Phacomatosis pigmentokeratotica: a case of HRAS mosaicism causing rhabdomyosarcoma

Abstract

A 17-year-old male presented with a large sebaceous naevus (SN) comprising part of his left face and scalp and a speckled lentiginous naevus (SLN) on his right trunk, hip, neck and scalp with a checkerboard pattern. His right oral hemimucosa showed extensive papillomatous lesions which were contiguous to the upper lip SN lesions. As for extracutaneous manifestations he suffered from cardiac, musculoskeletal and ocular alterations. On the other hand, he developed two primary rhabdomyosarcomas. DNA samples of the SN, SLN, the oral papillomatous hyperplasia and both rhabdomyosarcomas were analyzed by Sanger sequencing. A HRAS c.37G>C mutation was detected in all of them. Skin and blood DNA resulted wild type. PPK is characterized by the association of a sebaceous naevus with a papular naevus spilus and extracutaneous manifestations. Until not long ago, the etiopathogenetical hypothesis of didymosis was accepted. However, in 2013 Groesser et al. proved the existence of an activating HRAS mutation as the cause of this syndrome. A higher incidence of cancer has been observed in germline RASophaties. Furthermore, up to 30% of human cancers show dysregulation of the Ras-Raf-MEK-ERK pathways. In our patient, a HRAS mosaic mutation explains not only the cutaneous but also the extracutaneous manifestations. To our knowledge this is the first described case of PPK in which the existence of a HRAS mosaic mutation is the confirmed cause of rhabdomyosarcoma. Besides the HRAS 37G>C mutation has never been related to any type of rhabdomyosarcoma. Mosaicisms could be underdiagnosed causes of childhood tumours. As dermatologists we stand on a privileged position for the detection of these alterations.

This article is protected by copyright. All rights reserved.

http://ift.tt/2Ek1YIx

Patient, Surgeon, and Anesthesiologist Satisfaction: Who has the Priority?

No abstract available

http://ift.tt/2sos0F1

Acquired Central Hypoventilation Syndrome Unmasked by Propofol Sedation

No abstract available

http://ift.tt/2Ezh3Fl

Neurogenic Pulmonary Edema and Stunned Myocardium in a Patient With Meningioma: A Heart-Brain Cross Talk

No abstract available

http://ift.tt/2sqb0hC

Does needle biopsy cause an increased risk of extracapsular extension in the diagnosis of metastatic lymph node in melanoma?

Abstract

Background

Needle biopsy is a rapid, reliable, and reproducible procedure for histological confirmation of metastatic melanoma localization. Nonetheless, this procedure presents a theoretical risk of a mechanical weakening of the lymph node capsule with perinodal tumor seeding. The objective of the study was to evaluate the incidence of extracapsular extension after needle biopsy in comparison with surgical adenectomy in patients suspected of metastatic lymph node of melanoma.

Methods

We conducted a retrospective study of 1056 patients who underwent lymphadenectomy for melanoma between 2000 and 2016 in our unit. Sixty-nine patients were clinically and/or radiologically suspected of metastatic lymph node of melanoma. Patients were divided according to external lymph node biopsy or surgical adenectomy before lymphadenectomy. The primary endpoint was the histopathological identification of extracapsular extension in analyzed lymph nodes.

Results

The two populations were comparable except for the mitotic index, which was more frequently > 1/mm² in the group with surgical adenectomy (P = 0.005). The proportion of extracapsular extension was significantly greater in the needle biopsy group (28/37) than in patients who underwent surgical adenectomy (14/32) (P = 0.0067; OR = 4 [95% CI: 1.4–11]).

Conclusion

Our results suggest an increased risk of extracapsular extension after external lymph node biopsy in cases of suspicion of metastatic lymph node of melanoma. Thus, this encourages us to prefer surgical adenectomy in patients with suspected adenopathy accessible surgically. In other cases, needle biopsy should be carried out under radiological guidance using devices limiting tumor seeding.

http://ift.tt/2EYnb8c

Issue Information - TOC

http://ift.tt/2ElivYL

Erratum

http://ift.tt/2EYn9NC

Colchicine in dermatology: A review

Abstract

Colchicine is an anti-inflammatory agent that has been used for decades for the treatment of various diseases including gout, familial Mediterranean fever and pericarditis and in recent years for dermatological indications including chronic urticaria, cutaneous vasculitis and psoriasis. Despite its efficacy in various cutaneous diseases, the use of colchicine may be limited by concerns over its side-effects and the potential for toxicity. This article reviews the current literature on the pharmacology of colchicine and its clinical applications in dermatology.

http://ift.tt/2nSAMWX

Dystrophic calcinosis cutis within burns, successfully treated with excision and secondary intention wound healing

http://ift.tt/2sl5Fby

Is spinal anaesthesia in young infants really safer and better than general anaesthesia?

