Frequency of Radiation Therapy-Induced Malignancies in Patients With Li-Fraumeni Syndrome and Early-Stage Breast Cancer and the Influence of Radiation Therapy Technique.

PURPOSE: Breast cancer (BC) is the most common malignancy in female patients with Li-Fraumeni syndrome (LFS), a condition associated with an increased risk of various malignancies, including radiation therapy (RT)-induced malignancies (RIM) within previously irradiated areas. Our study aimed to assess the incidence of RIM in patients with LFS and early-stage BC treated with adjuvant RT, including the effect of RT dose and technique. METHODS AND MATERIALS: We examined patients with a germline pathogenic/likely pathogenic TP53 variant diagnosed with early-stage BC and monitored by a hereditary cancer team at a single cancer center. The study endpoints included RIM frequency, the association of RIM with the dose and type of RT (2-dimensional [2D] RT, 3-dimensional [3D] RT, and intensity modulated RT [IMRT]), and BC recurrence. RESULTS: We analyzed 48 patients with a median age of 39 years (range, 21-62). The majority (71%) had the TP53 R337H variant, and 87% were unaware of their LFS diagnosis at the time of BC treatment. Treatment modalities included mastectomy (62%), (neo)adjuvant chemotherapy (66%), and RT (62%), with RT being more common after breast-conserving surgery (87% vs 46% with mastectomy, P = .010). Among the 30 patients treated with RT, 10% developed RIM in the irradiated field, consisting of 3 soft tissue malignancies. RT dose (≤40.8 or >40.8 Gy) did not influence RIM occurrence, but the type of RT did. RIM was observed in 100% of cases with 2D RT (2/2), 50% with IMRT (1/2), and 0% with 3D RT (0/16) (P = .004). CONCLUSIONS: Our study underscores a concerning rate of RIM after adjuvant RT, emphasizing the importance of a thorough risk-benefit evaluation before recommending RT, with preference for its avoidance if possible. Although subgroup sizes were limited, the risk of RIM appeared to be influenced by the RT technique, with higher rates observed with 2D RT and IMRT compared with 3D RT. Early TP53 testing is essential to guide the BC treatment plan.

A matched case-control study of the prognosis of early breast cancer in patients with Li-Fraumeni syndrome (BREAST TP53).

INTRODUCTION: Breast cancer (BC) is the most common type of cancer in premenopausal women with germline TP53 pathogenic variants (mTP53) (Li Fraumeni syndrome - LFS). However, little is known about the BC prognosis in these patients. This study analyzed the BC-related oncologic outcomes of patients with LFS. METHODS: We evaluated a cohort of LFS patients with BC in comparison with a control cohort of BC patients with no pathogenic variant in a hereditary cancer panel. The primary endpoint was recurrence-free survival (RFS). Due to the risk of second malignancies in LFS, only locoregional and distant recurrences were considered events for RFS. Secondary endpoints included rates of contralateral BC, overall survival (OS), and breast cancer-specific survival (BCSS). RESULTS: Forty-one patients were evaluated in the mTP53 group and 82 in the control group. Median age at BC diagnosis was 40 and 41 years, respectively. The mTP53 group received less adjuvant radiotherapy than the control group (63.4% vs 93.9%, P < 0.001). Other relevant baseline characteristics and treatment received were similar between groups. 5y-RFS rates were 79.4% in the mTP53 versus 93.6% in the control group (HR 2.43, 95%CI 0.74-8.01, P = 0.143); and were not impacted by the use of adjuvant radiotherapy. 5y-BCSS rates were 92.2% and 98.6%, respectively (HR 1.87, IC95% 0.25-13.48, P = 0.534). CONCLUSIONS: Our results showed no statistically significant difference in BC-related RFS and BCSS between patients with mTP53 and a control group with no pathogenic variant. Larger multicentric studies are warranted to confirm these results.

Trastuzumab deruxtecan versus trastuzumab emtansine in patients with HER2-positive metastatic breast cancer: updated results from DESTINY-Breast03, a randomised, open-label, phase 3 trial.

