von Willebrand factor (vWF) antigen levels are elevated in patients with end-stage kidney disease (ESKD). We determined the quantitative and qualitative abnormalities of vWF and factors influencing vWF proteolysis in participants with ESKD compared with age-matched controls and determined the association between abnormalities in vWF and mortality over 4 years of follow-up. vWF : Ag and von Willebrand factor propeptide (vWFpp) levels, vWF functional activity (vWF :RCo), vWF multimer profiles, ADAMTS-13, thrombospondin 1 (TSP-1), and interleukin 6 (IL-6) were evaluated before and after a single hemodialysis treatment in 55 individuals with vascular disease and an age-matched group of controls (n = 21). vWF : Ag and vWF activity were significantly higher in the ESKD patients and levels increased further following the dialysis procedure. The percentage of high molecular weight multimers (%HMWMs) was significantly elevated in the ESKD patients compared with controls. TSP-1 was lower and IL-6 was higher providing possible explanation for the increase in %HMWM in ESKD. The %HMWM dropped significantly in the postdialysis sample. Mortality at 4 years was significantly associated with vWF : Ag. There are higher plasma vWF : Ag levels and a small increase in HMWMs in the ESKD milieu. The acute drop in the %HMWM of vWF postdialysis appears to be due to shear forces encountered during the dialysis procedure. The contribution of these abnormalities to either a pro-thrombotic and/or pro-bleeding phenotype in this population requires further study.
Dermatitis herpetiformis (DH) is an autoimmune blistering disease associated with gluten-sensitive enteropathy. The current standard of care for DH consists of a strict glutenfree diet, with the addition of oral dapsone for rapid control of symptoms. 1 We present a case of DH treated for more than 1 year with topical dapsone 5% gel for initial control of symptoms and a gluten-free diet for maintenance.A 66-year-old woman presented to dermatology with a 2-year history of intermittent episodes of severe pruritus, vesicles, and crusting localized on her elbows and knees. Her medical history included hypothyroidism, for which she was taking levothyroxine, as well as irritable bowel syndrome. Physical examination revealed grouped hemorrhagic crusts on the elbows and knees bilaterally ( Figure 1) and several small vesicles on the left elbow. DH was clinically suspected and confirmed by pathology. Skin biopsy demonstrated a subepidermal bulla with adjacent papillary dermal neutrophilic microabscesses. Direct immunofluorescence of perilesional skin was positive for continuous granular deposits of immunoglobulin A along the basement membrane zone.While awaiting baseline bloodwork, including glucose-6-phosphate dehydrogenase enzyme levels (subsequently reported as normal), the patient was given a trial of topical dapsone 5% gel twice daily to affected areas on her elbows and knees and was referred to a dietician to start on a glutenfree diet. Dramatic improvement was noted at follow-up 2 weeks later, despite the patient having been on a gluten-free diet for only 3 days. Only a few crusts remained on the left elbow (Figure 2), while the right elbow and knees were clear and pruritus had resolved. The patient declined oral dapsone, preferring to stay on the topical 5% gel and continue on a strict gluten-free diet. She has remained well controlled on this regimen for more than 1 year.Topical dapsone is a well-tolerated medication and has only mild side effects, including local dryness, rash, and sunburn. Currently it is approved only for treatment of acne vulgaris. 2,3 It is also considered safe to use in patients with glucose-6-phosphate dehydrogenase deficiency and sulfonamide allergies, 4 giving a possible treatment option for patients with contraindications to oral dapsone. However, there has been 1 case of methemoglobinemia due to topical dapsone reported in the literature. 5 The only other report of topical dapsone 5% gel used to treat DH was in a 14-year-old boy with confirmed DH who was treated with oral dapsone Medical Letter 651053C MSXXX10.1177/1203475416651053Journal of Cutaneous Medicine and SurgeryBurbidge and Haber research-article2016 Figure 1. Initial presentation: grouped hemorrhagic crusts accompanied by vesicles on the left elbow.Figure 2. Two-week follow-up: marked improvement of the left elbow, with only a few small crusts remaining.
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