Purpose of review Concerns regarding the potential neurotoxic effects of general anaesthesia have seen resurgence in awake spinal anaesthesia in neonates and infants. This review includes recently published data from a large prospective randomized controlled trial with view to determining if spinal anaesthesia is safer and better than general anaesthesia in this population. Recent findings Compared with general anaesthesia, spinal anaesthesia results in less haemodynamic instability and fewer early (

http://ift.tt/2Ewhe4v

Acute pain management in children: challenges and recent improvements

Purpose of review The evidence regarding the efficacy of analgesics available to guide postoperative pain treatment in pediatric patients is limited. Opioid medications are very often an important component of pediatric postoperative pain treatment but have been associated with perioperative complications. We will focus on initiatives aiming to provide effective treatment minimizing the use of opioids and preventing the long-term consequences of pain. Recent findings Interpatient variability in postoperative pain is currently managed by applying protocols or by trial and error, thus often leading to patients being either undertreated or overtreated. Few evidence-based reports are available to guide the use of opioid medications in children, including the prescription of opioids after hospital discharge. Using combinations of nonopioid analgesics in a multimodal approach may limit the need for opioids, thus decreasing the risk of toxicity and dose-related side effects. There is a lack of adequate research in this field, and more specifically on identifying which patient is at higher risk of poor postoperative pain management. Summary Treatment options have evolved in recent years, including the combinations of multimodal regimens and regional anesthetic techniques. Using combinations of nonopioid analgesics in a multimodal approach may limit the need for opioids. Correspondence to Pablo M. Ingelmo, MD, Department of Anesthesia, Montreal Children's Hospital, B.04.2427, 1001 Boulevard Decarie, Montreal, QC, Canada H4A3J1. Tel: +1 514 412 4448; e-mail: pablo.ingelmo@mcgill.ca Copyright © 2018 YEAR Wolters Kluwer Health, Inc. All rights reserved.

http://ift.tt/2sru278

Intensive care practitioner: I forgot half the things I learned and the other half seems to be all wrong!

No abstract available

http://ift.tt/2spwX0d

Long-term neurocognitive outcomes following surgery and anaesthesia in early life

Purpose of review Repeated controversial and alarming statements of the potential dangers of anaesthetic agents on neurological outcomes in children continue to be issued based primarily on preclinical studies. This review assesses the current evidence of laboratory and clinical data and identifies areas of concerns. Recent findings Published animal and laboratory data consistently indicate that prolonged and excessive use of anaesthetic agents can lead to morphological changes and neurocognitive impairment in animals without a clear cut-off age or a superiority of one technique over another. Retrospective human studies and prospective clinical trials indicate that short exposures to anaesthesia and surgery are safe and have no effect on long-term neurological outcomes. Small and consistent continuing improvements in the perioperative period (aggregation of marginal gains) will impact on long-term neurological morbidity in humans. Summary It is biologically plausible that anaesthetic agents may induce structural changes during mammalian brain development and beyond. However, in the absence of alternatives the impact of the choice of anaesthetic drugs on long-term neurocognitive outcomes is almost certainly to be of limited relevance in humans. The underlying disease processes, surgical intervention, and trauma as well as other known perioperative factors more significantly affect these outcomes. Correspondence to Tom G. Hansen, MD, PhD, Department of Anaesthesia & Intensive Care – Paediatric Section, Odense University Hospital, 5000 Odense C, Denmark. Tel: +45 6541 3812; fax: +45 6611 3415; e-mail: tomghansen@dadlnet.dk Copyright © 2018 YEAR Wolters Kluwer Health, Inc. All rights reserved.

http://ift.tt/2Ew76J5

Non-enhanced MRI in combination with color Doppler flow imaging for improving diagnostic accuracy of parotid gland lesions

Abstract

Purpose

To determine the value of non-enhanced MRI in combination with color Doppler flow imaging (CDFI) for differentiating malignant parotid tumors from benign ones.