BACKGROUND: An improvement in progression-free survival was shown with trastuzumab deruxtecan versus trastuzumab emtansine in patients with HER2-positive metastatic breast cancer in the progression-free survival interim analysis of the DESTINY-Breast03 trial. The aim of DESTINY-Breast03 was to compare the efficacy and safety of trastuzumab deruxtecan versus trastuzumab emtansine. METHODS: This open-label, randomised, multicentre, phase 3 trial was done in 169 study centres in North America, Asia, Europe, Australia, and South America. Eligible patients were aged 18 or older, had HER2-positive unresectable or metastatic breast cancer previously treated with trastuzumab and a taxane, had an Eastern Cooperative Oncology Group performance status 0-1, and at least one measurable lesion per Response Evaluation Criteria in Solid Tumours version 1.1. Patients were randomly assigned (1:1) to receive trastuzumab deruxtecan 5·4 mg/kg or trastuzumab emtansine 3·6 mg/kg, both administered by intravenous infusion every 3 weeks. Randomisation was stratified by hormone receptor status, previous treatment with pertuzumab, and history of visceral disease, and was managed through an interactive web-based system. Within each stratum, balanced block randomisation was used with a block size of four. Patients and investigators were not masked to the treatment received. The primary endpoint was progression-free survival by blinded independent central review. The key secondary endpoint was overall survival and this prespecified second overall survival interim analysis reports updated overall survival, efficacy, and safety results. Efficacy analyses were performed using the full analysis set. Safety analyses included all randomly assigned patients who received at least one dose of study treatment. This study is registered with ClinicalTrials.gov, NCT03529110. FINDINGS: Between July 20, 2018, and June 23, 2020, 699 patients were screened for eligibility, 524 of whom were enrolled and randomly assigned to receive trastuzumab deruxtecan (n=261) or trastuzumab emtansine (n=263). Median duration of study follow-up was 28·4 months (IQR 22·1-32·9) with trastuzumab deruxtecan and 26·5 months (14·5-31·3) with trastuzumab emtansine. Median progression-free survival by blinded independent central review was 28·8 months (95% CI 22·4-37·9) with trastuzumab deruxtecan and 6·8 months (5·6-8·2) with trastuzumab emtansine (hazard ratio [HR] 0·33 [95% CI 0·26-0·43]; nominal p<0·0001). Median overall survival was not reached (95% CI 40·5 months-not estimable), with 72 (28%) overall survival events, in the trastuzumab deruxtecan group and was not reached (34·0 months-not estimable), with 97 (37%) overall survival events, in the trastuzumab emtansine group (HR 0·64; 95% CI 0·47-0·87]; p=0·0037). The number of grade 3 or worse treatment-emergent adverse events was similar in patients who received trastuzumab deruxtecan versus trastuzumab emtansine (145 [56%] patients versus 135 [52%] patients). Adjudicated drug-related interstitial lung disease or pneumonitis occurred in 39 (15%) patients treated with trastuzumab deruxtecan and eight (3%) patients treated with trastuzumab emtansine, with no grade 4 or 5 events in either group. INTERPRETATION: Trastuzumab deruxtecan showed a significant improvement in overall survival versus trastuzumab emtansine in patients with HER2-positive metastatic breast cancer, as well as the longest reported median progression-free survival, reaffirming trastuzumab deruxtecan as the standard of care in the second-line setting. A manageable safety profile of trastuzumab deruxtecan was confirmed with longer treatment duration. FUNDING: Daiichi Sankyo and AstraZeneca.

Trastuzumab Deruxtecan versus Trastuzumab Emtansine for Breast Cancer.

