Pediatric Dermatology, EarlyView.
Medicine by Alexandros G. Sfakianakis,Anapafseos 5 Agios Nikolaos 72100 Crete Greece,00306932607174,00302841026182,alsfakia@gmail.com
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Παρασκευή 6 Απριλίου 2018
Compound heterozygous mutations in desmoplakin associated with skin fragility, follicular hyperkeratosis, alopecia, and nail dystrophy
Pediatric Dermatology, EarlyView.
Better kitchens and toilets: both needed for better health
Abstract
Both poor water, sanitation, hygiene (WaSH) and household air pollution (HAP) adversely affect the health of millions of people each year around the globe and specifically in developing countries. The objective of current work is to highlight the importance of HAP in parallel to WaSH for decision making to achieve better health specially in developing countries. There are examples, where developing countries are strengthening efforts to tackle the issue of poor water and sanitation such as 'Clean India Mission' was recently launched by the Government of India. However, there is lack of actions to address the issue related to HAP—to extend the coverage of clean fuel, efficient stoves and ventilated kitchens to the deprived population under the 'Clean India Mission'. Most of the rural household and urban slums in developing countries have only a single room, where people cook and sleep. This leads them to exposure to toxic HAP, which can be minimized by developing country specific indoor air quality guidelines and action framework. Hence, there should be policies to provide them not only the subsidy for clean fuel but also to build properly ventilated kitchens along with the promotion of clean toilets and water supplies. There is a need to strengthen global efforts, to jointly address the challenges associated with the risks related to WaSH and HAP in order to efficiently reduce the global burden of disease. Further, this will also help to timely attain the sustainable development goals for better health and environment.
Increased associated effects of topography and litter and soil nutrients on soil enzyme activities and microbial biomass along vegetation successions in karst ecosystem, southwestern China
Abstract
Studying the influence of topography and litter and soil nutrients on soil enzymes and microbial biomass is important to the understanding of soil nutrient transformation and cycling, but these relationships in heterogeneous soils of karst ecosystem remains poorly understood. We determined environment factors influencing the urease (URS) and alkaline phosphatase (ALP) activity and microbial biomass C and N (MBC and MBN) with advancing vegetation succession. The results showed that ALP increased but URS decreased with the advancing vegetation succession. The MBC and MBN were highest in shrubland, but both were lowest in grassland. The URS was positively correlated with the surface cover of rock outcrops (SRO) but negatively correlated with litter N, and soil available N and pH. Conversely, ALP was positively correlated with litter N, soil organic carbon (SOC), and soil available N and pH, but negatively correlated with soil total N. The MBC was positively related to litter quantities and SOC but negatively related to soil pH; the MBN was positively related to slope gradient (SLG), SOC, and soil total P and available P. Additionally, the trends of the index URS/MBN were grassland > secondary forest > shrubland > primary forest, but the index ALP/MBN increased with advancing vegetation succession. It indicated that soil microorganism mainly exudate extracellular URS and ALP to soils. We also found the interactions of topography (SLG and SRO), litter (nutrients and quantity), and soil (nutrients and pH) explained 42.00, 87.00, and 66.00% of the variations in URS, ALP, and microbial biomass, respectively. Path analysis showed that the topography had a directly positive effect on litter nutrients and quantities, but not on soil nutrients; the litter nutrients and quantities had direct positive effect on soil nutrients, which had direct effect on soil enzymes and microbial biomass; the relationships (R2) between the independent variable and enzymes activities and microbial biomass increased with advancing successions. Thus, it suggested that high SLG and SRO are good for collecting litters back to soils and then the topography, litter, and soil factors increased its controlling effect on soil enzymes activities and microbial biomass with advancing successions in karst ecosystem.
Techno-economic and environmental assessment of biogas production from banana peel ( Musa paradisiaca ) in a biorefinery concept
Abstract
Two scenarios for the biogas production using Banana Peel as raw material were evaluated. The first scenario involves the stand-alone production of biogas and the second scenario includes the biogas production together with other products under biorefinery concept. In both scenarios, the influence of the production scale on the process economy was assessed and feasibility limits were defined. For this purpose, the mass and energy balances were established using the software Aspen Plus along with kinetic models reported in the literature. The economic and environmental analysis of the process was performed considering Colombian economic conditions. As a result, it was found that different process scales showed great potential for biogas production. Thus, plants with greater capacity have a greater economic benefit than those with lower capacity. However, this benefit leads to high-energy consumption and greater environmental impact.