Methods

This retrospective study analyzed 51 parotid gland lesions (39 benign and 12 malignant) in 51 patients who underwent preoperative CDFI as well as non-enhanced MRI including T₁-weighted, T₂-weighted, and diffusion-weighted imaging (DWI). Degrees of intratumor vascularity were categorized into four grades basing on CDFI findings. The relationships between the lesion and its adjacent external carotid artery and retromandibular vein were inspected on T₁-weighted and T₂-weighted images. Apparent diffusion coefficient (ADC) values were calculated from diffusion-weighted images, and were used to classify the parotid gland lesions with and without reference to the CDFI findings. The classification results were compared using the McNemar test. Sensitivity, specificity, and accuracy percentages were calculated when the non-enhanced MRI/CDFI findings were used to differentiate benign lesions from malignant ones.

Results

The diagnostic accuracy (96.1 vs 82.4%) was significantly improved when ADCs were used together with CDFI findings for classifying parotid gland lesions compared to when ADCs were used alone. Pleomorphic adenomas had the highest ADCs. The ADC thresholds were 1.425 × 10⁻³ mm²/s for differentiating pleomorphic adenomas from carcinomas, 0.999 × 10⁻³ mm²/s for differentiating pleomorphic adenomas from other benign lesions, and 0.590 × 10⁻³ mm²/s for differentiating benign lesions other than pleomorphic adenomas from lymphomas.

Conclusion

Combining CDFI with non-enhanced MRI can improve the diagnostic accuracy of MRI for classifying parotid gland lesions.

http://ift.tt/2EipIZn

Dexmedetomidine as a part of general anaesthesia for caesarean delivery in patients with pre-eclampsia: Efficacy and foetal outcome a randomised double-blinded trial

BACKGROUND During general anaesthesia, endotracheal intubation of patients with pre-eclampsia causes stimulation of the sympathetic nervous system and catecholamine release, which may lead to maternal and neonatal complications. OBJECTIVE To attenuate both the stress response and the haemodynamic response to tracheal intubation in patients with pre-eclampsia. DESIGN A randomised, double-blind, controlled study. SETTING Single University Hospital. PATIENTS Sixty patients aged 18 to 45 years with pre-eclampsia receiving general anaesthesia for caesarean section. INTERVENTIONS The patients were randomly allocated to three groups. Groups D1and D2 received an infusion of dexmedetomidine 1 μg kg−1 over the 10 min before induction of general anaesthesia, then 0.4 and 0.6 μg kg−1 h−1 dexmedetomidine, respectively. Group C received equivalent volumes of 0.9% saline. MAIN OUTCOME MEASURES The primary outcome was the effect of dexmedetomidine on mean arterial blood pressure measured before induction of general anaesthesia at 1 and 5 min after intubation, and then every 5 min until 10 min after extubation. The secondary outcomes were blood glucose and serum cortisol (measured before induction of general anaesthesia, and at 1 and 5 min after intubation), postoperative visual analogue pain scores, time to first request for analgesia, the total consumption of analgesia, Ramsay sedation score, maternal and placental vein blood serum levels of dexmedetomidine and neonatal Apgar score at 1 and 5 min. RESULTS At all assessment times, the mean arterial pressures were significantly lower in the dexmedetomidine groups than in the control group. Compared with group C, the heart rate was significantly lower in both groups D1 and D2. In group D2, the heart rate was lower than in group D1. Serum glucose and cortisol were significantly higher in the controls than in either group D1 or D2. Group D2 patients were significantly more sedated on arrival in the recovery room followed by D1. Time to first analgesia was significantly longer in groups D2 and D1 than in group C, and the visual analogue pain scores were significantly lower in groups D1 and D2 than in group C at 1, 2, 3 and 5 h. Total morphine consumption was significantly lower in groups D1 and D2 than in the control group. There was no difference in Apgar scores across the three groups despite significantly higher dexmedetomidine concentrations in group D2 (both maternal and placental vein) than in group D1. CONCLUSION Administration of dexmedetomidine in doses 0.4 and 0.6 μg kg−1 h−1 was associated with haemodynamic and hormonal stability, without causing significant adverse neonatal outcome. TRIAL REGISTRATION Pan African Clinical Trial Registry (PACTR201706002303170), (www.pactr.org). Correspondence to Ashraf M. Eskandr, MD, Department of Anesthesia, ICU and Pain Therapy, Faculty of Medicine, Menoufiya University, 3 Yassin Abd-Elghafar Street, Shibin El-koom, Egypt E-mail: ameskandr@yahoo.com © 2018 European Society of Anaesthesiology

