5 489

Cited 0 times in

Retrospective analysis of 12 Korean patients with paraneoplastic pemphigus

Authors
 Yuri Choi  ;  Kyung-Hwa Nam  ;  Jee-Bum Lee  ;  Jun Young Lee  ;  Chull-Wan IHM  ;  Sang Eun Lee  ;  Sang Ho Oh  ;  Takashi Hashimoto  ;  Soo-Chan Kim 
Citation
 JOURNAL OF DERMATOLOGY, Vol.39(12) : 973-981, 2012 
Journal Title
JOURNAL OF DERMATOLOGY
ISSN
 0385-2407 
Issue Date
2012
MeSH
Adult ; Aged ; Autoantibodies/blood ; Castleman Disease/complications ; Dendritic Cell Sarcoma, Follicular/complications ; Female ; Humans ; Lymphoma, T-Cell, Peripheral/complications ; Male ; Middle Aged ; Paraneoplastic Syndromes/immunology* ; Paraneoplastic Syndromes/pathology* ; Pemphigus/immunology* ; Pemphigus/pathology* ; Plakins/immunology ; Prognosis ; Republic of Korea ; Retrospective Studies ; Survival Rate ; Thymoma/complications ; Thymus Neoplasms/complications ; Young Adult
Keywords
diagnostic criteria ; diversity ; paraneoplastic pemphigus
Abstract
Paraneoplastic pemphigus (PNP) is a rare, life-threatening, autoimmune, mucocutaneous blistering disease associated with neoplasia. Both humoral and cellular immunity are involved in the pathogenesis of PNP. Characteristically, PNP has a diverse spectrum of clinical and immunopathological features. We retrospectively analyzed 12 Korean patients with PNP who were diagnosed between 1993 and 2011. We performed analysis of the clinical features, clinical outcomes, underlying neoplasia, histological features and laboratory findings. All of the patients except one had severe mucosal involvement. Two patients had only mucosal lesions but no cutaneous involvement was observed. Erythema multiforme or lichen planus-like eruptions rather than bullous lesions were more commonly observed skin rashes. The most common histological features were interface dermatitis and apoptotic keratinocytes. There were associated hematological-related neoplasms in 11 patients, with Castleman's disease (n = 4) as the most frequent. Twelve patients were followed for 5-148 months (mean, 43.0). The prognosis depended on the nature of the underlying neoplasm. Six patients died due to respiratory failure (n = 3), postoperative septicemia (n = 1), lymphoma (n = 1) and sarcomatosis (n = 1). The 2-year survival rate was 50.0%, and the median survival period after diagnosis was 21.0 months. Immunoblotting was performed in 12 patients and autoantibodies to plakins were detected in 11 patients. The results of this study demonstrated the clinical, histological and immunological diversity of PNP. Widely accepted diagnostic criteria that account for the diversity of PNP are needed.
Full Text
http://onlinelibrary.wiley.com/doi/10.1111/j.1346-8138.2012.01655.x/abstract
DOI
22938021
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Dermatology (피부과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Soo Chan(김수찬) ORCID logo https://orcid.org/0000-0002-2327-4755
Oh, Sang Ho(오상호) ORCID logo https://orcid.org/0000-0002-4477-1400
Lee, Sang Eun(이상은) ORCID logo https://orcid.org/0000-0003-4720-9955
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/90765
사서에게 알리기
  feedback

qrcode

Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.

Browse

Links