Nonbullous pemphigoid: prodrome of bullous pemphigoid or a distinct pemphigoid variant?
We describe two patients with pruritic, mainly urticarial or eczematous lesions associated with peripheral blood eosinophilia. No vesicles or blisters developed in either patient throughout the course of the disease (29 and 38 months, respectively). To characterize the clinicopathologic features of these patients we performed histopathologic studies, direct and indirect immunofluorescence, immunoelectron microscopy (patient 2), and immunoprecipitation of both patients' serum. Histopathologic examination revealed a moderate eosinophilic infiltrate partly arranged along the basement membrane zone and focally invading the epidermis. Linear deposits of immunoglobulin and C3 along the dermoepidermal junction were localized within the lamina lucida and over the hemidesmosomal plaques. Immunoprecipitation revealed the presence of circulating autoantibodies against the 230 kd bullous pemphigoid antigen. These findings suggest that our patients had a distinct, nonbullous variant of the pemphigoid spectrum.
Strohal
R
R
Department of Dermatology I, University of Vienna School of Medicine, Austria.
Rappersberger
K
K
Pehamberger
H
H
Wolff
K
K
D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000032:analysis; D050577:Complement C3-C5 Convertases / Q000032:analysis; D004485:Eczema / Q000175:diagnosis / Q000276:immunology; D004802:Eosinophilia / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D004817:Epidermis / Q000473:pathology / Q000648:ultrastructure; D005455:Fluorescent Antibody Technique; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D008297:Male; D016253:Microscopy, Immunoelectron; D008875:Middle Aged; D010391:Pemphigoid, Bullous / Q000145:classification / Q000175:diagnosis* / Q000276:immunology; D011537:Pruritus / Q000175:diagnosis / Q000276:immunology; D014581:Urticaria / Q000175:diagnosis / Q000276:immunology
D001323:Autoantibodies; D007074:Immunoglobulin G; D050577:Complement C3-C5 Convertases