http://ift.tt/2EjETBM

Low anaesthetic waste gas concentrations in postanaesthesia care unit: A prospective observational study

BACKGROUND Volatile anaesthetics are a potential hazard during occupational exposure, pregnancy or in individuals with existing disposition to malignant hyperthermia. Anaesthetic waste gas concentration in postanaesthesia care units (PACU) has rarely been investigated. OBJECTIVE(S) The current study aims to assess concentrations of volatile anaesthetics in relation to room size, number of patients and ventilator settings in different PACUs. DESIGN A prospective observational study. SETTING Two different PACUs of the Hannover Medical School (Hannover, Germany) were evaluated in this study. The rooms differed in dimensions, patient numbers and room ventilation settings. PATIENTS During the observation period, sevoflurane anaesthesia was performed in 65 of 140 patients monitored in postanaesthesia unit one and in 42 of 70 patients monitored in postanaesthesia unit two. MAIN OUTCOME MEASURES Absolute trace gas room concentrations of sevoflurane measured with a compact, closed gas loop high-resolution ion mobility spectrometer. RESULTS Traces of sevoflurane could be detected in 805 out of 970 samples. Maximum concentrations were 0.96 ± 0.20 ppm in postanaesthesia unit one, 0.82 ± 0.07 ppm in postanaesthesia unit two. Median concentration was 0.12 (0.34) ppm in postanaesthesia unit one and 0.11 (0.28) ppm in postanaesthesia unit two. CONCLUSION Low trace amounts of sevoflurane were detected in both PACUs equipped with controlled air exchange systems. Occupational exposure limits were not exceeded. Correspondence to Dr. Sebastian Heiderich, MD, Clinic of Anaesthesiology and Intensive Care Medicine, Hannover Medical School, Carl-Neuberg-Str. 1, 30625 Hannover, Germany E-mail: heiderich.sebastian@mh-hannover.de © 2018 European Society of Anaesthesiology

http://ift.tt/2EY3yx2

Low perfusion pressure is associated with renal tubular injury in infants undergoing cardiac surgery with cardiopulmonary bypass: A secondary analysis of an observational study

BACKGROUND Earlier work on adults undergoing surgery with cardiopulmonary bypass suggests that there is a close relationship between the lower limit of the cerebral and renal autoregulation pressures. Although cerebral autoregulation during bypass in infants has been extensively investigated, the impact of bypass on kidney function is not well known. It is, nevertheless, acknowledged that the main pathophysiological process involved in cardiac surgery-related kidney damage is tubular injury, and that urine neutrophil gelatinase-associated lipocaline (uNGAL) is a reliable biomarker of injury. OBJECTIVE To identify the most predictive bypass variable for the measurement of renal injury, its threshold value and the most predictive time below that threshold. DESIGN Observational study linking electronically recorded bypass perfusion pressure and oxygen delivery rate with intra-operative uNGAL excretion. Variations in bypass variables were accounted for by their excursions below several thresholds. SETTING French tertiary referral paediatric cardiac centre. PATIENTS A total of 72 infants in whom uNGAL was measured within 1 h of bypass. INTERVENTIONS None. MAIN OUTCOME MEASURE Renal injury, identified by a high creatinine normalised uNGAL concentration (>21.2 μg mmol−1). RESULTS At the end of bypass, 43.75% of infants had high uNGAL. A more than 40% pressure drop below the normal age-standardised mean arterial pressure was associated with high uNGAL. Receiver operating curve [interquartile range] areas were 0.626 [0.501 to 0.752] for a more than 40% drop, and 0.679 [0.555 to 0.804] for a more than 50% drop. A more than 40% pressure drop for 19.5 min provided a 0.65 negative predictive value for high uNGAL, and a more than 50% pressure drop for 5.4 min provided a 0.67 negative predictive value. The link between uNGAL and oxygen delivery rate was negligible. CONCLUSION Maintaining the perfusion pressure above 60% of the normal age-standardised mean arterial pressure may provide an effective renal protective strategy. TRIAL REGISTRATION Registered on October 11, 2010, ClinicalTrials.gov Identifier: NCT01219998. Correspondence to Mirela Bojan, MD, PhD, Department of Anaesthesiology and Critical Care, Necker-Enfants Malades University Hospital, Assistance Publique – Hôpitaux de Paris, 149, rue de Sèvres, 75743 Paris, France Tel: +33 171396663; e-mail: mirela.bojan@nck.aphp.fr © 2018 European Society of Anaesthesiology

