[Epidermolysis bullosa acquisita of childhood].
BACKGROUND
Epidermolysis bullosa acquisita (EBA) is a subepidermal autoimmune blistering disease characterized immunologically by autoantibodies to type VII collagen. Its occurrence in childhood is rare. Thirty-five cases have been described to date in the literature.


METHODS
We report the case of an 8-year-old girl presenting blistering lesions on the cheeks, extremities and limb extension areas. The diagnosis of EBA was confirmed by histology, direct immunofluorescence of a perilesional skin biopsy specimen, indirect immunofluorescence on salt-split skin substrate and direct electron microscopy. The patient was controlled clinically under treatment with dapsone alone.


CONCLUSIONS
This 36th childhood case of EBA presented typical clinical features, a similar prognosis and comparable treatment response to other paediatric cases. Clinical presentation is inflammatory and affects the face. As in our case, in childhood, prognosis is often better than in adults without the need for immunosuppressive agents.
Bordier-Lamy
F
F
Service de dermatologie, hôpital Robert-Debré, CHU de Reims, avenue du Général-Koenig, 51092 Reims cedex, France. florence.bordier@wanadoo.fr
Eschard
C
C
Coste
M
M
Ploton
D
D
Durlach
A
A
Tabary
T
T
Bernard
P
P
D001323:Autoantibodies / Q000097:blood; D001485:Basement Membrane / Q000276:immunology; D002648:Child; D003622:Dapsone / Q000627:therapeutic use; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D005260:Female; D006801:Humans; D007917:Leprostatic Agents / Q000627:therapeutic use
D001323:Autoantibodies; D007917:Leprostatic Agents; D003622:Dapsone