[Acquired epidermolysis bullosa with a highly varied clinical picture and successful treatment with mycophenolate mofetil].
A 60-year-old woman with epidermolysis bullosa acquisita (EBA) presented with a highly variable clinical picture. First she developed oral erosions and ulcers covered with shaggy epithelial rests. Later she developed pustular and target lesions as well as tense cutaneous blisters. Clinically the alterations were strongly suggestive of aphthae or a pustular dermatosis. Histological and immunological examinations revealed subepidermal blister formation, neutrophilic infiltration, linear deposition of IgG and C3 at the basement membrane in the direct immunofluorescence and the evidence of circulating IgG-antibodies against basement membrane (monkey esophagus) in the indirect immunofluorescence. Thus our working diagnosis was a pustular or nodular form of bullous pemphigoid, as a linear IgA dermatosis was excluded. Indirect immunofluorescence using NaCl-separated primate skin localized the basement membrane antibodies in the base of the artificial blister, while immunoblotting of dermal extracts disclosed binding of serum antibodies to a 290-kDa protein. Bullous systemic lupus erythematosus was excluded, leading to the definitive diagnosis of EBA. The clinical variability of this disease is well known; the polymorphic picture in our patient with erosions, pustules, target lesions and blisters is unique in the literature. The similarity in histology and immunofluorescence of the clinically quite different lesions is another unexpected event. Also the speedy response to therapy with the immunosuppressive agent mycophenolate mofetil has only been described once before. The patient remains in remission after 6 months. Because of the poor response to therapy in most cases, the significant skin lesions are a source of physical as well as emotional distress. Additional complications include stenosis of trachea or bronchi injure.
Trebing
D
D
Hautklinik und Immunologisches Zentrum, Städtisches Klinikum Dessau.
Ziemer
A
A
D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D003176:Complement C3 / Q000032:analysis; D003879:Dermatologic Agents / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis* / Q000188:drug therapy* / Q000276:immunology; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D015151:Immunoblotting; D007136:Immunoglobulins / Q000032:analysis; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use*
D000894:Anti-Inflammatory Agents, Non-Steroidal; D003176:Complement C3; D003879:Dermatologic Agents; D007136:Immunoglobulins; D007166:Immunosuppressive Agents; D009173:Mycophenolic Acid