http://ift.tt/2Ejad3I

Ethosuximide-induced Stevens–Johnson syndrome: Beneficial effect of early intervention with high-dose corticosteroid therapy

Abstract

We report two rare cases of childhood epilepsy patients who developed ethosuximide-induced Stevens–Johnson syndrome (SJS). Unlike typical SJS, the initial eruption of both patients presented well-demarcated, infiltrating firm papules mainly on the cheeks and the extensor aspects of the arms (case 1), and multiple vesicles on the soles and oral aphthosis (case 2), which closely mimicked viral exanthema. We diagnosed both patients with ethosuximide-induced SJS, based on the dosing period and the positive results of drug-induced lymphocyte stimulation test. Systemic corticosteroids are usually selected as a standard therapy for SJS, despite controversial results regarding their effectiveness. In case 1, an i.v. pulse therapy of methylprednisolone (30 mg/kg, 3 days consecutively) was initiated on day 7 from the onset of illness, and an i.v. immunoglobulin (400 mg/kg, 5 days consecutively) was added the following day. In case 2, an i.v. prednisone treatment (1 mg/kg, for 1 week) was initiated on day 4 from the onset. Eventually, the early therapeutic interventions resulted in good outcomes in both patients.

http://ift.tt/2Cd5y1e

Case of deep vein thrombosis in a patient with advanced malignant melanoma treated with dabrafenib and trametinib

http://ift.tt/2Ej2XJ0

Immunocryosurgery as monotherapy for lentigo maligna or combined with surgical excision for lentigo maligna melanoma

Abstract

The incidence of lentigo maligna (LM), in situ (LM) or invasive (lentigo maligna melanoma, LMM), has increased during the last decades. Due to functional or cosmetic outcomes, optimal treatment with surgical excision may not be appropriate in some cases. We tried less invasive therapy, immunocryosurgery, as a single treatment for LM or combined with surgery for LMM, with better aesthetic results. Three patients with LM or LMM not amenable to complete surgical excision were selected. LMM patients underwent limited surgical resection of the invasive area. Subsequently, a combined treatment with topical imiquimod and cryosurgery was performed. The LM patient received immunocryosurgery directly. All of them were free of local and systemic disease at 48, 42 and 41 months after discontinuation of therapy. We consider that immunocryosurgery is an alternative option for LM or even for LMM (after removal of the invasive tissue with narrow margins) in poor surgical candidates, with good therapeutic, functional and cosmetic results.

http://ift.tt/2CfeqDu

Benign cephalic histiocytosis: A case with infiltration of CD1a-positive langerin-negative cells

http://ift.tt/2EhYmqB

Cutaneous infiltration of plasmacytoid dendritic cells and t regulatory cells in skin lesions of polymorphic light eruption

Abstract

Background

Polymorphic light eruption (PLE) is the most common autoimmune photodermatosis. Plasmacytoid dendritic cells (PDCs) are important mediators of innate anti-microbial immunity involved in the pathogenesis of many inflammatory skin diseases. In addition to PDCs, regulatory T cells (Tregs) are involved in controlling inflammation and adaptative immunity in skin by their immunosoppressive capacity.

Objective

The aim of the present study was to investigate the presence of PDCs and Tregs in photoexposed skin from PLE compared to healthy skin.

Methods

Patients with PLE diagnosis and healthy controls were recruited and underwent a photoprovocative test. A 4- mm punch biopsy was taken from the site of positive photoprovocation test reaction and immunohistochemistry for BDCA2 as marker for PDCs, CD4 and FOXP3 as markers for Tregs was performed. Double immunostain for FOXP3 and CD4 was performed as well.