BACKGROUND: Trastuzumab emtansine is the current standard treatment for patients with human epidermal growth factor receptor 2 (HER2)-positive metastatic breast cancer whose disease progresses after treatment with a combination of anti-HER2 antibodies and a taxane. METHODS: We conducted a phase 3, multicenter, open-label, randomized trial to compare the efficacy and safety of trastuzumab deruxtecan (a HER2 antibody-drug conjugate) with those of trastuzumab emtansine in patients with HER2-positive metastatic breast cancer previously treated with trastuzumab and a taxane. The primary end point was progression-free survival (as determined by blinded independent central review); secondary end points included overall survival, objective response, and safety. RESULTS: Among 524 randomly assigned patients, the percentage of those who were alive without disease progression at 12 months was 75.8% (95% confidence interval [CI], 69.8 to 80.7) with trastuzumab deruxtecan and 34.1% (95% CI, 27.7 to 40.5) with trastuzumab emtansine (hazard ratio for progression or death from any cause, 0.28; 95% CI, 0.22 to 0.37; P<0.001). The percentage of patients who were alive at 12 months was 94.1% (95% CI, 90.3 to 96.4) with trastuzumab deruxtecan and 85.9% (95% CI, 80.9 to 89.7) with trastuzumab emtansine (hazard ratio for death, 0.55; 95% CI, 0.36 to 0.86; prespecified significance boundary not reached). An overall response (a complete or partial response) occurred in 79.7% (95% CI, 74.3 to 84.4) of the patients who received trastuzumab deruxtecan and in 34.2% (95% CI, 28.5 to 40.3) of those who received trastuzumab emtansine. The incidence of drug-related adverse events of any grade was 98.1% with trastuzumab deruxtecan and 86.6% with trastuzumab emtansine, and the incidence of drug-related adverse events of grade 3 or 4 was 45.1% and 39.8%, respectively. Adjudicated drug-related interstitial lung disease or pneumonitis occurred in 10.5% of the patients in the trastuzumab deruxtecan group and in 1.9% of those in the trastuzumab emtansine group; none of these events were of grade 4 or 5. CONCLUSIONS: Among patients with HER2-positive metastatic breast cancer previously treated with trastuzumab and a taxane, the risk of disease progression or death was lower among those who received trastuzumab deruxtecan than among those who received trastuzumab emtansine. Treatment with trastuzumab deruxtecan was associated with interstitial lung disease and pneumonitis. (Funded by Daiichi Sankyo and AstraZeneca; DESTINY-Breast03 ClinicalTrials.gov number, NCT03529110.).

Radiotherapy-induced malignancies in breast cancer patients with TP53 pathogenic germline variants (Li-Fraumeni syndrome).

The risk of radiotherapy-induced malignancies (RIMs) is a concern when treating Li-Fraumeni syndrome (LFS) or Li-Fraumeni Like (LFL) patients. However, the type of TP53 pathogenic germline variant may possibly influence this risk. TP53 p.R337H mutation is particularly prevalent in Brazil. We aimed to evaluate the outcomes of patients with pathogenic TP53 variants treated for localized breast cancer in a Brazilian cohort. We evaluated retrospectively a cohort of patients with germline TP53 pathogenic variants treated for localized breast cancer between December 1999 and October 2017. All patients were followed by the Hereditary Cancer Group of an academic cancer center. Our primary objective was to evaluate the occurrence of RIMs after adjuvant radiotherapy. Sixteen patients were evaluated; 10 (62.5%) had a germline TP53 p.R337H pathogenic variant. Median age was 39.8 years. Thirteen patients had invasive ductal carcinoma: 8 (61.5%) were hormone receptor-positive; 6 (46.1%), human epithelial growth factor receptor 2 (HER2)-amplified. Three patients had ductal carcinoma in situ. Most patients (N = 12/16, 75%) received adjuvant radiotherapy. After a median follow-up of 52.5 months, 2 patients (2/12, 16.6%) had RIMs. One had a fibrosarcoma and the other, a low-grade leiomyosarcoma. In the group treated with radiotherapy, one distant recurrence was diagnosed (1/12), and no loco-regional recurrence occurred. Among 4 patients who did not receive radiotherapy, 2 presented with loco-regional recurrence. In this cohort of patients with LFS enriched in TP53 p.R337H pathogenic variant, the incidence of RIMs after treatment of localized breast cancer was lower than previous literature. Nevertheless, rates of RIMs were still alarming. Early molecular diagnosis and careful evaluation of treatment risks and benefits are essential for these patients.

Return to work after breast cancer diagnosis: An observational prospective study in Brazil.

