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Bullous disease child

Sayyeda Ghazala Irfan Kazi, Emaduddin Siddiqui, Irfan Habib, Saadia Tabassum, Badar Afzal, Irum Qamar Khan
Neonatal Purpura Fulminans is a rare and fatal disorder associated with perivascular haemorrhage and disseminated intravascular coagulation. Early clinical recognition, timely investigation and treatment is utmost important. A 6 days old baby boy was brought to emergency with blackish ulcers all over the body. Initially these were over the feet and scalp but later appeared on the abdomen. On examination, child was vitally stable, mildly icteric and had multiple erythematous large bullous blackish lesions on scalp, lower abdomen, perineum, back and soles...
March 2018: JPMA. the Journal of the Pakistan Medical Association
Georgi Tchernev, Ivan Terziev
For the first time in the world medical literature, we describe a rare form of cutaneous dermatophytosis - a bullous form of Tinea incognito, classified by clinical picture, histopathological findings and an isolated infectious agent from the microbiological culture. After a thorough review of Medline/PubMed's relevant literature, we could not find similar cases of patients with Tinea incognito who are clinically presented with bullous lesions at the same time. Local application of corticosteroids in infants with unknown lesions may lead to progression of the underlying disease and may cause some serious problems in differential diagnosis aspect, while the clinical expression remains completely masked...
February 15, 2018: Open Access Macedonian Journal of Medical Sciences
Rosalind Ashton, Karen W Wong, Miriam Weinstein
BACKGROUND: Erythema multiforme (EM) is a mucocutaneous disease most often associated with preceding herpes simplex virus or Mycoplasma pneumoniae infection. It is characterized by targetoid lesions occurring on the limbs and head and neck. Mucosal involvement can be extensive. OBJECTIVE: We report a case of lip adhesion as a complication of EM in a child who required corrective surgery and review similar oral commissure complications. METHODS: We completed a review of the literature for similar cases using PubMed and Medline...
February 1, 2018: Journal of Cutaneous Medicine and Surgery
N Ilham El Makrini, H Zaouri, O El Anzi, M Meziane, N Ismaili, L Benzekri, K Senouci, B Hassam
Bullous pemphigoid (BP) is an acquired autoimmune disease that mainly affects the elderly. It is very rare in children. We report a sudden polymorphic case of BP in a 12-year-old child.
April 17, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Joanne R Chalmers, Fenella Wojnarowska, Gudula Kirtschig, James Mason, Margaret Childs, Diane Whitham, Karen Harman, Anna Chapman, Shernaz Walton, Enno Schmidt, Thomas R Godec, Andrew J Nunn, Hywel C Williams
BACKGROUND: Bullous pemphigoid (BP) is an autoimmune blistering skin disorder with increased morbidity and mortality in the elderly. OBJECTIVES: To evaluate the effectiveness, safety and cost-effectiveness of a strategy of initiating BP treatment with oral doxycycline or oral prednisolone. We hypothesised that starting treatment with doxycycline gives acceptable short-term blister control while conferring long-term safety advantages over starting treatment with oral prednisolone...
March 2017: Health Technology Assessment: HTA
Hywel C Williams, Fenella Wojnarowska, Gudula Kirtschig, James Mason, Thomas R Godec, Enno Schmidt, Joanne R Chalmers, Margaret Childs, Shernaz Walton, Karen Harman, Anna Chapman, Diane Whitham, Andrew J Nunn
BACKGROUND: Bullous pemphigoid is a blistering skin disorder with increased mortality. We tested whether a strategy of starting treatment with doxycycline gives acceptable short-term blister control while conferring long-term safety advantages over starting treatment with oral corticosteroids. METHODS: We did a pragmatic, multicentre, parallel-group randomised controlled trial of adults with bullous pemphigoid (three or more blisters at two or more sites and linear basement membrane IgG or C3)...
