Generalized atrophic benign epidermolysis bullosa: either 180-kd bullous pemphigoid antigen or laminin-5 deficiency
MF Jonkman, MCJM De Jong, K Heeres… - Archives of …, 1996 - jamanetwork.com
Background: Generalized atrophic benign epidermolysis bullosa (GABEB) is a form of
nonlethal junctional epidermolysis bullosa, clinically characterized by generalized blistering
after birth, atrophic healing, and incomplete universal atrophic alopecia with onset in
childhood. Recently, we discovered a deficiency of the 180-kd bullous pemphigoid antigen
(BP180) and a reduced amount of BP180 messenger RNA in three patients with GABEB. It is
not yet clear, however, whether GABEB is invariably caused by BP180 deficiency. Results …
nonlethal junctional epidermolysis bullosa, clinically characterized by generalized blistering
after birth, atrophic healing, and incomplete universal atrophic alopecia with onset in
childhood. Recently, we discovered a deficiency of the 180-kd bullous pemphigoid antigen
(BP180) and a reduced amount of BP180 messenger RNA in three patients with GABEB. It is
not yet clear, however, whether GABEB is invariably caused by BP180 deficiency. Results …
