TY - JOUR
T1 - A nonfatal case and 2 fatal cases of paraneoplastic pemphigus
T2 - Can a complement indirect immunofluorescent test help to identify fatal "group A" paraneoplastic pemphigus cases?
AU - Beutner, Ernst H.
AU - Pelton, Stephen
AU - Hashimoto, Takashi
AU - Xu, Yuelin
AU - Plunkett, Richard W.
AU - Korman, Neil J.
AU - Helm, Thomas N.
AU - Jablonska, Stefania
N1 - Funding Information:
From the Departments of Microbiology and Dermatology, Univer-sity at Buffalo, State University of New Yorka; Barrington Park Der-matology, Rochesterb; Department of Dermatology, Keio Univer-sity, Tokyoc; GG Messenger MD, PC, Lansingd; Department of Dermatology, Case Western Reserve University, Clevelande; De-partment of Dermatology, University at Buffalo, State University of New Yorkf; Klinika Dermatologiczna, Akademia Medyczna, Warsaw.g Funding sources: National Institutes of Health grant RR0016 to the Oregon Primate Center and Beutner Labs, Inc. Conflict of interest: None. This report does not imply that findings and views expressed are endorsed by the institutions with which the authors and mem-bers of the Advisory Committee of the Association for Proficiency in Skin Immunopathology (APSIP) are affiliated. Accepted for publication December 31, 2001. *Members of the Advisory Committee of the Association for Profi-ciency in Skin Immunopathology are listed at the end of the article. Reprint requests: Ernst H. Beutner, PhD, Beutner Laboratories, PO Box 26, Buffalo, NY 14215. Copyright © 2002 by the American Academy of Dermatology, Inc. 0190-9622/2002/$35.00 + 0 16/1/123486 doi:10.1067/mjd.2002.123486
PY - 2002/12/1
Y1 - 2002/12/1
N2 - We studied 3 recent cases of paraneoplastic pemphigus (PNP) in detail. Two patients died despite concerted management efforts. One patient received no treatment after the appearance of PNP and recovered completely from both PNP and lymphoma. Multiple serum studies of these 3 patients plus 9 other proven PNP cases revealed that 8 of 9 fatal PNP cases (referred to here as "group A") had distinctive cell surface antibodies detected by complement indirect immunofluorescent (CIIF) tests on monkey esophagus sections. By contrast, none of the sera from 3 patients with PNP who experienced long-term survival (referred to here as "group B") and none of 20 pemphigus vulgaris or 10 pemphigus foliaceus control sera revealed similar beaded cell surface CIIF reaction patterns, a difference that is statistically significant (P < .0001). Cell surface CIIF reaction patterns of group A PNP antibodies resemble the pattern of pemphigus antibody reactions in indirect immunofluorescent tests on the same substrate; however, the latter tend to be thinner and more linear, whereas the cell surface CIIF pattern tends to be more beaded, suggesting possible desmosomal reactions. We believe this test is useful in identifying an aggressive group A form of PNP.
AB - We studied 3 recent cases of paraneoplastic pemphigus (PNP) in detail. Two patients died despite concerted management efforts. One patient received no treatment after the appearance of PNP and recovered completely from both PNP and lymphoma. Multiple serum studies of these 3 patients plus 9 other proven PNP cases revealed that 8 of 9 fatal PNP cases (referred to here as "group A") had distinctive cell surface antibodies detected by complement indirect immunofluorescent (CIIF) tests on monkey esophagus sections. By contrast, none of the sera from 3 patients with PNP who experienced long-term survival (referred to here as "group B") and none of 20 pemphigus vulgaris or 10 pemphigus foliaceus control sera revealed similar beaded cell surface CIIF reaction patterns, a difference that is statistically significant (P < .0001). Cell surface CIIF reaction patterns of group A PNP antibodies resemble the pattern of pemphigus antibody reactions in indirect immunofluorescent tests on the same substrate; however, the latter tend to be thinner and more linear, whereas the cell surface CIIF pattern tends to be more beaded, suggesting possible desmosomal reactions. We believe this test is useful in identifying an aggressive group A form of PNP.
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U2 - 10.1067/mjd.2002.123486
DO - 10.1067/mjd.2002.123486
M3 - Article
C2 - 12451367
AN - SCOPUS:0036895354
SN - 0190-9622
VL - 47
SP - 841
EP - 851
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
IS - 6
ER -