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Huidziekten --- Psoriasis --- Diagnosis --- Alternative treatment
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Dermatologie --- Huidziekten --- Academic collection --- Theses
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De ziekte van Hailey-Hailey (Hailey-Hailey disease, HHD) is een autosomaal dominante dermatose die optreedt bij volwassenen in de lichaamsplooien. Er ontstaan plaques met pijnlijke, parallel verlopende fissuren. De belangrijkste uitlokkende factoren zijn wrijving, zweten, UV-licht en infecties. De ziekte wordt veroorzaakt door mutaties in het ATP2C1 gen. Dit gen codeert voor hSPCA1, een pomp die calcium en mangaan naar het lumen van het golgiapparaat pompt. Er ontstaat een verstoring van de celadhesie bij suprabasale keratinocyten, wat resulteert in partiële acantholyse. Histologisch zien we intra-epidermale blaren. In deze blaren bevinden zich clusters van nog gedeeltelijk verbonden keratinocyten. Dit resulteert in een beeld dat doet denken aan een vervallen bakstenen muur (‘dilapidated brick wall’). De diagnose wordt vaak langdurig verward met andere aandoeningen van de plooien. De behandeling van deze chronische aandoening is erop gericht surinfecties te bestrijden en opstoten te voorkomen. Verder onderzoek omtrent een gerichte behandeling is nodig.
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Background. Calciphylaxis is a rare disorder predominantly seen in patients with chronic kidney disease. Calcification of cutaneous vessels leads to extremely painful plaques and ulcerations. Mortality is high, sepsis being the leading cause of death. There is no standardized treatment. In the last years, administration of sodium thiosulfate (STS) has yielded some promising results. Methods. We retrospectively collected data of 8 calciphylaxis patients treated with STS at the University Hospital of Leuven between June 2009 and June 2014. Results. Eight patients, of whom 2 without related renal risk factors, received STS treatment in different dosing schemes. Four patients (50%) achieved complete healing of skin lesions; two patients had only stabilization of the disease while experiencing pain relief. In the two remaining patients who had further progression of the disease, STS treatment led to analgesia in one patient. Overall, 7 patients (87.5%) had some benefit from STS treatment. However, 4 patients (50%) eventually died due to causes related to calciphylaxis. STS was generally well tolerated, although almost all patients (87,5%) had metabolic acidosis. Conclusions. Treatment of calciphylaxis remains challenging. Although STS therapy, together with other treatment modalities, seems to improve disease outcome, more studies focusing on optimal dosing schemes and duration of treatment are necessary.
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The high prevalence of psoriasis and the high spending on pharmaceuticals motivates a more evidence-based and cost-effective usage of biopharmaceuticals. A growing body of evidence exists that the implementation of therapeutic drug monitoring (TDM) for biopharmaceuticals in psoriasis patients optimizes patient management and clinical outcome and enhances their efficacy. Therefore, the aim of this review is to give an overview of the literature on therapeutic drug monitoring of biopharmaceuticals in the treatment of psoriasis and to provide the useful information to dermatologists to improve health care in psoriasis patients.
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