Paraneoplastic pemphigus secondary to fludarabine evolving into unusual oral pemphigus vegetans.
We report a patient with chronic lymphocytic leukaemia who developed paraneoplastic pemphigus (PNP) soon after the initiation of fludarabine therapy. He presented with severe oral and cutaneous erosions. Initially, he had high titres of circulating autoantibodies as detected by indirect immunofluorescence (IF) on multiple epithelial substrates (normal human skin, monkey oesophagus, and rat bladder) and by desmoglein 1 and 3 enzyme-linked immunosorbent assays (ELISAs). His oral erosions have subsequently progressed into unusual hyperplastic papillomatous lesions affecting the inner aspect of lips and buccal mucosae, histologically consistent with pemphigus vegetans. Desmoglein 1 antibodies and IF on rat bladder substrate have become negative after 18 months of therapy. Several agents had been initiated to bring the disease under control originally, but a partial remission was achieved and maintained with mycophenolate mofetil and low-dose prednisolone.
Powell
A M
AM
Immunofluorescence Department, St John's Institute of Dermatology, St Thomas' Hospital, Lambeth Palace Road, London SE1 7EH, UK. amipowell@doctors.org.uk
Albert
S
S
Oyama
N
N
Sakuma-Oyama
Y
Y
Bhogal
B
B
Black
M M
MM
D000368:Aged; D001707:Biopsy, Needle; D019084:Fluorescent Antibody Technique, Indirect; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000175:diagnosis / Q000188:drug therapy; D008297:Male; D009173:Mycophenolic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D010257:Paraneoplastic Syndromes / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D010392:Pemphigus / Q000139:chemically induced* / Q000188:drug therapy / Q000473:pathology; D018570:Risk Assessment; D012720:Severity of Illness Index; D013280:Stomatitis / Q000139:chemically induced* / Q000175:diagnosis / Q000188:drug therapy; D016896:Treatment Outcome; D014740:Vidarabine / Q000009:adverse effects* / Q000031:analogs & derivatives* / Q000627:therapeutic use
D014740:Vidarabine; D009173:Mycophenolic Acid; C024352:fludarabine