TY - JOUR
T1 - Exacerbation of paraneoplastic pemphigus by cyclophosphamide treatment
T2 - Detection of novel autoantigens and bronchial autoantibodies
AU - Preisz, K.
AU - Horváth, A.
AU - Sárdy, M.
AU - Somlai, B.
AU - Hársing, J.
AU - Amagai, M.
AU - Hashimoto, T.
AU - Nagata, Y.
AU - Fekete, S.
AU - Kárpáti, S.
PY - 2004/5/1
Y1 - 2004/5/1
N2 - A 48-year-old woman with a follicular, grade III, B-cell non-Hodgkin lymphoma developed clinical, immunopathological and histological features of paraneoplastic pemphigus. The skin symptoms flared after repeated cyclophosphamide infusions, and were preceded and accompanied by a progressive dyspnoea. Although the skin and oral mucosal disease went into remission with high-dose steroid and intravenous immunoglobulin therapy, the severe alveolitis led to death. Immunoblotting of human epidermal extracts showed that the patient's serum IgG reacted with the 210-kDa envoplakin, 190-kDa periplakin, as well as the recombinant protein of BP180 NC16a domain, IgG and IgA enzyme-linked immunosorbent assays for desmoglein 3 were positive, too. Indirect immunofluorescence studies on COS-7 cells transiently transfected with desmocollin 1-3 cDNAs showed that the patient's serum contained IgG and IgA antibodies to desmocollin 3 as well as IgG antibodies to desmocollin 2. Serum IgG and IgA strongly stained rat bronchial epithelium, corresponding to autoantibodies possibly involved in the pathomechanism of the severe lung disease. In this case, which was characterized by a mixed IgA/IgG antibody panel displaying known and unique antigenicity, the serious episodes of paraneoplastic pemphigus flared after cyclophosphamide treatment.
AB - A 48-year-old woman with a follicular, grade III, B-cell non-Hodgkin lymphoma developed clinical, immunopathological and histological features of paraneoplastic pemphigus. The skin symptoms flared after repeated cyclophosphamide infusions, and were preceded and accompanied by a progressive dyspnoea. Although the skin and oral mucosal disease went into remission with high-dose steroid and intravenous immunoglobulin therapy, the severe alveolitis led to death. Immunoblotting of human epidermal extracts showed that the patient's serum IgG reacted with the 210-kDa envoplakin, 190-kDa periplakin, as well as the recombinant protein of BP180 NC16a domain, IgG and IgA enzyme-linked immunosorbent assays for desmoglein 3 were positive, too. Indirect immunofluorescence studies on COS-7 cells transiently transfected with desmocollin 1-3 cDNAs showed that the patient's serum contained IgG and IgA antibodies to desmocollin 3 as well as IgG antibodies to desmocollin 2. Serum IgG and IgA strongly stained rat bronchial epithelium, corresponding to autoantibodies possibly involved in the pathomechanism of the severe lung disease. In this case, which was characterized by a mixed IgA/IgG antibody panel displaying known and unique antigenicity, the serious episodes of paraneoplastic pemphigus flared after cyclophosphamide treatment.
KW - BP 180
KW - Cyclophosphamide
KW - Desmocollin
KW - Paraneoplastic pemphigus
KW - Pulmonary involvement
UR - http://www.scopus.com/inward/record.url?scp=3042780292&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=3042780292&partnerID=8YFLogxK
U2 - 10.1111/j.1365-2133.2004.05978.x
DO - 10.1111/j.1365-2133.2004.05978.x
M3 - Article
C2 - 15149520
AN - SCOPUS:3042780292
VL - 150
SP - 1018
EP - 1024
JO - British Journal of Dermatology
JF - British Journal of Dermatology
SN - 0007-0963
IS - 5
ER -