BACKGROUND: In North America and Europe, return-to-work (RTW) rates vary among breast cancer (BC) survivors, from 24% to 66% and from 53% to 82% at 6 and 36 months after diagnosis, respectively. To date, there is a lack of data on RTW rates after BC diagnosis in Latin America. Therefore, the primary objectives of this study were to define RTW rates at 12 and 24 months after BC diagnosis and to identify the factors associated with RTW in this population. METHODS: In total, 125 employed women from a single institution with newly diagnosed BC were interviewed by telephone at 6, 12, and 24 months after diagnosis. Those who had inoperable or metastatic disease were excluded. RESULTS: Overall, RTW rates were 30.3% and 60.4% at 12 and 24 months after BC diagnosis, respectively. Most women reported that they received support from their employer, but only 29.1% reported having been offered work adjustments. In multivariate analysis, the factors associated with positive RTW outcomes included higher household income (odds ratio [OR], 17.76; 95% confidence interval [CI], 3.33-94.75; P = .001), breast-conserving surgery (OR, 9.77; 95% CI, 2.03-47.05; P = .004), and work adjustments (OR, 37.62; 95% CI, 2.03-47.05; P = .004). The factors associated with negative RTW outcomes included adjuvant endocrine therapy (OR, 0.11; 95% CI, 0.02-0.74; P = .023), and depression diagnosed after BC (OR, 0.07; 95% CI, 0.01-0.63; P = .017). CONCLUSIONS: RTW rates in the current study were lower than those observed in developed countries but similar to the rates among low-income Americans. Workplace adjustments, higher income, breast-conserving surgery, endocrine therapy, and depression after BC played an important role in the RTW decision.

Serum levels of VEGF and MCSF in HER2+ / HER2- breast cancer patients with metronomic neoadjuvant chemotherapy.

Metronomic therapy has been gaining importance in the neoadjuvant setting of breast cancer treatment. Its clinical benefits may involve antiangiogenic machinery. Cancer cells induce angiogenesis to support tumor growth by secreting factors, such as vascular endothelial growth factor (VEGF). In breast cancer, Trastuzumab (TZM) based treatment is of key importance and is believed to reduce diameter and volume of blood vessels as well as vascular permeability. Here in we investigated serum levels of angiogenic factors VEGF and MCSF in patients receiving metronomic neoadjuvant therapy with or without TZM. We observed in HER2+ cohort stable levels of MCSF through treatment, whereas VEGF trend was of decreasing levels. In HER2- cohort we observed increasing levels of MCSF and VEGF trend. Overall, HER2+ patients had better pathological response to treatment. These findings suggest that angiogenic pathway may be involved in TZM anti-tumoral effect in the neoadjuvant setting.

Bone Metastases in Patients with Neuroendocrine Neoplasm: Frequency and Clinical, Therapeutic, and Prognostic Relevance.

BACKGROUND: The incidence and prevalence of neuroendocrine neoplasms (NEN) are rising. In view of continuously improving imaging techniques, more than half of the patients present with distant metastases at initial diagnosis. An advanced disease stage negatively correlates with the 5-year survival rate. In stage IV disease, bone metastases (BM) are frequent, yet knowledge concerning their clinical or prognostic relevance is rare. This study presents a single-center experience on the frequency and management of BM in patients with gastroenteropancreatic NEN and lung carcinoids. METHODS: Between 2000 and June 2015, 327 of 677 patients treated in the European Neuroendocrine Tumor Society (ENETS) center in Marburg (Germany) presented with distant metastases (48.3%), including 85 patients (12.6%) with BM. Data of both groups were analyzed using descriptive statistics. Overall survival was assessed by Kaplan-Meier curves and compared by log-rank test. RESULTS: Median age in the BM group was 54.9 years, the small intestine and the pancreas being the most common primaries. 83.5% of the tumors were well and moderately differentiated (G1/G2). Nearly half of the patients with BM were symptomatic and suffered either from pain (42.4%) or had fractures (11.7%). Bisphosphonates were employed in almost two-thirds of the patients, radiation therapy in 25.9%. Overall survival was significantly inferior in patients with BM than in those with other distant metastases (p = 0.01; 49.0 vs. 100.8 months). CONCLUSION: BM appear to have a significant clinical and prognostic impact. Further studies are needed to evaluate therapeutic approaches directed to the treatment of BM in particular for asymptomatic patients.

