Paraneoplastic pemphigus secondary to fludarabine evolving into unusual oral pemphigus vegetans

A M Powell; S Albert; N Oyama; Y Sakuma-Oyama; B Bhogal; M M Black

doi:10.1111/j.1468-3083.2004.00917.x

Paraneoplastic pemphigus secondary to fludarabine evolving into unusual oral pemphigus vegetans

A M Powell, S Albert, N Oyama, Y Sakuma-Oyama, B Bhogal, M M Black

King's College London

Research output: Contribution to journal › Article › peer-review

28 Citations (Scopus)

Abstract

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 I 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.

Original language	English
Pages (from-to)	360 - 364
Number of pages	5
Journal	Journal of The European Academy of Dermatology and Venereology
Volume	18
Issue number	3
DOIs	https://doi.org/10.1111/j.1468-3083.2004.00917.x
Publication status	Published - May 2004

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

Access to Document

10.1111/j.1468-3083.2004.00917.x

Cite this

@article{9941a49e2afd411e88ea3aeb3a0988f2,

title = "Paraneoplastic pemphigus secondary to fludarabine evolving into unusual oral pemphigus vegetans",

abstract = "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 I 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.",

author = "Powell, {A M} and S Albert and N Oyama and Y Sakuma-Oyama and B Bhogal and Black, {M M}",

year = "2004",

month = may,

doi = "10.1111/j.1468-3083.2004.00917.x",

language = "English",

volume = "18",

pages = "360 -- 364",

journal = "Journal of The European Academy of Dermatology and Venereology",

publisher = "Wiley-Blackwell",

number = "3",

}

TY - JOUR

T1 - Paraneoplastic pemphigus secondary to fludarabine evolving into unusual oral pemphigus vegetans

AU - Powell, A M

AU - Albert, S

AU - Oyama, N

AU - Sakuma-Oyama, Y

AU - Bhogal, B

AU - Black, M M

PY - 2004/5

Y1 - 2004/5

N2 - 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 I 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.

AB - 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 I 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.

UR - http://www.scopus.com/inward/record.url?scp=2442610919&partnerID=8YFLogxK

U2 - 10.1111/j.1468-3083.2004.00917.x

DO - 10.1111/j.1468-3083.2004.00917.x

M3 - Article

VL - 18

SP - 360

EP - 364

JO - Journal of The European Academy of Dermatology and Venereology

JF - Journal of The European Academy of Dermatology and Venereology

IS - 3

ER -

Paraneoplastic pemphigus secondary to fludarabine evolving into unusual oral pemphigus vegetans

Abstract

UN SDGs

Access to Document

Other files and links

Fingerprint

Cite this