Absolute counts for CD4, BDCA2 and FOXP3 were performed in at least 5 High Power Fields (HPF). Percentage of CD4, BDCA2 and CD4FOXP3 positive cells over the total inflammatory infiltrate was assessed for each case.

Results

We enrolled 23 patients and controls. BDCA2+ cells were present in 91.3% of PLE skin samples, and 100% of healthy volunteer. Both in PLE patients and healthy controls, PDCs distribution was mainly dermic (p<0.05).

Compared to healthy controls, both epidermic and dermic BDCA2+ cells count was significantly higher in PLE patients (p<0.05).

Both in PLE patients and healthy controls, Tregs distribution was mainly dermic (p<0.05).

The presence of both CD4+ cells and FOXP3+ cells was significantly higher in the dermis of PLE patients compared to controls (p<0.05). Relative percentages of cellular infiltrations confirmed these results.

Conclusions

D-PDCS and Tregs may play a significant role in the development of PLE and dermal distribution of PDCs in PLE skin biopsies seems to confirm a possible overlap with Cutaneous Lupus Erythematosus (CLE).

This article is protected by copyright. All rights reserved.

http://ift.tt/2CcNXqn

Focal facial hyperhidrosis associated with internal carotid artery dissection successfully treated with botulinum toxin A

Abstract

Hyperhidrosis affects up to 3% of the population⁽¹⁾. Secondary hyperhidrosis may be associated with infections, malignancies, endocrine or neurological diseases⁽²⁾. We report the first case of localized hyperhidrosis associated to Pourfour du Petit syndrome secondary to internal carotid artery dissection successfully and safely treated with botulinum toxin.

This article is protected by copyright. All rights reserved.

http://ift.tt/2EkkUH0

The relationship between clinical characteristics including presence of exposed lesions and health-related quality of life (HRQoL) in patients with psoriasis: Analysis from the nationwide epidemiologic study for psoriasis in Korea (EPI-PSODE study)

Abstract

Background

Psychological aspect and quality of life should be considered in treating patients with psoriasis.

Objective

We sought to ascertain which clinical characteristics including presence of exposed lesions are associated with impairment of health-related quality of life (HRQoL) in patients with psoriasis.

Methods

The EPI-PSODE study was a nationwide, multicenter, cross-sectional study conducted in Korea that included 1,260 adult patients with psoriasis. In addition to clinical characteristics including presence of exposed lesions, data were collected using the psoriatic arthritis (PsA) screening and evaluation (PASE), dermatology life quality index (DLQI), MOS 36-item short-form health survey (SF-36), work productivity and activity impairment questionnaire psoriasis (WPAI: PSO), and medication satisfaction questionnaire (MSQ).

Results

Patients with a DLQI score>5 (n = 990) were younger, had an earlier onset of psoriasis, scored higher on the Psoriasis Area and Severity Index (PASI), had higher body surface area (BSA), and had higher PASE scores than patients with DLQI≤5 (n = 266). The group of patients with exposed lesions (n=871) were younger and male predominance, earlier onset of psoriasis, longer disease duration, higher PASI/BSA score and a higher proportion with drinking and smoking history each than the group of patients without exposed lesions (n=389). Presence of exposed lesions negatively influenced DLQI, 36-Item Short-Form Health Survey (SF-36) (mental component), presenteeism, total work productivity impairment, and total activity impairment in the WPAI: PSO. In multiple regression model, PASI score was the only variable which was significantly associated with all HRQoL measures. Presence of exposed lesions was a significant factor affecting DLQI and SF-36 (mental).

Conclusion

The presence of exposed lesions has a negative impact on quality of life, mental health, and work productivity. Therefore, effective treatments are particularly needed for psoriasis patients with exposed lesions.

