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    28 results match your criteria Bullous Disease of Dialysis

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    The Inpatient Burden of Autoimmune Blistering Disease in US Children: Analysis of Nationwide Inpatient Sample Data.
    Am J Clin Dermatol 2017 Apr;18(2):287-297
    Departments of Dermatology, Preventive Medicine and Medical Social Sciences, Northwestern University Feinberg School of Medicine, Suite 1600, 676 N. St. Clair St., Chicago, IL, 60611, USA.
    Background: Little is known about the epidemiology of pediatric autoimmune blistering disorders (PAIBD).

    Objective: We sought to determine the inpatient burden and comorbidities of PAIBD.

    Methods: We analyzed data from the Nationwide Inpatient Sample from 2002 to 2012, which contained a representative 20% sample of all US hospitalizations. Read More

    Development of bullous pemphigoid during the haemodialysis of a young man: case report and literature survey.
    Int Wound J 2017 Feb 17;14(1):288-292. Epub 2016 Oct 17.
    Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland.
    Haemodialysis is the most frequent form of renal replacement therapy (RRT) in patients with end-stage renal disorder (ESRD). Patients with ESRD frequently develop skin problems, mainly xerosis, pruritus and hyperpigmentation, as well as bullous diseases, mainly porphyria or pseudoporphyria and, in some cases, bullous pemphigoid (BP). BP is the most common autoimmune sub-epidermal blistering disease, and it predominantly affects elderly people. Read More

    Combined integrated protocol/basket trial design for a first-in-human trial.
    Orphanet J Rare Dis 2016 10 4;11(1):134. Epub 2016 Oct 4.
    Department of Clinical Pharmacology, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria.
    Background: Innovative trial designs are sought to streamline drug development in rare diseases. Basket- and integrated protocol designs are two of these new strategies and have been applied in a handful oncologic trials. We have taken the concept outside the realm of oncology and report about a first-in-human integrated protocol design that facilitates the transition from phase Ia in healthy volunteers to phase Ib in patients with rare complement-mediated disorders driven by the classical pathway. Read More

    Drug induced pseudoporphyria in CKD: A case report.
    Indian J Nephrol 2015 Sep-Oct;25(5):307-9
    Department of Medicine, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India.
    Pseudoporphyria (PP) is used to describe a photodistributed bullous disorder with clinical and histologic features of porphyria cutanea tarda (PCT) but without accompanying biochemical porphyrin abnormalities. Medications, excessive sun and ultraviolet radiation exposure, have all been reported to develop PP. We report a case of PP in a 49-year-old man with CKD stage 3a, caused due to torsemide intake. Read More

    Combined acute interstitial pneumonitis and pancytopenia induced by low-dose methotrexate in a hemodialysis patient treated for bullous pemphigoid.
    An Bras Dermatol 2015 May-Jun;90(3 Suppl 1):43-5
    Nanjing University, Nanjing, CN.
    Methotrexate has been widely used for many years in the treatment of a variety of diseases. Acute pneumonitis and bone marrow suppression are very serious side effects in methotrexate treatment. A 48-year-old man with end-stage renal disease undergoing chronic hemodialysis developed combined acute pneumonitis and pancytopenia after a cumulative dose of 20 mg methotrexate for bullous pemphigoid. Read More

    Dialysis-associated pseudoporphyria successfully treated with vitamin D. Report of two cases.
    G Ital Dermatol Venereol 2015 Jun;150(3):327-9
    Unit of Dermatology, NESMOS Department, Faculty of Medicine, Sant'Andrea" Hospital "Sapienza" University, Rome, Italy -
    Pseudoporphyria refers to a rare bullous dermatosis characterized by the clinical and histological features of porfiria cutanea tarda without abnormalities in porphyrin metabolism. The pathogenesis is heterogeneous and several exogenous factors may promote the bullous lesion formation, including medications, end stage renal disease, dialysis and tanning beds. Regarding treatment of this condition, in literature different therapy have been reported, such as glutathione and his precursor N-acetylcysteine, which presents anti-oxidant properties; however even more toxic drugs, such as chloroquine, are used. Read More

    Nafcillin-induced bullous skin eruption with granulocytopenia in a patient with end-stage renal disease.
    Intern Med 2012 1;51(3):287-9. Epub 2012 Feb 1.
    Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Republic of Korea.
    Nafcillin is an antibiotic used for infections due to penicillin-resistant Staphylococcus aureus. In general, the adverse reactions to nafcillin have not been frequent and serious. We report here a new type of adverse reaction to nafcillin in a patient with end-stage renal disease in whom nafcillin caused the unexpected complication of bullous drug eruption and subseqent neutropenia. Read More

