scholarly journals Hereditary epidermolysis bullosa: A case series

Author(s):  
S. Guillen-Climent ◽  
L. Fernández García ◽  
A. García-Vázquez ◽  
J.M. Martín
2019 ◽  
Vol 18 (1) ◽  
pp. 56-64
Author(s):  
Nikolay N. Murashkin ◽  
Leonid A. Opryatin ◽  
Alexander I. Materikin ◽  
Eduard T. Ambarchyan ◽  
Roman V. Epishev ◽  
...  

Background. Epidermolysis bullosa acquisita (EBA) is chronic disease accompanied with subepidermal blistering on skin and mucous membranes as a result of autoimmune aggression to type VII collagen. EBA diagnostics in children is complicated due to similarity of clinical presentation with other bullous dermatosis in children.Clinical Case Description. The description of three clinical cases of EBA in children is provided. It is shown that for establishing the diagnosis it is necessary to estimate clinical evidence and to define the depth of blisters according to the results of histological examination of skin biopsy sample. Determination of IgG deposition positions relatively to the skin basal membrane due to performed indirect immunofluorescence test helps us to establish final diagnosis and specify patient management. Medical drug Dapsone was used in children with EBA, it has shown to be effective and safe to use as the first-line drug in management of such patients.Conclusion. The algorithm for EBA differential diagnosis with other bullous dermatosis in children is provided. Successful results of medical treatment are described. 


PEDIATRICS ◽  
1986 ◽  
Vol 78 (1) ◽  
pp. 172-174
Author(s):  
MARGARET A. KENNA ◽  
SYLVAN E. STOOL ◽  
SUSAN B. MALLORY

Epidermolysis bullosa is a rare genetically determined, dermatologic disease in which minor trauma causes blister formation.1 A new variant of hereditary epidermolysis bullosa, generalized atrophic benign epidermolysis bullosa, junctional form, has been recently reported.2 Airway involvement has not been a notable feature of this disease. We report the first case of an infant having benign junctional epidermolysis bullosa with laryngeal involvement. CASE REPORT An 11-month-old white boy with known junctional epidermolysis bullosa and mild stridor since birth was referred by his dermatologist for increasing stridor of 24 hours duration. He was initially thought to have croup; however, conservative treatment with mist and racemic epinephrine did not improve his symptoms.


Author(s):  
Delphine Darbord ◽  
Geoffroy Hickman ◽  
Nathalie Pironon ◽  
Claire Barbieux ◽  
Mathilde Bonnet‐des‐Claustres ◽  
...  

1996 ◽  
Vol 34 (4) ◽  
pp. 271-279 ◽  
Author(s):  
J.T. Wright ◽  
K.I. Hall ◽  
T.G. Deaton ◽  
J.-D. Fine

1985 ◽  
Vol 13 (2) ◽  
pp. 252-278 ◽  
Author(s):  
Richard M. Haber ◽  
Wedad Hanna ◽  
Colin A. Ramsay ◽  
Lionel B.H. Boxall

2014 ◽  
Vol 15 (1) ◽  
pp. 1-6 ◽  
Author(s):  
Chao-Kai Hsu ◽  
Sheng-Pei Wang ◽  
Julia Yu-Yun Lee ◽  
John A. McGrath

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