Glazer MD Alex M 
Published: September 2020

Introduction: Electronic Health Records (EHR) have been adopted and integrated into medical practices over the past 20 years. Many positive and negative implications have been described by physicians using EHR. This study aims to US dermatologists' perceptions and use of EHR within their clinical practice.
Methods: A validated survey was administered to US dermatologists at a national educational conference to assess use and perceptions of EHR.
Results Seventy-two percent (291/400) of those sampled completed greater than 90% survey and were included in outcome analysis. Eighty-six percent of the participants were currently using or had used EHR. Most dermatologists felt that EHR negatively impacted their workflow efficiency and face-to-face time with patients. A portion of dermatologists thought that EHR improved their documentation.
Limitations: Selection bias may have led those with strong beliefs with EHR more likely to complete the entire survey.
Conclusion: Despite widespread adoption, most dermatologists have a negative impression of EHR and felt that it interfered with their ability to effectively see patients. Interventions to improve EHR should focus on improving workflow efficiency and maximizing the amount of time dermatologists can spend with patients.
Integrating Electrical Impedance Spectroscopy into Clinical Decisions for Pigmented Skin Lesions Improves Diagnostic Accuracy: A Multitiered Study
Authors: Litchman Graham H , Marson Justin W , Svoboda Ryan M , Rigel Darrel S 
Published: September 2020

Introduction: The number-needed-to-biopsy (NNB) metric measures the efficiency of a clinician’s ability to accurately diagnose and recommend pigmented skin lesions (PSLs) for biopsy for suspected melanomas. Electrical impedance spectroscopy (EIS) is a non-invasive technique that measures differences in resistance between healthy and cancerous skin cells, intended as an aid to enhance diagnostic accuracy.Methods: Dermatology clinicians of three distinct groups (residents, physician assistants/nurse practitioners, and practicing dermatologists) were evaluated on their ability to accurately recommend suspect PSLs for biopsy before and after the integration of EIS data.Results: All three groups had a reduction in NNB after the inclusion of EIS. Instances of missed biopsies for malignant melanoma were significantly reduced with simultaneous significant reductions in unnecessary biopsies for benign lesions. There was a material improvement of biopsy selection for PSLs having clinically challenging features. EIS also greatly improved the diagnostic acumen of clinicians whose assessments were less accurate than their peers prior to EIS incorporation.Conclusions: The integration of EIS technology into the PSL biopsy decision was demonstrated to be effective in significantly enhancing clinician NNB and more accurate PSL biopsy selection.
Cetuximab Induced Hidrocystomas: A Case Report
Authors: Nagrani Nicole , Castillo David E , Miteva Mariya , Nichols Anna 
Published: September 2020

Cetuximab is an epidermal growth factor receptor (EGFR) inhibitor that commonly results in follicular-based acneiform eruptions. EGFR is expressed in the epidermis, hair follicle epithelium, sweat gland apparatus, and plays an important role in the differentiation and development of the hair follicle. In this report we describe a 70-year-old man who developed an acneiform eruption on his nose, cheeks, neck, and back when cetuximab was started for metastatic colorectal carcinoma. This initial eruption improved with cessation of cetuximab but left residual cystic papules on his nose and multiple superficial white cysts on his bilateral cheeks, neck and back. Skin biopsy of a representative lesion on the nose revealed a cyst-like cavity lined with epithelium similar to sweat glands within the dermis consistent with a hidrocystoma. In this case, it is plausible that the use of an EGFR inhibitor resulted in a cutaneous inflammatory reaction, that subsequently healed with blockage of the sweat duct apparatus causing the formation of cutaneous cysts, including both hidrocystomas and milia. Alternatively, the blockage of the duct may have resulted from inhibition of basal cell migration and increased cell adhesion within the eccrine gland causing accumulation of eccrine gland secretion, and eventually hidrocystomas. To our knowledge, this is the first case describing the resolution of a typical cetuximab-induced acneiform eruption with residual hidrocystomas and milia.
Reproducibility of a new classification of the anterior clinoid process of the sphenoid bone
Authors: Chaddad-Neto Feres , Costa Marcos Devanir Silva da , Santos Bruno , Caramanti Ricardo Lourenco , Costa Bruno Lourenco , Doria-Netto Hugo Leonardo , Figueiredo Eberval Gadelha 
Published: September 2020

Background:
Pneumatization of the anterior clinoid process (ACP) affects paraclinoid region surgery, this anatomical variation occurs in 6.6–27.7% of individuals, making its preoperative recognition essential given the need for correction based on the anatomy of the pneumatized process. This study was conducted to evaluate the reproducibility of an optic strut-based ACP pneumatization classification by presenting radiological examinations to a group of surgeons.

