Neurofibromatosis, which was first described in 1882 by Von Recklinghausen, is a genetic disease characterized by a neuroectodermal abnormality and by clinical manifestations of systemic and progressive involvement which mainly affect the skin, nervous system, bones, eyes and possibly other organs. The disease may manifest in several ways and it can vary from individual to individual. Given the wealth of information about neurofibromatosis, we attempted to present this information in different ways. In the first part of this work, we present a chronological history, which describes the evolution of the disease since the early publications about the disorder until the conclusion of this work, focusing on relevant aspects which can be used by those wishing to investigate this disease. In the second part, we present an update on the various aspects that constitute this disease.
The scientific community and general public have been exposed to a series of achievements attributed to a new area of knowledge: Nanotechnology. Both abroad and in Brazil, funding agencies have launched programs aimed at encouraging this type of research. Indeed, for many who come into contact with this subject it will be clear the key role that chemical knowledge will play in the evolution of this subject. And even more, will see that it is a science in which the basic structure is formed by distilling different areas of inter-and multidisciplinary knowledge along the lines of new paradigms. In this article, we attempt to clarify the foundations of nanotechnology, and demonstrate their contribution to new advances in dermatology as well as medicine in general. Nanotechnology is clearly the future.
We report five never reported cases of chromoblastomycosis, and we review the clinical experience in diagnosis and treatment of chromoblastomycosis, an infrequent and neglected subcutaneous mycosis.
BACKGROUNDOnychomycosis is a fungal infection of the nails caused in most cases by dermatophytes Trichophyton rubrum and Trichophyton mentagrophytes. Despite numerous available antifungal drugs for therapy of this infection, the cure rate is low, with high rates of relapse after treatment and side effects.OBJECTIVESTo present a new option for the treatment of onychomycosis, in search of a more effective and rapid method than conventional ones.METHODSPatients underwent two sessions of CO2 fractional laser 10.600nm associated with photodynamic therapy. Mycological and digital photography were performed before and after the treatment.RESULTSMcNemar test with continuity correction and degrees of freedom = 1: for clinical cure rate, 13.06, with p=0.00005; for mycological cure, 17.05, with p=0.00005; 72% felt fully satisfied with the procedure.CONCLUSIONSThe use of fractional CO2 laser 10.600nm associated with photodynamic therapy can be effective in the treatment of onychomycosis, decreasing the risk of systemic lesions that may be triggered with prolonged use of oral antifungals.
A) and four polymorphisms (c.3315-27G>A, V1146I, V1317A, c.4514+11C>G) were identified. These data were recently published.]]>
This case report is about a 48-year-old female patient with systemic amyloidosis and multiple myeloma simultaneously. Amyloid cutaneous infiltrative lesions like papules, nodules, or plaques with a serous-hemorrhagic aspect were found in the eyelids, neck and retroauricular region, among others. She had presented intermittent papular lesions on the upper eyelids one year before, which worsened following local trauma. A local skin biopsy showed amorphous and eosinophilic substance in the dermis. Congo red staining confirmed the amyloid deposits. Abnormal exams: proteinuria (570mg/24h), Bence-Jones proteinuria and clonal plasma cells (70%) found in myelogram. Following the diagnosis of multiple myeloma based on amyloid skin lesions, the patient was referred to the Hematology service and died 5 months after the diagnosis. Keywords: Amyloidosis; Eyelids; Multiple myeloma; Skin Resumo: Relatamos um caso de uma paciente de 48 anos com amiloidose sistêmica associada a mieloma múltiplo. Lesões infiltrativas cutâneas como pápulas, nódulos ou placas com aspecto sero-hemorrá-gico podem ser localizados nas pálpebras, pescoço, região retroauricular dentre outras. No presente caso, as pálpebras foram acometidas por pápulas, há 1 ano, de caráter intermitente e piora após trauma local. Biópsia local evidenciou material amorfo e eosinofílico na derme. A coloração vermelho do Congo confirmou presença de substância amiloide. Exames anormais: proteinúria de 570mg/24 horas, proteinúria de Bence-Jones positiva e mielograma com 70% de plasmócitos atípicos. Assim, realizou-se o diagnóstico de mieloma múltiplo a partir de manifestações cutâneas de amiloidose. Paciente encaminhada ao serviço de hematologia e foi a óbito em 5 meses.
Three monthly pulses of azithromycin 500 mg for 3 consecutive days is safe, well tolerated, effective and promotes increased patient adhesion to the treatment.
IntroductionPsoriasis is an immune-mediated, chronic, inflammatory disease, which has a substantial humanistic and economic burden. This study aimed to assess the impact of this disease on health-related quality of life (HRQoL), work productivity, and direct and indirect costs from a societal perspective among Brazilian patients.MethodsThis is a cross-sectional, observational, multicenter study, enrolling patients with moderate to severe plaque psoriasis according to physician evaluation. Data collection was performed from December 2015 to November 2016 through face-to-face interviews using a structured questionnaire and five standardized patient-reported outcomes instruments. Direct costs were estimated by multiplying the amount of resources used (12-month recall period) by the corresponding unit cost. Indirect costs were grouped in two time horizons: annual costs (income reduction and absenteeism) and lifetime costs (demission and early retirement).ResultsA total of 188 patients with moderate to severe plaque psoriasis were included, with mean age of 48.0 (SD 13.1). “Anxiety and depression” and “pain and discomfort” were the most impaired dimensions, according to the EuroQol Five-Dimension-Three-Level (EQ-5D-3L). The highest effect was found for “symptoms and feelings” [mean (SD) 2.4 (1.7)] Dermatology Life Quality Index (DLQI) subscale. Psoriatic arthritis (PsA) presence and biologic-naïve status were associated with worse HRQoL. Presenteeism was more frequent than absenteeism, according to the Work Productivity and Activity Impairment questionnaire-General Health (WPAI-GH) [17.4% vs. 6.3%], while physical demands and time management were the most affected Work Limitations Questionnaire (WLQ) subscales [means (SD) 23.5 (28.5) and 17.7 (24.9), respectively]. The estimated annual cost per patient was USD 4034. Direct medical costs accounted for 87.7% of this estimate, direct non-medical costs for 2.4%, and indirect costs for 9.9%.ConclusionsResults evidenced that moderate to severe plaque psoriasis imposes substantial costs to society. Our data showed that this disease negatively affects both work productivity and HRQoL of Brazilian patients. Subgroups with PsA and biologic-naïve patients presented lower HRQoL, showing the impact of this comorbidity and the relevance of biologics in psoriasis treatment.FundingNovartis Biociências S.A.Electronic supplementary materialThe online version of this article (10.1007/s12325-019-01049-7) contains supplementary material, which is available to authorized users.
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