Linear
IgA disease of adults,
chronic bullous disease of childhood, and the rare childhood
cicatricial pemphigoid currently are regarded as separate clinical entities despite their many shared features. All are
sulfone-responsive subepidermal bullous diseases associated with linear
IgA deposition at the basement membrane zone. In this paper we present a long-term study of 25 cases of adult linear
IgA disease, 25 cases of
chronic bullous disease of childhood, and four cases of childhood
cicatricial pemphigoid, which has revealed further similarities among all three groups. The morphology and distribution of the cutaneous and mucosal lesions were similar; mucosal involvement was present in 80% of patients with adult linear
IgA disease, 64% of those with
chronic bullous disease of childhood, and 100% of those with childhood
cicatricial pemphigoid, and ocular
scarring affected patients in all groups. Remission occurred in 64% of those with
chronic bullous disease of childhood (the disease was active in 12% after puberty), 48% of those with adult linear
IgA disease, and in no cases of childhood
cicatricial pemphigoid. HLA B8 and circulating
IgA anti-basement membrane zone antibody were more common in
chronic bullous disease of childhood than adult linear
IgA disease. There were no absolute differences among the three groups, and we suggest that adult linear
IgA disease, chronic bullous disease of childhood, and childhood
cicatricial pemphigoid are the same disease, with childhood
cicatricial pemphigoid being a more severe form of
chronic bullous disease of childhood.