This article is protected by copyright. All rights reserved.

http://ift.tt/2CcNWTl

Dermoscopic Features of Tungiasis

Abstract

Tungiasis is a parasitic infection caused by the sand flea Tunga penetrans. Tungiasis is prevalent in rural Central and South America, the Caribbean and sub-Saharan Africa.¹ In some endemic areas, the overall infestation prevalence exceeds 50 percent, and it remains an important public health problem for poor communities.^1,2 In some cases, the eruptions can mimic viral warts, furuncles, abscesses and paronychia.¹ Dermoscopy is reported to be a useful tool for differentiating between these and tungiasis lesions.^3,4 Moreover, the majority of cases have multiple lesions, with the ectoparasites causing more than 50 lesions in a single individual in some cases,⁵ and dermoscopy can help find the lesions. Despite the clinical utility of dermoscopy, no reports have assessed the significance of each dermoscopic finding in tungiasis. Here we report a case of tungiasis and we review the dermoscopic findings of 11 lesions of the patient to reveal the characteristic findings.

This article is protected by copyright. All rights reserved.

http://ift.tt/2EhTt0O

Psoriatic arthritis screening by the dermatologist: development and first validation of the “PURE-4 scale”

Abstract

Background

Dermatologists are recommended to ask psoriasis patients about musculoskeletal complaints to allow early detection and treatment of psoriatic arthritis (PsA). Screening tools have been developed to help identifying patients warranting further rheumatologic assessment, but evidence suggests room for improvement in their diagnostic value and ease of use for outpatient practice.

Objective

To develop and internally validate a brief tool for dermatologists to screen patients to refer to a rheumatologist for PsA diagnosis.

Methods

After literature review, 23 items were selected, covering pain at various locations and inflammatory signs of PsA. The validation study was conducted in medically diagnosed psoriasis patients consecutively recruited between 2012-2014 (Saint Joseph Hospital, Paris, France). Patients were enrolled by a dermatologist who helped to complete the questionnaire. Diagnosis of PsA was established by a rheumatologist based on CASPAR criteria. Multivariate logistic regression models were performed to build the scale, assessing discrimination through sensitivity, specificity and area under the ROC curve (AUC). Final model was internally validated using bootstrapping techniques.

Results

168 patients were recruited, of whom 9 were excluded for known PsA and 21 did not attend the rheumatologist consultation. Out of 137 included patients (median age 43 years, 59.6% men), 21 (15.3%) had a PsA diagnosis. Final regression model retained 4 independent items, including evocative signs of dactylitis, inflammatory heel pain, bilateral buttock pain and peripheral joint pain with swelling in patients aged<50. A total score (the PURE-4) was computed (0-4 points) that demonstrated excellent discriminative power (AUC=87.6%; Sensitivity=85.7% and Specificity=83.6% at the threshold of ≥1/4 points), with no evidence for over-optimism in bootstrapped internal validation.

Conclusion

These findings demonstrate the good diagnostic properties of a new screening scale using only 4 easy-to-collect items. If confirmed in other populations, it may prove useful in outpatient dermatology clinics for triage of psoriasis patients requiring further assessment by the rheumatologist.

This article is protected by copyright. All rights reserved.

http://ift.tt/2CcNUuH

Patients affected by Dent disease 2 could be predisposed to hidradenitis suppurativa

Abstract

Dent disease 2 (DD2) is a rare X-Linked disorder characterized by proximal tubule dysfunction. It is considered as mild variant of Lowe Syndrome (LS) and both conditions are secondary to OCRL1 gene mutations.¹ Mutations in this gene drastically reduce (<10%) inositol polyphosphate 5-phosphatase (OCRL1) activity.¹

This article is protected by copyright. All rights reserved.

http://ift.tt/2El4i28

Rapid Improvement of Psoriasis in a Patient with Lung Cancer after Treatment with Erlotinib

Abstract

We report the case of a 48-year-old woman with a rapid improvement of her psoriasis after treatment with erlotinib.A metastasised non-small cell lung cancer (NSCLC) was diagnosed and the psoriasis treatment with ustekinumab was stopped which caused a flare up of her psoriasis.