Bacterial skin colonization and infections in patients with atopic dermatitis / Colonização bacteriana e infecções da pele em pacientes com dermatite atópica

Atopic Dermatitis is a chronic inflammatory skin disease that affects a large number of children and adults. The disease results from an interaction between genetic predisposition, host environment, skin barrier defects, and immunological factors. A major aggravating factor associated with Atopic Dermatitis is the presence of microorganisms on the patient's skin surface. Staphylococcus aureus and Streptococcus pyogenes, for instance, can exacerbate chronic skin inflammation. As a result, antimicrobials have often been prescribed to control the acute phase of the disease. However, increased bacterial resistance to antimicrobial agents has made it difficult for dermatologists to prescribe appropriate medication. In the presence of disseminated dermatitis with secondary infection, systemic antibiotics need to be prescribed; however, treatment should be individualized, in an attempt to find the most effective antibiotic with fewer side effects. Also, the medication should be used for as short as possible in order to minimize bacterial resistance.

A dermatite atópica é uma doença inflamatória crônica da pele que afeta um grande número de crianças e adultos. A doença resulta da interação entre predisposição genética, fatores ambientais, defeitos da barreira cutânea e fatores imunológicos. Um dos grandes fatores agravantes associados à dermatite atópica é a presença de microorganismos na superfície cutânea desses pacientes. Staphylococcus aureus e Streptococcus pyogenes, por exemplo, podem exacerbar a inflamação crônica da pele. Como resultado, antimicrobianos são prescritos para controlar a fase aguda da doença. O constante crescimento da resistência bacteriana aos antimicrobianos tem tornado a escolha do mais adequado medicamento uma difícil decisão para os dermatologistas. Na presença de dermatite disseminada com infecção secundaria, antibióticos sistêmicos necessitam ser prescritos; no entanto, o tratamento deve ser individualizado, de forma a encontrar o antimicrobiano mais eficaz e com menores efeitos colaterais. Além disso, esse medicamento deve ser utilizado pelo menor tempo possível, a fim de minimizar a resistência bacteriana.

Identificação de espécies de malassésia na pitiríase versicolor em um serviço de dermatologia do sul do Brasil / Identification of Malassezia yeast species isolated from patients with pityriasis versicolor

A pitiríase versicolor é uma doença de distribuição universal. Existe a descrição de 12 espécies de malassezia. O objetivo deste estudo foi determinar quais as espécies de malassezia mais prevalentes nos pacientes com pitiríase versicolor. Foram realizadas as coletas através de raspado das lesões nos pacientes com suspeita clínica de pitiríase versicolor e posterior exame micológico e cultural para identificação final da espécie. Foram coletadas amostras de 87 pacientes. Quanto às culturas, 30 percent foram de Malassezia sympodialis, 25,7 percent de Malassezia furfur, 22,7 percent de Malassezia globosa, 12,1 percent de Malassezia retrita, 7,6 percent de Malassezia obtusa e 1,5 percent de Malassezia sloofiae.

Pityriasis versicolor (PV) is a disease with worldwide distribution. Twelve different species of Malassezia yeast have been described. The objective of this study was to determine which species of Malassezia are more prevalent in patients with pityriasis versicolor. Samples were collected by scraping the lesions of 87 patients with a clinical suspicion of pityriasis versicolor. The samples were then submitted to fungal microscopy and culture to identify the species. The species found were: Malassezia sympodialis (30 percent), Malassezia furfur (25.7 percent), Malassezia globosa (22.7 percent), Malassezia restricta (12.1 percent), Malassezia obtusa (7.6 percent) and Malassezia slooffiae (1.5 percent).

