[Linear IgA bullous dermatosis in children with autoantibodies against 180 kDa pemphigoid antigen].
BACKGROUND
Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal blistering disease defined on the basis of direct immunofluorescence findings.


METHODS
An 18 month-old girl suffering from LABD was studied by indirect immunofluorescence on salt-split skin and by Western blot in an attempt to characterize the involved autoantigen. Direct immunofluorescence showed an exclusive linear IgA deposit at the dermal-epidermal junction. Indirect immunofluorescence revealed circulating autoantibodies that reacted with the epidermal side of salt-split skin; they reacted by Western blot with a 180 kDa epidermal antigen, as in bullous pemphigoid.


CONCLUSIONS
This dermatosis fulfilling the clinical features and direct immunofluorescence criteria for childhood LABD seems to represent a case of IgA bullous pemphigoid. It further underscores the nosologic heterogeneity of LABD, which probably includes, apart from bullous pemphigoid, epidermolysis bullosa acquisita and cicatricial pemphigoid.
Bérard
F
F
Clinique dermatologique, hôpital Edouard-Herriot, Lyon, France.
Kanitakis
J
J
Di Maio
M
M
Ghohestani
R
R
Hermier
C
C
David
L
L
Claudy
A
A
Faure
M
M
D001323:Autoantibodies / Q000032:analysis*; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis*; D007223:Infant; D008970:Molecular Weight; D010391:Pemphigoid, Bullous / Q000276:immunology*; D012872:Skin Diseases, Vesiculobullous / Q000276:immunology*
D001323:Autoantibodies; D007070:Immunoglobulin A
Dermatose bulleuse à IgA linéaire de l'enfant avec autoanticorps dirigés contre l'antigène 180 kDa de la pemphigoïde.