Methods:
Thirty cranial computer tomography (CT) scans performed from 2013 to 2014 were selected for analysis by neurosurgery residents and neurosurgeons. The evaluators received Google Forms with questionnaires on each scan, DICOM files to be manipulated in the Horos software for multiplanar reconstruction, and a collection of slides demonstrating the steps for classifying each type of ACP pneumatization. Interobserver agreement was calculated by the Fleiss kappa test.

Results:
Thirty CT scans were analyzed by 37 evaluators, of whom 20 were neurosurgery residents and 17 were neurosurgeons. The overall reproducibility of the ACP pneumatization classification showed a Fleiss kappa index of 0.49 (95% confidence interval: 0.49–0.50). The interobserver agreement indices for the residents and neurosurgeons were 0.52 (0.51–0.53) and 0.49 (0.48–0.50), respectively, and the difference was statistically significant (P < 0.00001).

Conclusion:
The optic strut-based classification of ACP pneumatization showed acceptable concordance. Minor differences were observed in the agreement between the residents and neurosurgeons. These differences could be explained by the residents’ presumably higher familiarity with multiplanar reconstruction software.
Spontaneous improvement of secondary empty sella syndrome due to re-expansion of an intrasellar cyst: A case report
Authors: Yamada Shodai , Yagi Kenji , Hirano Kazuhiro , Uno Masaaki 
Published: September 2020

Background:
In patients with secondary empty sella syndrome (ESS), optic nerve herniation into the sella turcica is caused by shrinkage of the mass lesion at the sella turcica, resulting in visual disturbance. ESS is often surgically treated using chiasmapexy. Here, we report the first case of spontaneous improvement in a patient with ESS.

Case Description:
A 69-year-old woman presented with a month-long history of visual disturbance in the right eye, poor visual acuity, and quadrantanopia in her upper temporal visual field. Magnetic resonance (MR) imaging showed herniation of her right optic nerve and gyrus rectus into the sella turcica. The visual disturbance gradually improved, and the patient’s vision became almost normal after a month without any treatment. On repeated MR imaging, it was observed that the herniation of the right optic nerve and gyrus rectus disappeared due to an intrasellar cyst re-expansion. The secondary ESS caused by the shrinkage of the intrasellar cyst resulted in the visual disturbance and re-expansion of the cyst resulted in spontaneous improvement of symptoms. The visual disturbance did not recur for a year.

Conclusion:
Patients with secondary ESS without severe symptoms may be followed up conservatively. However, surgical treatment should be applied if symptoms deteriorate or do not improve.
One-year follow-up for type II odontoid process fractures in octogenarians: Is there a place for surgical management?
Authors: Borsotti Francois , Starnoni Daniele , Ecker Timo , Coll Juan Barges 
Published: September 2020

Background:
Type II odontoid fractures are becoming one of the most common injuries among elderly patients and are associated with increased morbidity rates. Here, we compared the safety/efficacy of conservative versus surgical treatment for type II C2 fractures and, in particular, evaluated the complications, hospital lengths of stay, and mortality rates for patients over 80 years of age.

Methods:
We retrospectively reviewed the records of 63 nonsurgically versus 18 surgically treated C2 fractures in patients over 80 years of age (2003–2018). Cervical computed tomography images, X-rays, and magnetic resonance images were reviewed by both a neurosurgeon and a neuroradiologist. The following patient data were included in the analysis; Glasgow Coma Scale score, injury severity score, the abbreviated injury scale scores, their comorbidities (e.g., utilizing the Charlson comorbidity index), their primary outcomes, and mortality rates (e.g., at 6 weeks and 1 year after treatment).

Results:
Eighty-one patients were included in the study; 63 were treated conservatively and 18 underwent surgical management of type II C2 fractures. Patients averaged 87.0 ± 5.0 years of age, and their combined mortality rates were 13.6% at 6 weeks and 25.9% at 1 year. Notably, at 1 year, the mortality rates were not statistically different between the two groups: 18 (30.0%) patients from the conservatively treated group versus 3 (16.7%) patients from the surgically managed patients died indicating (e.g., using the Kaplan–Meier analysis) no survival advantage for either treatment strategy.