This article is protected by copyright. All rights reserved.

http://ift.tt/2CcNTa7

Hard onset therapy for functional hypoadduction and presbylaryngis: our experience in 22 patients

Abstract

Objectives

The hard onset, also known as a hard attack, or glottal onset, is a technique of vocal production in which the vocal folds are adducted prior to exhalation and forced open to begin phonation. In this study, we assessed the short-term efficacy of voice therapy incorporating hard onset techniques for patients with functional hypoadduction and presbyphonia. Design: Prospective cohort study. Setting: Tertiary academic medical center. Participants: Twenty-two patients with functional hypoadduction who underwent voice therapy, including hard-onset therapy, for 3 months. Main outcome measures: Vocal function pre-therapy and 3 months post-therapy assessed objectively by quantitative aerodynamic and acoustic analysis and subjectively by vocal handicap index (VHI) and grade, roughness, breathiness, asthenia, strain (GRBAS) scale. Results: Voice quality as graded by the VHI and GRBAS were statistically significantly improved following voice therapy. Objective comparisons reflected improvement in fundamental frequency, jitter, shimmer, normalized noise energy, and maximal phonation time. Conclusions: Voice therapy, including hard onset therapy, improved vocal quality and performance by both subjective and objective measures in patients with functional hypoadduction/presbyphonia.

This article is protected by copyright. All rights reserved.

http://ift.tt/2Eiw3Eh

Tongue exercise and ageing effects on morphological and biochemical properties of the posterior digastric and temporalis muscles in a Fischer 344 Brown Norway rat model

Publication date: May 2018
Source:Archives of Oral Biology, Volume 89
Author(s): Brittany N. Krekeler, Glen Leverson, Nadine P. Connor
ObjectiveThis study sought to examin effects of age and tongue exercise on the posterior digastric (opener) and the temporalis (closer). We hypothesized 1) age would result in differing morphological (cross sectional area) and biochemical (myosin heavy chain isoform) components of these muscles; 2) tongue exercise would result in coactivation of these muscles inducing a decrease in age-related differences between age groups.DesignYoung adult (9 months) and old (32 months) Fischer 344 Brown Norway rats were randomized into a tongue exercise or control group. Post-training, posterior digastric and temporalis muscles were harvested and analyzed using: 1) Sodium Dodecyl Sulfate-Polyacrylamide Gel Electrophoresis (SDS-PAGE) to assess percent myosin heavy chain (MyHC) content; 2) Immunohistochemical staining to determine cross sectional area (CSA).ResultsA larger proportion of slowly contracting MyHC isoforms in the posterior digastric and temporalis muscles were found in old. No significant main effects for age or exercise in fiber size were found in posterior digastric muscle. An interaction between age and exercise for temporalis cross sectional area indicated the old exercise group had smaller average cross sectional area than all other groups.Conclusions findingssuggest that: 1) Increasing age induces biochemical changes in muscles of the jaw, specifically showing an increase the proportion of slower contracting MyHC isoforms; 2) Increasing age and tongue exercise induce a reduction in muscle fiber cross sectional area in the temporalis muscle only. However, continued study of these cranial muscle systems is warranted to better understand these changes that occur with age and exercise.



http://ift.tt/2Ee4qfx

No Relation between Psoriasis and Renal Abnormalities: A Case-Control Study

Multiple observational studies have demonstrated that psoriasis is associated with nephropathy; however, the renal involvement in psoriasis remains largely a matter of debate. The current study was designed to investigate if psoriatic patients are at increased risk of renal abnormalities, in absence of any other comorbidities. Forty patients (11 women, 29 men, mean age 44.9 ± 15.45 years) with moderate to severe chronic plaque type psoriasis who were not under systemic therapy and 40 age- and gender-matched control subjects were enrolled in the study. Patients and controls with history of diabetes, hypertension, and chronic renal disease were excluded. Urinalysis by dipstick and microscopic evaluation and 24 h proteinuria and albuminuria were measured in all patients and controls. Patients with psoriasis and controls were not significantly different with respect to the prevalence of abnormal urinalysis (7.5% versus 5%, P = 1.0), mean 24 h proteinuria (70.40 ± 24.38 mg/24 h versus 89.40 ± 26.78 mg/24 h, P = 0.30), and albuminuria (14.15 ± 8.12 mg/24 h versus 16.62 ± 8.21 mg/24 h, P = 0.18). The presence of abnormal urinalysis was not more common in patients with psoriasis than in controls. Our study demonstrated that psoriatic patients without any other comorbidities are not at increased risk of kidney disease.

http://ift.tt/2CcoInV

Apple, condom, and cocaine – body stuffing in prison: a case report

Drug dealers and drug users resort to body stuffing to hastily conceal illicit drugs by ingesting their drug packets. This practice represents a medical challenge because rupture of the often insecure packagin...

http://ift.tt/2EhGCvf