    Lepromatous leprosy in a kidney transplant recipient: a case report.
    Exp Clin Transplant 2011 Jun;9(3):203-6
    Department of Nephrology, Dialysis and Transplantation, Tabriz University of Medical Sciences, Tabriz, Iran.
    Leprosy is a chronic granulomatous disease of the skin and peripheral nerves caused by Mycobacterium leprae. Among mycobacterial infections, leprosy is rare in renal transplant recipients. Here, we report the manifestations of lepromatous leprosy in a 41-year-old renal transplant recipient. Read More

    Comparison of cutaneous manifestations in chronic kidney disease with or without dialysis.
    Postgrad Med J 2010 Nov;86(1021):641-7
    Department of Dermatology & STD, and Medicine (Nephrology Division), University College of Medical Sciences & GTB Hospital, Dilshad Garden, Delhi, India.
    Purpose: To study and compare dermatological manifestations in patients with various stages of chronic kidney disease (CKD) and end stage renal disease (ESRD), undialysed and dialysed, in a developing country.

    Study Design: 200 patients were recruited, 50 each in stages 3, 4 and 5 CKD (undialysed) and 50 in stage 5 undergoing maintenance haemodialysis (MHD) for at least 1 month. Patients in stages 3 and 4 constituted pre-ESRD while stage 5 (both dialysed and undialysed) formed the ESRD group. Read More

    Bullous skin lesions in a patient undergoing chronic hemodialysis.
    Semin Dial 2010 Jan-Feb;23(1):83-7
    Section of Nephrology, Rush University Medical Center, Chicago, Illinois 60607-1878, USA.
    Porphyria cutanea tarda (PCT) is a vesiculobullous skin disorder characterized by a defect in heme biosynthesis. Reduced activity of the hepatic enzyme uroporphyrinogen decarboxylase (URO-D) results in accumulation of photosensitive porphyrins; this ultimately leads to the skin fragility and blistering that is characteristic of this disease. The majority of cases of PCT are associated with acquired deficiencies of the enzyme URO-D, secondary to hepatic injury precipitated by medications or infections. Read More

    Hematopoietic stem cell transplantation for systemic lupus erythematosus, the antiphospholipid syndrome and bullous skin diseases.
    Autoimmunity 2008 Dec;41(8):639-47
    Divisione di Ematologia e Trapianto di Cellule Staminali, Azienda Ospedaliera-Universitaria S.Martino, Genoa, Italy.
    Systemic lupus erythematosus (SLE) is considered the paradigm of autoimmune diseases (AD), and the murine models are known to be curable by means of allogeneic hematopoietic stem cell transplantation (HSCT). However autologous transplantations were predominantly utilized in the clinic, starting from 1996, and by now well over 150 very severe patients have been transplanted worldwide. Transplant-related mortality (TRM) in 153 cases was 7%, with a wide center effect (from 0-2% to 13%). Read More

    [Kidney diseases in dystrophic epidermolysis bullosa: case report].
    Nephrol Ther 2008 Jun 20;4(3):187-95. Epub 2008 Feb 20.
    Unité de néphrologie-hémodialyse, centre hospitalier d'Annecy, 74000 Annecy, France.
    Dystrophic epidermolysis bullosa is a genetic skin disease of which the recessive subtype also named Hallopeau-Siemens is the most severe. It is due to lack of expression of type VII collagen which is essential for dermal anchoring. Severe obstructive uropathies of the urethral and bladder area may occur during the first years of life, in relation to local bullous activity. Read More

    A case of haemodialysis-associated pseudoporphyria successfully treated with oral N-acetylcysteine.
    Clin Exp Dermatol 2007 Jan;32(1):64-6
    Department of of Dermatology, Belfast City Hospital, Belfast, UK.
    We report a 33-year-old woman with haemodialysis-associated pseudoporphyria successfully responding to treatment with oral N-acetylcysteine. We briefly review the current literature on bullous skin disorders in end-stage renal disease, and compare and contrast the pathogenesis of pseudoporphyria and porphyria cutanea tarda in this context. We also discuss the antioxidant properties and clinical applications of N-acetylcysteine, including the treatment of haemodialysis-associated pseudoporphyria. Read More

    Pyoderma gangrenosum occurring near an arteriovenous dialysis shunt.
    Int J Dermatol 2006 Jul;45(7):851-3
    Walter Reed Army Medical Center, Washington, DC, USA.
    Pyoderma gangrenosum (PG) is an uncommon cutaneous disease of unknown etiology. In 50 percent of affected patients, PG is associated with systemic disease including inflammatory bowel disease, arthritis, and hematologic malignancies.(1) Diagnosis of PG is based on clinical presentation, histopathology and on the exclusion of other diseases that can produce clinically similar lesions, e. Read More

    [Nephrologists and porphyrias].
    G Ital Nefrol 2002 Jul-Aug;19(4):393-412
    Dipartimento di Medicina Interna, Cattedra di Nefrologia dell'Universita' di Torino, Torino.
    As usually occurs for rare diseases, the word "PORPHYRIA" often recalls a confused topic with shaded boundaries, presenting "bullous" skin lesions, rare opportunity of diagnosis in clinical practice, unknown pathogenesis, and almost absent therapeutic options. The goal of this review is to draw attention to this topic, as new diagnostic and therapeutic tools might change the natural history of this disease. Porphyrias are disorders resulting from abnormalities of porphyrin metabolism. Read More