Conclusion:
Surgical versus conservative management of type II odontoid fractures were associated with comparable high mortality rates at 1 year.
Profile of genetic variations in severely calcified carotid plaques by whole-exome sequencing
Authors: Katano Hiroyuki , Nishikawa Yusuke , Yamada Hiroshi , Iwata Takashi , Mase Mitsuhito 
Published: September 2020

Background:
The precise mechanisms of carotid calcification and its clinical significance have not been established.

Methods:
We classified ten plaques from carotid endarterectomy patients into high- and low-calcified plaques based on the Agatston calcium scores. We performed whole-exome sequencing for genetic profiles with single nucleotide variations (SNVs), insertions, and deletions. Bioinformatic data mining was then conducted to disclose specific gene variations to either high- or low-calcified carotid plaques.

Results:
In the carotid plaques, G:C>A:T/C:G>T:A transitions as SNVs, insT after C/insC after A as insertions, and delA after G/delT after C as deletions were most frequently observed, but no significant difference was observed between the high- and low-calcified plaque groups in their proportion of base-pair substitution types. In the bioinformatic analysis, SNVs of ATP binding cassette subfamily C member 6 (ADCC6) were more commonly found in high-calcified plaques and SNVs of KLKB1 were more commonly found in low-calcified plaques compared to the other group. No new genetic variants related to calcification or atherosclerosis among those not registered in dbSNP was detected.

Conclusion:
Our findings clarified the features of base-pair substitutions in carotid plaques, showing no relation to calcification. However, genetic variants in ADCC6 relating to vascular calcification for high-calcified plaques, and in KLKB1 encoding kallikrein associated with vascular regulation of atherosclerosis for low-calcified plaques were more specifically extracted. These results contribute to a better understanding of the genetic basis of molecular activity and calcium formation in carotid plaques.
A rare case of spinal dural arteriovenous fistula mimicking malignant glioma of the medulla oblongata: Significance of cerebral angiography for accurate diagnosis of brain stem region
Authors: Shigekawa Seiji , Inoue Akihiro , Nakamura Yawara , Kohno Daisuke , Tagawa Masahiko , Kunieda Takeharu 
Published: September 2020

Background:
The findings of a hyperintense sign on T2-weighted imaging (T2-WI) and gadolinium (Gd) contrast enhancement on magnetic resonance imaging (MRI) of the brain stem suggest malignant glioma. However, this pathological condition is probably uncommon, and it may be unknown that a dural arteriovenous fistula (DAVF) can imitate this radiological pattern. In addition, it is extremely rare to be caused by a spinal DAVF. Here, a rare case of spinal DAVF that mimicked malignant glioma of the medulla oblongata is presented.

Case Description:
A 56-year-old woman was admitted with a progressive gait disturbance, vertigo, and dysphasia. MRI showed a hyperintense signal in the medulla oblongata on fluid-attenuated inversion recovery (FLAIR) and moderate contrast enhancement on Gd-enhanced MRI. Interestingly, Gd-enhanced MRI demonstrated abnormal dilated veins around the brain stem and cervical spinal cord. Cerebral angiography showed spinal DAVF at the left C4/C5 vertebral foramen fed by the C5 radicular artery. The fistula drained into spinal perimedullary veins and flowed out retrograde at the cortical vein of the posterior cranial fossa. Therefore, surgical disconnection of the spinal DAVF was performed by a posterior approach. The patient’s postoperative course was uneventful. Cerebral angiography showed complete disappearance of the DAVF, with marked reductions of the hyperintense sign of the medulla oblongata on FLAIR.

Conclusion:
This important case illustrates MRI findings mimicking brain stem glioma. In cases with the hyperintense sign-on T2-WI associated with contrast enhancement suspicious of brainstem glioma, careful checking for perimedullary abnormal vessels and additional cerebral angiography should be performed.
Rare spondylodiscitis due to Mycobacterium mucogenicum
Authors: Furnari Massimo , Scalia Gianluca , Umana Giuseppe Emmanuele , Giuffrida Massimiliano , Ponzo Giancarlo , Garozzo Sebastiano Fabio , Nicoletti Giovanni Federico 
Published: September 2020

Background:
Nontuberculous mycobacteria (NTM) represents an important cause of infection, particularly in immunocompromised patients. Spondylodiscitis is unusual and may be associated with underlying causes such as drug abuse. Timely diagnosis and treatment are critical, as without this, patients will demonstrate progressive neurological deterioration. Here, we present a rare case of Mycobacterium mucogenicum spondylodiscitis in a 36-year-old male, along with a focused literature review.

