Childhood epidermolysis bullosa acquisita. Report of three cases and review of literature.
Epidermolysis bullosa acquisita is an acquired subepidermal blistering disease with variable clinical, pathologic, and immunologic features. The disease has been reported infrequently in adults and only rarely in children. We describe three new cases of childhood epidermolysis bullosa acquisita, review three previously reported cases, and contrast the features of the disease in children with those in adults. The results suggest that both children and adults with epidermolysis bullosa acquisita have variable clinical and pathologic features that may mimic other bullous diseases. Epidermolysis bullosa acquisita is characterized by a chronic course, poor response to therapy, and occasional clinical remissions.
Arpey
C J
CJ
Department of Dermatology, University Hospitals of Cleveland, Case Western Reserve University School of Medicine, OH 44106.
Elewski
B E
BE
Moritz
D K
DK
Gammon
W R
WR
D000293:Adolescent; D001323:Autoantibodies / Q000032:analysis; D001485:Basement Membrane / Q000276:immunology / Q000648:ultrastructure; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D003176:Complement C3 / Q000032:analysis; D003622:Dapsone / Q000008:administration & dosage / Q000627:therapeutic use; D003937:Diagnosis, Differential; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007223:Infant; D008297:Male; D011241:Prednisone / Q000008:administration & dosage / Q000627:therapeutic use
D001323:Autoantibodies; D003176:Complement C3; D007074:Immunoglobulin G; D003622:Dapsone; D011241:Prednisone