Assessment of toxicity of heavy metal-contaminated soils toward Collembola in the paddy fields supported by laboratory tests
Abstract
Effects on soil Collembola of Cu, Zn, Pb, and Cd pollution from Cu smelters over 40 years were investigated in paddy fields from an area of Eastern China. We compared the field effects to those observed in single-species laboratory tests employing the hemiedaphic collembolan Folsomia candida and the epedaphic Sinella curviseta obtained from laboratory cultures and exposed to field-collected polluted soil. The results indicated that different collembolan species responded differently to the pollution in the fields and could be divided into sensitive, indifferent, and tolerant types accordingly. The abundance of sensitive species decreased as the pollution increased, but this was not the same for indifferent and tolerant species. The dominant species changed from sensitive to tolerant species as the pollution increased. The reproduction of F. candida and S. curviseta was most sensitive to the contaminated soil compared to growth and survival; the sensitivity of the two species was similar. The growth was more sensitive than the survival for F. candida but not for S. curviseta. The growth and survival of F. candida were much more sensitive than those of S. curviseta. Sensitivity of field populations of F. candida (EC10 31 [15–46]) and hemiedaphic species Folsomia quadrioculata (EC10 52 [0.7–102]) were comparable with sensitivity of the reproduction of F. candida in the single-species tests (EC10 21 [14–27]), suggesting that single-species test based on laboratory cultures and field soil could be used to link laboratory and field data and then reflect the field situation. S. curviseta could be used as an epedaphic species in single-species tests and F. quadrioculata as an indicator species for assessment of field effect.
Vitamin D-Dependent rickets Type II with alopecia: A rare case report
Indian Journal of Dermatology 2018 63(2):176-179
Vitamin D-dependent rickets type II is a rare hereditary disorder. It occurs due to mutations in the gene chr. 12q12-q14, which codes for vitamin D receptor. End-organ resistance to 1,25-(OH)2vitamin D3 and alopecia in severe cases are the characteristic features. We report a case of a 4-year-old boy with loss of hair over the scalp and body – first observed after 1 month of birth. The boy also developed difficulty in walking at the age of 2 year. On analysis, reduced serum calcium level (7.5 mg/dL) and elevated alkaline phosphatase level (625 IU/L) were reported. Initially, the treatment included intramuscularly administered single dose of 600,000 IU vitamin D, followed by 400 IU of vitamin D along with 1 g of supplemental calcium every day. Periodic follow-up was conducted for 2 months. Improvement was observed in the biochemical parameters and X-rays of the distal radial and ulnar metaphyses, although no improvement was observed in alopecia.
Common contact allergens in patients with palmoplantar and scalp psoriasis and impact of their avoidance on dermatology life quality index: A hospital-based study
Indian Journal of Dermatology 2018 63(2):160-164
Background: Scalp psoriasis and psoriasis limited to palms and/or soles have been referred to as difficult to control psoriasis. Contact allergy has long been suspected to aggravate existing lesions and cause resistance to therapy in these psoriasis variants. Objectives: The objective of the study was to assess common contact allergens in patients with palmoplantar and scalp psoriasis and the impact of their avoidance on dermatology life quality index (DLQI). Materials and Methods: A total of 54 patients with palmoplantar and scalp psoriasis were patch tested with Indian Standard Series. The patch test results were read on day 2 and day 4. DLQI was calculated before patch testing and at 1 month and 3 month in patch test positive patients after instructing allergen avoidance. Results: Sixteen out of fifty-four patients (29.62%) showed positive patch test reactions. Metal antigens like nickel and cobalt were the most common sensitizers identified. Statistically significant improvement in DLQI was observed at 1 month and at 3 month of allergen avoidance. Conclusion: Patch testing is a useful test to determine the triggering or aggravating antigens in patients with palmoplantar and scalp psoriasis and subsequent allergen avoidance should be stressed on.
