Epidermolysis bullosa acquisita and neuroendocrine pancreatic cancer - Coincidence or patho-genetic relationship?
The etiology of epidermolysis bullosa acquisita (EBA) is unknown. EBA may be associated with other autoimmune systemic diseases; it also has been described in connection with different malignant tumors, showing complete remission after successful treatment of the tumor. In such cases, EBA may be regarded as a paraneo-plastic dermatosis. We detected a highly differentiated neuroendocrine pancreatic cancer in a 78-year-old woman with EBA. Even thought her tumor was completely removed and the patient has been disease-free for over seven years, a complete regression of her autoimmune bullous dermatosis could not be induced. By using intravenous immunoglobulins in combination with mycophenolate mofetil, further blister formation could be ameliorated.
Busch
Jan-Ole
JO
Clinic for Dermatology, Venerology and Allergology, University Dermatology Clinic, Kiel, Germany. obusch@dermatology.uni-kiel.de
Sticherling
Michael
M
D000368:Aged; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D000903:Antibiotics, Antineoplastic / Q000008:administration & dosage; D018278:Carcinoma, Neuroendocrine / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D016107:Epidermolysis Bullosa Acquisita / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D020022:Genetic Predisposition to Disease; D006801:Humans; D007136:Immunoglobulins / Q000008:administration & dosage*; D008297:Male; D009173:Mycophenolic Acid / Q000008:administration & dosage*; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*
D000893:Anti-Inflammatory Agents; D000903:Antibiotics, Antineoplastic; D007136:Immunoglobulins; D009173:Mycophenolic Acid