Case Description:
A 36-year-old female with previous drug abuse presented with 3-years of progressive thoracolumbar pain. The MRI of the spine revealed paravertebral abscesses from Th10–L1 with vertebral lesions involving Th11–Th12 levels (e.g., vertebral body collapse/deformity and destruction of the posterior vertebral walls). After a needle CT-guided biopsy of the paravertebral tissues, real time-polymerase chain reaction (RT-PCR) amplification documented NTM; the final identification was M. mucogenicum. The patient then underwent a Th11–Th12 decompressive laminectomy, facetectomy, granulomatous tissue debridement, and posterior pedicle screw fusion from Th8–Th10, and L1–L3. Postoperatively, the patient’s pain resolved, and she was left with residual lower extremities dysesthesias; 6-months later, she could walk without assistance.

Conclusion:
Spondylodiscitis caused by M. mucogenicum is rare, and the medical and surgical treatment is comparable to that for other NTM groups.
Modern view on management tactics of patients with endometrial pathologic processes in the perimenopausal period and postmenopause
Authors: Pestrikova Tatyana Yu. , Yurasova Elena A. , Kovaleva Tamara D. , Yurasov Igor V. , Knyazeva T. P. , Adamyan Evgeniya S. , Mashtagova Khava M. 
Published: September 2020

Aim. To evaluate the effectiveness of combination therapy of pathological endometrial processes (benign forms) based on the determination of one of the variants of etiopathogenesis in patients of perimenopausal age and in postmenopause.
Materials and methods. The examination included 34 patients with a diagnosis of endometrial hyperplastic processes in the perimenopausal period and in postmenopause. To verify the diagnosis, in addition to the results of a clinical examination (general and gynecological), the patients underwent laboratory (microscopic and molecular biological studies using real-time polymerase chain reaction method of aspirates from the uterine cavity), diagnostic (ultrasound, hysteroscopy) and morphological examination methods.
Results. Conducting combined antimicrobial and antiviral therapy in patients with endometrial hyperplastic processes, endometrial polyp in combination with chronic endometritis, allowed us to eliminate microbial agents from the endometrium before starting hormonal therapy. A positive result of the combined approach showed the absence of recurrence of endometrial hyperplastic processes within 2 years in 91.17% of patients of the examination group.
Conclusion. In our study, we evaluated the effectiveness of the combined treatment of endometrial hyperplastic processes in patients in the perimenopausal period and in postmenopause, against a background of chronic endometritis, using systemic therapy with antibiotics and antiviral drugs, followed by hormonal therapy. A clinical study allows us to formulate recommendations on the management of patients with endometrial hyperplastic processes, endometrial polyps in the perimenopausal period and in postmenopause.
Clinical and morphological stages vulvar lichen sclerosus
Authors: Sokolova Anastasiia V. , Apolikhina Inna A. , Zaitsev Nikon V. , Chernukha Lyudmila V. 
Published: September 2020

Aim. To determine leading clinical symptoms and risk factors for vulvar lichen sclerosus, identify the features of the disease in the early and late stages.
Materials and methods. The investigation included 102 patients with clinical symptoms of aged 21 to 79 (the average age was 50.613.9 years), 67/102 (66%) conducted punch biopsy of vulva. The control group consisted of 113 women without signs of vulvar lichen sclerosus according to clinical and anamnestic examination.
Results and discussion. Patients with VLS are overweight and obese 1 degree (BMI26.5), have a low level of physical activity and sleep durations 6.6 hours, experience stress and have low resistance to stress factors. Among gynecological diseases, patients with VLS are more often diagnosed with uterine myoma (42%) and genital endometriosis (29%). Among extragenital diseases, endocrine (54%), autoimmune (53%), diseases of the urinary system (54%) and skin (14%), benign diseases of the mammary glands (28.57%) prevail. Scars and deformations of the perineum, as a risk factor for VLS, are observed in 46.21%; 5% of women have a family history of VLS.
Conclusion. Vulvar lichen sclerosus is a multifactorial disease, divided into early and late stages. Subjective symptoms of VLS are not related to age, duration and stage of the disease. Mucosal edema is characteristic of the early stage, and the late stage is characterized by a change in the architectonics of the external genitalia. In 11% of cases, the disease has no visual changes on the vulva. In this case, the most exact diagnostic method for VLS is a histological examination, which allows doctors to establish a diagnosis even at an early stage.