Stamping a case of cutaneous adverse drug reaction: Proving beyond causality assessment
Indian Journal of Dermatology 2018 63(2):99-106
Different types of skin testing with a suspected drug have been reported to be helpful in determining the cause of cutaneous adverse drug reactions (CADRs). It is of utmost importance for practicing dermatologists to have a first hand knowledge of different types of skin tests available in a case of CADR. In each suspected case, a detailed investigation with the suspected agent and correlation of the positive skin reaction with clinical variant of CADR is advocated. Drug skin tests are performed 6 weeks to 6 months after complete healing of the CADR. Drug patch tests are performed similar to the methods used in patch testing in studying contact dermatitis. The commercialized form of the drug used by the patient is tested at 30% dilution. The pure drug is tested at 10% dilution. In severe CADR, drug patch tests should be performed at lower concentrations. It is also of value to test on the most affected site of the initial CADR.
Autoimmune progesterone dermatitis
Indian Journal of Dermatology 2018 63(2):188-190
Unusual and interesting adverse cutaneous drug reactions
Indian Journal of Dermatology 2018 63(2):107-116
Any drug can cause any rash! Cutaneous adverse drug reactions (CADRs) are great mimickers and can be included in the differential diagnosis of any inflammatory dermatoses. Several drugs can cause rash of similar morphology and the same drug can cause rash of different morphology. While some common and specific drug reaction patterns are recognized easily by the clinicians, many a times unusual and interesting patterns can be induced by drug(s), thus leading to erroneous diagnosis and mistreatment. This review aims to familiarize clinicians with some rare, yet interesting patterns of CADR.
Peutz-Jeghers syndrome: A circumventable emergency
Indian Journal of Dermatology 2018 63(2):168-171
Peutz-Jeghers syndrome (PJS) is characterized by multiple hamartomatous polyps in the gastrointestinal tract and mucocutaneous pigmentation. Here we present, a case of multiple gastrointestinal hamartomatous polyps in a 22-year-old male who had been operated for intestinal obstruction due to ileocolic intussusception. Resection of the affected segment was done with proximal ileostomy and distal mucous fistula formation. Clinicopathological diagnosis of PJS was made. Later, during ileostomy closure, it was found that the patient had a transverse colonic mass which was resected. Histological examination with immunohistochemistry confirmed it to be a Mucosa-Associated Lymphoid Tissue Lymphoma (MALToma). Colonic MALToma in the background of PJS is a unique case for which it has been reported.
Management of stevens-johnson syndrome-toxic epidermal necrolysis: Looking beyond guidelines!
Indian Journal of Dermatology 2018 63(2):117-124
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions, which are mainly caused by drugs; and these are usually associated with high degree of morbidity and mortality. Recently, two detailed guidelines were published on the management of SJS/TEN, Indian guidelines and UK guidelines. Still, there is no consensus on the management of SJS/TEN. In this article, our aim is to conceptualize the management aspect of SJS/TEN considering Indian setup. Early discontinuation of all medicines, supportive measures (hydration, electrolytes, and care of denuded skin), corticosteroids and cyclosporine has been found to be useful. Oral provocation test is reserved for patients, who undergo complete remission and this is to be done after hospitalization, under strict vigilance. As there is no consensus, the treatment should be individualized on case to case basis.
Cutaneous metastasis leading to the diagnosis of carcinoma of the prostate: A rare case report and review of literature
Indian Journal of Dermatology 2018 63(2):182-185
Prostate adenocarcinoma is a common urologic malignant neoplasm in man. Distant cutaneous metastases (CMs) of prostate carcinoma are extremely rare with a reported incidence of 0.36% and usually they occur late. Clinically, cutaneous metastasis of prostate carcinoma can mimic other skin conditions such as cellulitis, sebaceous cyst, zosteriform lesions, telangectasias, and more, resulting in a poor recognition. Few cases of true cutaneous metastatic prostate carcinoma exist in the literature. We present a case, where the first sign of carcinoma of the prostate was CM over the anterior abdominal wall. Radiological and histopathological confirmation pointed to a diagnosis of carcinoma of the prostate. The patient was treated with orchidectomy and was started on bicalutamide. After 1 month of bicalutamide therapy there was subjective decrease in the size of the metastasis. A high index of suspicion is required while evaluating the different differential diagnoses of this entity particularly in elderly patients.
