Epidermolysis bullosa hereditaria letalis: report of a case surviving for two and a half years.
نویسنده
چکیده
Epidermolysis bullosa is a chronic hereditary disease of unknown aetiology involving the skin and mucous membranes. It is characterized by the formation of multiple superficial vesicles and bullae which develop spontaneously or as a result of minimal trauma. The disease may occur in varying grades of severity but there is one form with distinct characteristics which has been classified as epidermolysis bullosa hereditaria letalis. In all other cases it has been uniformly fatal within the first few months of life. The purpose of this paper is to report a patient who has this type of the disease but is still alive at the age of 30 months.
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Abstract Background: Epidermolysis bullosa is an uncommon disease with a wide spectrum of severity. Here we report a patient presenting with unusual symptoms. Case Report: The patient is a 22 years old female with progressive dysphasia and odynophagia to solids and liquids and a history of spontaneously remitting blisters caused after mild trauma from her childhood till she was 13 years old...
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عنوان ژورنال:
- Archives of disease in childhood
دوره 32 163 شماره
صفحات -
تاریخ انتشار 1957