Exposição solar, fotoproteção e câncer da pele: comparação entre os conhecimentos auto-referidos por médicos residentes em pediatria e dermatologia / Sun exposure, photoprotection and skin cancer: a comparison of self-reported knowledge by pediatrics and dermatology residents

Objetivo: Avaliar os dados com os quais trabalham os residentes dos Serviços de Pediatria de Porto Alegre ­ RS sobre fotoproteção e câncer da pele, comparados com os especializandos de dermatologia da mesma cidade. Materias e métodos: Estudo descritivo transversal; incluindo 58 residentes de pediatria (RP) e 34 especializandos de dermatologia (ED) que responderam a questionário estruturado sobre fotoproteção e câncer da pele. Resultados: Dos RP 38% e dos ED 97% obtiveram informações sobre fotoproteção na pós-graduação. Quando perguntado aos RP se indicam protetor solar, 15% indicam sempre, 36,20% freqüentemente, 36,20% algumas vezes, 11,9% raramente. Quando realizado o mesmo questionamento aos ED, 67,64% indicam sempre, 29,41% freqüentemente e 2,95% algumas vezes (p= 0,0002). Quanto à capacidade de avaliar sinais em crianças, dentre os RP ,17,24% afirmam sentirem-se muito capazes, 70,68% pouco capazes e 12,06% incapazes. Já dentre os ED, 44,11% sentem-se muito capazes, 52,94% pouco capazes e 2,94% incapazes (p= 0,015). Conclusão: Os dados sugerem que os residentes de pediatria provavelmente devam aprimorar seus estudos em fotoproteção. É importante ressaltar o papel dos programas de pós-graduação em Pediatria em enfatizar a necessidade de estudos sobre fotoproteção e em capacitar adequadamente os futuros profissionais para avaliações de lesões cutâneas em crianças (AU)

Objective: To evaluate information concerning photoprotection and skin cancer as used by pediatrics residents in comparison to dermatology residents in Porto Alegre ­ RS. Material and Methods: This is a transversal descriptive study including 58 pediatrics residents (PRs) and 34 dermatology residents (DRs) who answered a questionnaire about their knowledge and practices as regards photoprotection and skin cancer. Results: Thirty eight percent of the PRs and 97% of the DRs had received information about photoprotection in postgraduate courses. When asked if they prescribe sunscreens, 15% of the PRs said they always do, 36.20% often do, 36.20% sometimes, and 11.9% rarely. On the other hand, 67.64% of the DRs said they always prescribe sunscreens, 29.41% often do and 2.95% sometimes (p=0.0002). Concerning the ability to evaluate signs in children, 17.24% of the PR said they feel very capable, 70.68% little capable and 12.06% incapable. Among the DRs, 44.11% felt very capable, 52.94% little capable and 2.94% incapable (p=0,015). Conclusion: The data suggest that the pediatrics residents should improve their knowledge on photoprotection. It is important to highlight the role of the pediatrics graduate programs in emphasizing the need for studies into photoprotection and in adequately qualifying the future professionals to evaluate skin lesions in children (AU)

Relação entre de prurido e qualidade de vida de pacientes em hemodiálise / Relationship between pruritus and quality of life in patients on hemodialysis

FUNDAMENTOS: O prurido é um sintoma freqüente nos pacientes em hemodiálise. Tem etiologia não totalmente esclarecida e difícil manejo, o que piora de modo considerável a qualidade de vida dos doentes. OBJETIVOS: Avaliar o grau de prurido e sua influência na qualidade de vida dos pacientes renais crônicos em hemodiálise. MÉTODOS: Estudo transversal com pacientes renais crônicos em hemodiálise no Complexo Hospitalar Santa Casa de Porto Alegre, que apresentassem prurido sem outras dermatoses. Utilizou-se a escala análoga visual para mensuração do grau de prurido e o questionário do Índice de Qualidade de Vida para Dermatologia. RESULTADOS: Dos 200 pacientes avaliados, 69 (34,5 por cento) apresentaram prurido, dos quais 16 (23 por cento) de grau leve, 39 (56,5 por cento) moderado e 14 (20,2 por cento) grave. Quando analisada a qualidade de vida, verificou-se que em 14 (20,3 por cento) não houve influência do prurido na qualidade de vida. Entretanto, para 26 pacientes (37,4 por cento), o prurido teve um pequeno efeito, em nove (13 por cento) houve efeito moderado, 13 (18,8 por cento) foram afetados de maneira grave e em sete (10,1 por cento) houve influência extremamente grave. CONCLUSÕES: Neste estudo, 57 por cento dos indivíduos não demonstraram alterações importantes na qualidade de vida relacionadas ao prurido, mas um número significativo estava na faixa de muito e extremamente alterado, o que denota a necessidade de atenção ao tratamento deste sintoma neste grupo de pacientes.