April 22, 2017: Lancet
Wallace Austin Smith, Austin Cope, Martin Fernandez, Palak Parekh
Epidermolytic Ichthyosis (EI) is a rare autosomal dominant genodermatosis. Although an inherited disorder, 50% of cases represent novel mutations. This disorder presents as a bullous disease in newborns progressing to a lifelong ichthyotic skin disorder.  Other manifestations include palmoplantar keratoderma (PPK).  EI results from mutations in the keratin 1 and keratin 10 genes. Phenotypic variability is seen in affected individuals based on the genotypic mutation.  We present a mother and her newborn son with EI and prominent PPK in the mother, which also developed in the child at a few months of age...
April 18, 2016: Dermatology Online Journal
J Heichel, T Bredehorn-Mayr, H-G Struck
BACKGROUND: Pathologies of the lacrimal duct system show a frequent occurrence in paediatric ophthalmology. Mostly, the connection between the nasolacrimal duct and the nose fails to open but also combined diseases or congenital anomalies may be the reason. Because of complications, the chance for healing after a conservative therapeutic approach decreases and surgical intervention is necessary. PATIENTS AND METHODS: The opportunity for transcanalicular endoscopy of the lacrimal duct system in children is shown by the presentation of three different case reports...
July 2015: Klinische Monatsblätter Für Augenheilkunde
Noopur Gupta, Praveen Vashist, Radhika Tandon, Sanjeev K Gupta, Sadanand Dwivedi, Kalaivani Mani
OBJECTIVE: The present population-based study was undertaken to estimate the prevalence, determinants and causes of corneal morbidity and blindness in a rural North Indian population. DESIGN: Population-based study in India with 12 899 participants of all ages. METHODS: Participants were recruited from 25 village clusters of district Gurgaon, Haryana, India using random cluster sampling strategy. All individuals were examined in detail with a portable slit lamp for evidence of any corneal disease during the door-to-door examination...
February 2015: British Journal of Ophthalmology
Asha C Bowen, Steven Y C Tong, Ross M Andrews, Irene M O'Meara, Malcolm I McDonald, Mark D Chatfield, Bart J Currie, Jonathan R Carapetis
BACKGROUND: Impetigo affects more than 110 million children worldwide at any one time. The major burden of disease is in developing and tropical settings where topical antibiotics are impractical and lead to rapid emergence of antimicrobial resistance. Few trials of systemic antibiotics are available to guide management of extensive impetigo. As such, we aimed to compare short-course oral co-trimoxazole with standard treatment with intramuscular benzathine benzylpenicillin in children with impetigo in a highly endemic setting...
December 13, 2014: Lancet
Kwei-Lan Liu, Jui-Lung Shen, Chii-Shuenn Yang, Yi-Ju Chen
Bullous systemic lupus erythematosus is a subepidermal blistering disorder that primarily affects young women and only rarely occurs in children. We report a case of bullous systemic lupus erythematosus refractory to corticosteroid therapy in a 12-year-old boy who was successfully treated with oral dapsone.
July 2014: Pediatric Dermatology
S Kourouma, A Sangaré, M Kaloga, I Kouassi, E Ecra, I Gbery, C Ahogo, A Kouassi, K Kassi, B Camara
BACKGROUND: Cutaneous bullous adverse drug eruptions are a dreaded complication of drug use. OBJECTIVE: The purpose of this study was to document the epidemiological features, outcomes, and causes of these reactions, in particular, Stevens-Johnson syndrome and toxic epidermal necrolysis (Lyell disease), in a teaching hospital in Abidjan (Côte d'Ivoire). PATIENTS AND METHODS: A retrospective study reviewed the records of severe cutaneous drug reactions in patients managed in the dermatology department of the University Hospital of Treichville (Abidjan) over a period of ten years (from September 2000 through August 2010)...
January 2014: Médecine et Santé Tropicales
Sara Missaglia, Eugenia Ribeiro Valadares, Laura Moro, Eleonora Druve Tavares Faguntes, Raquel Quintão Roque, Bruno Giardina, Daniela Tavian
BACKGROUND: α/β-hydrolase domain-containing protein 5 (ABHD5) plays an important role in the triacylglycerols (TAG) hydrolysis. Indeed, ABHD5 is the co-activator of adipose triglyceride lipase (ATGL), that catalyses the initial step of TAG hydrolysis. Mutations in ABHD5 gene are associated with the onset of Chanarin-Dorfman syndrome (CDS), a rare autosomal recessive lipid storage disorder, characterized by non-bullous congenital ichthyosiform erythroderma (NCIE), hepatomegaly and liver steatosis...