    Cutaneous manifestations of end-stage renal disease.
    J Am Acad Dermatol 2000 Dec;43(6):975-86;quiz 987-90
    Department of Dermatology, Brown University School of Medicine/Rhode Island Hospital, Providence, 02903, USA.
    Examination of the skin and nails can reveal many abnormalities in patients with end-stage renal disease that precede or follow initiation of dialysis treatment or kidney transplantation. This article focuses on specific and nonspecific cutaneous signs of end-stage renal disease, reviewing both banal and life-threatening conditions, including pruritus, perforating disorders, calcifying disorders, and bullous dermatoses. The pathogenesis, clinical findings, histologic findings, differential diagnosis, and treatment of these diseases are discussed. Read More

    Bullous dermatoses in end-stage renal failure: porphyria or pseudoporphyria?
    Am J Kidney Dis 1999 Jul;34(1):155-60
    Renal Section, Imperial College School of Medicine, London, UK.
    Bullous dermatoses (BD) are well recognized in patients with end-stage renal disease (ESRD). It is important to distinguish pseudoporphyria (porphyrin accumulation due to decreased clearance) from true porphyrias, particularly those in which acute neurological attacks may occur. Investigation of the dialysis patient poses practical diagnostic difficulties because urinary porphyrin profiles are not available. Read More

    [Porphyria cutanea tarda in a long-term hemodialysis patient].
    Hautarzt 1996 Dec;47(12):932-5
    Dermatologische Klinik, Albert Szent-Györgyi Medizinischen Universität, Szeged, Ungarn.
    A 61 year old man developed bullous skin lesions typical for porphyria cutanea tarda (PCT) after being on maintenance hemodialysis for 3 years. The porphyrin level in the urine, plasma, erythrocytes and stool supported the diagnosis of porphyria cutanea tarda, a very rare disease in patients with chronic renal failure on hemodialysis. Therapeutic possibilities are reviewed. Read More

    Bullous dermatosis of end-stage renal disease: a possible association between abnormal porphyrin metabolism and aluminium.
    Nephrol Dial Transplant 1996 Sep;11(9):1787-91
    Department of Nephrology, Hasharon Hospital, Petah-Tikva, Israel.
    Background: Bullous dermatosis (BD) is becoming increasingly recognized in patients with end-stage renal disease (ESRD). It is clinically reminiscent of porphyria cutanea tarda, but its detailed pathogenesis remains unclear. Studies have shown increased porphyrin levels in dialysis patients, and this may partly explain the skin lesions and photosensitivity evident in these patients. Read More

    Porphyria cutanea tarda associated with chronic renal disease and hemodialysis.
    Arch Dermatol 1980 Feb;116(2):191-5
    A fourth case of symptomatic porphyria associated with hemodialysis for chronic renal failure is reported. Subepidermal bullous dermatoses of patients who have undergone hemodialysis have not usually been associated with elevated porphyrin levels. However, this patient and three previously reported cases have been found to have abnormal porphyrin study results in association with skin lesions typical for porphyria cutanea tarda, occurring after hemodialysis. Read More

    [Pseudoporphyria cutanea tarda in hemodialysis patients].
    Hautarzt 1979 Mar;30(3):154-7
    A bullous disease very similar to late onset cutaneous porphyria has been found in approximately 16 percent of the patients on intermittend hemodialysis. The bullae occur during the summer and are situated on exposed areas, particularly hands, head and neck. There is skin fragility and the bullae are transient, giving place to erosions and later to atrophic scarring. Read More

    Porphyria cutanea tarda-like syndrome in hemodialyzed patients.
    Nephron 1978 ;20(5):258-66
    Among 500 patients on maintenance hemodialysis, 6 patients (5 young women and a 49-year-old man) developed bullous dermatosis, 2-54 months after initiating dialysis treatment. The skin lesions occurred mainly in sunlight-exposed areas, and 4 out of the 6 patients showed increased cutaneous fragility in response to trauma. Skin biopsy revealed subepidermal blisters for all of them, and skin immunofluorescence studies were negative for 2 patients. Read More

    Porphyria cutanea tarda-like dermatosis by hemodialysis. Ultrastructural study of exposed skin.
    Arch Dermatol Res 1977 Aug;259(2):177-85
    The authors studied by electron microscope, the sun-exposed skin of the back of the hand from three heavily hemodialysed patients with a porphyria cutanea tarda-like bullous skin disease. The vascular impairment, like that of PCT, closely resembles that seen during medicamentous phototoxic processes. The connective tissue is infiltrated by large granulo-filamentous masses and the fibroblasts are secretory in appearance. Read More

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