Controversies in the management of cutaneous adverse drug reactions
Indian Journal of Dermatology 2018 63(2):125-130
Some cutaneous adverse drug reactions (CADRs) are severe life-threatening conditions due to multisystem involvements with a high morbidity and mortality rates ranging from 25 - 70% and require immediate medical care. But there are huge controversies regarding the management because large clinical trials are lacking. Most frequent discussion and division occur regarding the use of systemic corticosteroid as early intervention with corticosteroids controls inflammation. Corticosteroids are potent agents that target several intracellular processes to modify almost all components of inflammatory and immune responses but their impact on the long term disease course is not known. Controlled relapses of rash and hepatitis may occur as corticosteroids are tapered. A chronic HHV6 activation promoted by systemic steroids could explain these relapses. Second important issue is the use of antitubercular drugs (ATD) in case of CADR due to multidrug therapy of ATD. As both the tuberculosis and CADR are life threatening conditions and we can not spare treatment of tuberculosis for CADR, we should come to a conclusion which is not yet decided. In the same way the use of antileprotic MDT in CADR due to MDT raises a similar controversy. So, here we focus on those controversies and discuss the issues.
Hidradenitis suppurativa: Inside and out
Indian Journal of Dermatology 2018 63(2):91-98
Hidradenitis suppurativa is a chronic, disabling, suppurative disease characterized by deep tender subcutaneous nodules; complicated by fibrosis and extensive sinuses affecting primarily the apocrine gland bearing areas. It affects all races in early 20s with greater prevalence seen in women (3 to 5:1). The estimated disease prevalence is 1 - 4 %. The disease is speculated to be caused by follicular structural abnormalities with associated risk factors as smoking, obesity, positive family history and shaving. Certain co-morbidities can also be seen such as inflammatory bowel disease, spondyloarthropathies, epithelial tumors, pyoderma gangrenosum etc. Treatment modalities include counseling of the patient to lose weight if obese, to wear loose clothes, stop smoking and maintain good hygiene. Topical antibiotics, like 1% clindamycin, have shown to give good results along with benzoyl peroxide wash. Orally cocktail of antibiotics can be given, though biologicals remain the best treatment option. Surgical excision can be done in later stages and in recalcitrant cases.
Evaluation of adolescents diagnosed with acne vulgaris for quality of life and psychosocial challenges
Indian Journal of Dermatology 2018 63(2):131-135
Background: Acne vulgaris is a common skin condition which affects most adolescents. It has a major impact on quality of life and psychosocial well-being. Aims: The aims of the study were to examine the psychosocial effects of acne on adolescents and changes in quality of life, and to reveal any difference in the possible effect between genders. In addition, an investigation of the association between acne severity and quality of life as well as psychosocial stress was conducted. Materials and Methods: The present study included 164 adolescents with a mean age of 12–18 years and was diagnosed with acne vulgaris without any previous treatment. The control group consisted of 188 healthy volunteers. Acne severity was evaluated by the global acne grading system. All patients filled in a Children's Dermatology Life Quality Index, Pediatric Quality of Life Questionnaire (PedsQL), and a Strength and Difficulties Questionnaire (SDQ). Results: The scores of SDQ and PedsQL were significantly lower in the case group. There was no significant correlation found between the genders in the control group for acne severity and scale scores. No significant correlation was found between acne severity and psychosocial challenges. Conclusions: The results of the present study show that acne has a significant effect on quality of life for adolescents, and this has an impact on their psychosocial life. Another important finding of the present study is that worsening in quality of life is not affected by some factors such as duration, severity of acne and age.
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A Case of Diprosopus Tetraophthalmos: Ocular Findings and Surgical Treatment of Exposure KeratopathyDiprosopus is a rare variation of conjoined twinning. In this report, ophthalmic findings in an infant with diprosopus tetraophthalmos are p...