Chronic pemphigoid gestationis: comparative clinical and immunopathological study of 10 patients.
BACKGROUND
Pemphigoid gestationis (PG) is a rare autoimmune bullous disorder occurring during the last trimester of pregnancy and usually regressive within 3 months after delivery. Prolonged forms of the disease lasting more than 6 months after delivery have been reported as chronic PG.


OBJECTIVE
The aim of the present study was to compare the clinical and immunopathological findings between 4 patients presenting a normal regression of the disease after delivery and 6 patients with a chronic course.


METHODS
All patients were evaluated and studied by clinical patterns (age, mucosal and cutaneous involvement, obstetrical history, duration of the blistering disease and response to treatment), by direct and indirect immunofluorescence and Western blot. Eight patients were studied by immunoelectron microscopy (IEM) and 3 patients had an indirect IEM.


RESULTS
Patients with chronic PG were older, had multigravidity, a history of PG during previous pregnancies, widespread cutaneous eruption and mucosal involvement. Subclass analysis of circulating autoantibodies showed an IgG1 anti-BP180 response in all patients except 1 with disease of 7 years' duration. Direct IEM was positive in 6/8 patients showing a labeling of the lamina lucida, and indirect IEM using colloidal gold probes confirmed the localization of the target antigens to the proximal part of the anchoring filaments in the lamina lucida.


CONCLUSIONS
This study suggests that, even in chronic long-lasting PG, IgG1 remains the predominant subtype of IgG. Therefore no biological and predictable marker of chronicity can be ascertained from this series.
Boulinguez
Serge
S
Department of Dermatology, Hôpital Dupuytren, Limoges, France.
Bédane
Christophe
C
Prost
Catherine
C
Bernard
Philippe
P
Labbé
Laurence
L
Bonnetblanc
Jean Marie
JM
D000328:Adult; D001327:Autoimmune Diseases / Q000276:immunology / Q000473:pathology; D015153:Blotting, Western; D002908:Chronic Disease; D003176:Complement C3 / Q000032:analysis; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D016253:Microscopy, Immunoelectron; D009092:Mucous Membrane / Q000473:pathology; D010391:Pemphigoid, Bullous / Q000276:immunology / Q000473:pathology; D011247:Pregnancy; D011248:Pregnancy Complications / Q000276:immunology / Q000473:pathology; D012867:Skin / Q000276:immunology / Q000473:pathology
D003176:Complement C3; D007074:Immunoglobulin G