Epidermolysis bullosa and Kindler syndrome - where is the border in oral manifestaions
نویسندگان
چکیده
منابع مشابه
Cutaneous and Laryngeal Squamous Cell Carcinoma in Mixed Epidermolysis Bullosa, Kindler Syndrome
Kindler syndrome is a rare autosomal recessive genodermatosis characterized by trauma-induced acral blisters in infancy and childhood, photosensitivity, and progressive poikiloderma. Other clinical features include chronic erosive gingivitis, dysphagia, esophageal and urethral strictures, ectropion, and an increased risk of mucocutaneous squamous cell carcinoma. We describe a patient with Kindl...
متن کاملEpidemiology of epidermolysis bullosa in the antipodes: the Australasian Epidermolysis Bullosa Registry with a focus on Herlitz junctional epidermolysis bullosa.
OBJECTIVE To present epidemiologic and clinical data from the Australasian Epidermolysis Bullosa (EB) Registry, the first orphan disease registry in Australia. DESIGN Observational study (cross-sectional and longitudinal). SETTING Australian private dermatology practice, inpatient ward, and outpatient clinic. PATIENTS Systematic case finding of patients with EB simplex, junctional EB (JEB...
متن کاملEpidermolysis bullosa in the newborn.
Epidermolysis bullosa is an intractable skin condition characterized by the development of vesicles and bullae spontaneously or as a result of minimal trauma. It is usually hereditary and appears to be due to a congenital defect of skin structure. It has been divided into a simple or non-scarring type inherited as a Mendelian dominant trait and a dystrophic or scarring variety of greater severi...
متن کاملThe eye in epidermolysis bullosa.
AIMS To describe the ophthalmic findings in a large cohort of epidermolysis bullosa (EB) patients managed in one large specialist centre. METHODS A case note review of consecutive patients seen at Great Ormond Street Children's Hospital. Data on the dermatological disease, ophthalmic history, and examination were collected and coded onto a data sheet. RESULTS 181 patients: 50 (28%) simplex ...
متن کاملEpidermolysis bullosa and cancer.
Epidermolysis bullosa (EB) encompasses a group of inherited blistering skin disorders classified into three main subtypes of simplex, junctional and dystrophic. In recent years there have been substantial advances in our understanding of the molecular basis of these conditions and in the management of such patients. In spite of this progress, squamous cell carcinoma (SCC) is still a major cause...
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ژورنال
عنوان ژورنال: Scripta Scientifica Medicinae Dentalis
سال: 2015
ISSN: 2367-7244,2367-7236
DOI: 10.14748/.v1i1.591