INTRODUCTION: Pruritus is a frequent symptom affecting patients on hemodialysis. Its etiology is not known and treatment is difficult. This symptom considerably worsens the patients’ quality of life. OBJECTIVES: To evaluate the degree of pruritus and its influence on the quality of life of patients on hemodialysis. METHODS: Patients with chronic renal disease on hemodialysis and presenting pruritus in a Hospital of Porto Alegre were enrolled in this transversal study. A visual analogue scale for measuring the degree of pruritus was used and the Dermatology Life Quality Index questionnaire was applied. RESULTS: Of 200 evaluated patients; 69 (34.5 percent) presented pruritus, of whom 16 (23 percent) of mild degree, 39 (56.5 percent) moderate, 14 (20.2 percent) severe. Quality of life assessment revealed that in 14 of the patients (20.3 percent) there was no influence of pruritus, in 26 (37.4 percent) there was a small effect, in nine (13 percent) a moderate effect, in 13 (18.8 percent) a serious effect and in seven (10.1 percent) there was an extremely serious influence of pruritus on the quality of life. CONCLUSIONS: In this study, 57 percent of the individuals did not demonstrate important alterations in the quality of life related to the pruritus. A significant number of patients on hemodialysis have their quality of life from moderately to extremely modified by pruritus, which demonstrates the need for treating this symptom.

Necrólise epidérmica tóxica induzida porlamotrigina / Toxic epidermal necrolisis induced bylamotrigine

A Necrólise Epidérmica Tóxica (NET) é uma reação muco-cutânea potencialmentefatal, aguda e febril, comumente induzida por drogas e caracterizada por necrose e esfoliaçãodisseminadas na epiderme, envolvendo mais de 30% da superfície corpórea. Suaincidência é de 0,4 a 1,2 casos por milhão de pessoas por ano, com mortalidade entre 30 a70% dos casos. As drogas mais comumente associadas são as sulfonamidas, os anticonvulsivantes,os antiinflamatórios não-esteróides e o alopurinol. A lamotrigina, anticonvulsivanteindicado para o tratamento de crises convulsivas, epilepsia em gestantes e estabilizadordo humor, pode acarretar em até 15% dos casos lesões máculo-papulares simples.A ocorrência de reações cutâneas graves como NET é rara. O objetivo deste trabalhoé apresentar um caso de NET ocorrido pelo uso de lamotrigina, cuja evolução e recuperaçãoforam bastante favoráveis. Diante do quadro clínico, faz-se necessário o diagnósticodiferencial com síndrome da hipersensibilidade aos anticonvulsivantes e síndrome de Stevens-Johnson.

Toxic Epidermal Necrolysis (TEN) is a acute, but potencially lethal mucocutaneousreaction, usually caused by drugs leading to epidermal necrosis and exfoliation of over30% of body skin surface. Its incidence ranges from 0,4 to 1,2 cases per million peopleevery year and 30-70% death. He most commom drugs related to this condition are sulfonamides,antiepileptic drugs, NSAIDs and alopurinol. Lamotrigine, a drug used in thetreatment of seizures, epilepsy in pregnant women and as a mood stabilising agent, maylead to simple maculopapular rashes in 15% of cases. Serious cutaneous reactions, likeTEN, are rare. The purpose of this paper is to present the case of a patient who developedTEN due to use of lamotrigine and had a great recovery. Differencial diagnosis withanticonvulsant hypersensitivity syndrome and Stevens-Johnson syndrome may be done,once clinical manifestations may be similar.