2014: BMC Medical Genetics
Rajesh Verma, Biju Vasudevan, Pragasam Vijendran, Debdeep Mitra
No abstract text is available yet for this article.
June 2014: International Journal of Dermatology
Zhi-Dan Fan, Xiao-Qing Qian, Hai-Guo Yu
BACKGROUND: Stevens-Johnson syndrome (SJS) is a severe skin and mucosal bullous disease. When complicated with Hemophagocytic lymphohistiocytosis (HLH), the condition is especially life-threatening. CASE PRESENTATION: Here we report the case of a 4-year-old boy suffering from SJS with extensive erythema multiforme and bulla. Despite active intervention and supportive care, the boy experienced increased skin lesions and a higher fever. Meanwhile, decreases in white blood cell count and hemoglobin were observed...
2014: BMC Pediatrics
Douglas J Coster, Marie T Lowe, Miriam C Keane, Keryn A Williams
OBJECTIVES: To investigate changing patterns of practice of keratoplasty in Australia, graft survival, visual outcomes, the influence of experience, and the surgeon learning curve for endothelial keratoplasty. DESIGN: Observational, prospective cohort study. PARTICIPANTS: From a long-standing national corneal transplantation register, 13 920 penetrating keratoplasties, 858 deep anterior lamellar keratoplasties (DALKs), and 2287 endokeratoplasties performed between January 1996 and February 2013 were identified...
May 2014: Ophthalmology
Johnson C H Tan, Simon P Holland, Paul J Dubord, Gregory Moloney, Martin McCarthy, Sonia N Yeung
PURPOSE: The aim of this study was to report the evolving indications for keratoplasty and the shift in the type of keratoplasty performed in British Columbia, Canada, over a 10-year period from 2002 to 2011. METHODS: This was a retrospective database review of all the records of corneal transplant tissues at the Eye Bank of British Columbia, Canada, from January 2002 to December 2011. The patient demographics, indications, and types of transplant performed were analyzed...
March 2014: Cornea
Jessica H Mouledoux, Erin L Albers, Zengqi Lu, Benjamin R Saville, Daniel J Moore, Debra A Dodd
Autoimmune and allergic diseases cause morbidity and diminished quality of life in pediatric organ transplant recipients. We hypothesize that younger age at transplantation and immunosuppression regimen play a role in the development of immune-mediated disease following heart transplant. A single institution retrospective review identified all patients undergoing heart transplant at ≤18 yr of age from 1987 to 2010 who survived ≥1 yr. Using medical record and database review, patients were evaluated for development of autoimmune or severe allergic disease...
March 2014: Pediatric Transplantation
Sonali Sharma, Sumit Bedi
Epidermolysis bullosa (EB) is a genetic disease associated with fragility and bullous lesions of the skin and mucous membranes. There are various patterns of inheritance and histopathology. The disease is associated with systemic and oral manifestations, among which may be dental decay necessitating oral rehabilitation. The aim of this article is to present the course of the condition in a child with dystrophic EB and also to report an association between EB, hypodontia, and supernumerary teeth which has not been reported earlier in literature...
October 2013: Indian Dermatology Online Journal
Beatriz Cavalcanti de Souza, Nádire Cristina Freire Pontes Fregonesi, Antônio José Tebcherani, Ana Paula Galli Sanchez, Valéria Aoki, Juliana Christien Fernandes
Linear immunoglobulin A dermatosis is a rare autoimmune bullous disease, but the most common autoimmune bullous dermatosis in children. We report a typical exuberant case of linear IgA dermatosis in a ten-month old child, who showed good response to treatment with corticosteroids and dapsone.
November 2013: Anais Brasileiros de Dermatologia
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