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1.
Skin Health Dis ; 2(1): e92, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-35665202

RESUMO

Background: Flagellate dermatitis (FD) is a rare skin rash, which may occur following the administration of antineoplastic agents. It has been reported following the administration of bleomycin, docetaxel, trastuzumab, cisplatin, bendamustine and doxorubicin. We provide a summary of the epidemiology, aetiology, pathophysiology, and distribution of chemotherapy-induced FD. Methods: PubMed was searched using ((flagellat*) AND (Dermat*)) OR ((Flagellat*) AND (Erythema)). The search yielded 206 publications, out of which 54 individual case reports were identified which fulfilled our inclusion criteria. Statistical analysis was performed where appropriate. Results: Female patients were slightly more likely to develop FD compared to males. In the majority of cases FD appeared on the upper and lower limbs and pruritus was an accompanying feature in 51% of cases. Most cases developed after the first cycle of chemotherapy and females were statistically more likely to present within the first 72 hr (p <0.05). Skin biopsies were taken in 41% of cases and this was not statistically associated with the patient's gender, (p = 0.651), presentation within 72 hr (p = 0.076) or cancer diagnosis. Chemotherapy was stopped in 62% of patients and was associated with female gender (p = 0.0098). Most patients who received treatment were managed with topical steroids. Time for rash resolution ranged from a few weeks to four months following the discontinuation of the causative drug. Conclusion: FD is a rare adverse skin effect of chemotherapeutic treatment, most commonly presenting on the upper and lower limbs of patients following their first cycle of chemotherapy. Early presentation is more common in females leading to increased likelihood of stopping chemotherapy. Biopsy findings poorly correlate with disease severity. Continuation of chemotherapy treatment in combination with topical steroids may not adversely affect rash resolution.

2.
Orphanet J Rare Dis ; 17(1): 6, 2022 01 06.
Artigo em Inglês | MEDLINE | ID: mdl-34991670

RESUMO

BACKGROUND: Fabry disease (FD) is a treatable X-linked condition leading to progressive cardiac disease, arrhythmia and premature death. We aimed to increase awareness of the arrhythmogenicity of Fabry cardiomyopathy, by comparing device usage in patients with Fabry cardiomyopathy and sarcomeric HCM. All Fabry patients with an implantable cardioverter defibrillator (ICD) implanted in the UK over a 17 year period were included. A comparator group of HCM patients, with primary prevention ICD implantation, were captured from a regional registry database. RESULTS: Indications for ICD in FD varied with 72% implanted for primary prevention based on multiple potential risk factors. In FD and HCM primary prevention devices, arrhythmia occurred more frequently in FD over shorter follow-up (HR 4.2, p < 0.001). VT requiring therapy was more common in FD (HR 4.5, p = 0.002). Immediate shock therapy for sustained VT was also more common (HR 2.5, p < 0.001). There was a greater burden of AF needing anticoagulation and NSVT in FD (AF: HR 6.2, p = 0.004, NSVT: HR 3.1, p < 0.001). CONCLUSION: This study demonstrates arrhythmia burden and ICD usage in FD is high, suggesting that Fabry cardiomyopathy may be more 'arrhythmogenic' than previously thought. Existing risk models cannot be mutually applicable and further research is needed to provide clarity in managing Fabry patients with cardiac involvement.


Assuntos
Cardiomiopatia Hipertrófica , Desfibriladores Implantáveis , Doença de Fabry , Taquicardia Ventricular , Arritmias Cardíacas , Cardiomiopatia Hipertrófica/terapia , Humanos , Fatores de Risco , Taquicardia Ventricular/terapia
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