[Successful treatment of subcorneal pustular type of IgA pemphigus].
IgA pemphigus is a rare autoimmune intraepidermal blistering disease characterized by in vivo bound, and circulating IgA autoantibodies that target cell surface components (cadherins) of epidermal keratinocytes. The average age of onset is approximately 50 years. Histological hallmarks of IgA pemphigus are epidermal acantholysis, subcorneal or intraepidermal pustulosis, and neutrophilic infiltration. The main clinical characteristics are erythematous skin lesions with vesiculopustules, erosions, crusts and desquamation, favoring the trunk, groins, auxiliaries, and proximal extremities. The drug of choice for treating IgA pemphigus is diaminodiphenylsulfone. In this case study, we describe a patient who suffered from IgA pemphigus and responded well to diaminodiphenylsulfone.
Raap
U
U
Klinik und Poliklinik für Dermatologie und Venerologie der Medizinischen Hochschule Hannover. mail@ulrike-raap.de
Völker
B
B
Petering
H
H
Hashimoto
T
T
Zillikens
D
D
Kapp
A
A
Werfel
T
T
D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D003316:Corneal Diseases / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology / Q000473:pathology*; D003622:Dapsone / Q000627:therapeutic use*; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000150:complications / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology*; D020127:Recovery of Function; D016896:Treatment Outcome
D000894:Anti-Inflammatory Agents, Non-Steroidal; D007070:Immunoglobulin A; D003622:Dapsone