Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans

T. Chorzelski, T. Hashimoto, B. Maciejewska, Masayuki Amagai, G. J. Anhalt, S. Jablonskaa

Research output: Contribution to journalArticle

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Abstract

Background: Cases of paraneoplastic pemphigus (PNP) have been reported associated with various lymphoproliferative malignancies and benign Castleman tumors, with the most severe course and fatal outcome seen in patients with bronchiolitis obliterans. Objective: The aim was to establish immunologic associations by coexistence of Castleman tumor, myasthenia gravis, and bronchiolitis obliterans and to evaluate the treatment modalities. Methods: Clinical studies included computed tomography of the mediastinum, computed tomography and magnetic resonance imaging of the abdominal cavity, and quantitative electromyography. Direct and indirect immunofluorescence on various substrates, immunoblot analysis, immunoprecipitation, and specific enzyme-linked immunosorbent assay using recombinant desmogleins (Dsg) were performed as immunologic assays. Results: Direct and indirect immunofluorescence including rat bladder showed intercellular antibodies. Immunoblotting disclosed antibodies to envoplakin (210 kd protein) and periplakin (190 kd protein); in addition, immunoprecipitation detected antibodies to desmoplakin I (250 kd protein). Antibodies to Dsg3 (pemphigus vulgaris antigen) were detected by specific enzyme-linked immunosorbent assay. Myasthenia gravis was controlled by drugs; however, mucocutaneous changes were not fully responsive to corticosteroids and cyclophosphamide pulses, cyclosporine, and intravenous immunoglobulins. The surgical removal of Castleman tumor did not change the course of the disease. The fatal outcome was the result of bronchiolitis obliterans that occurred after the surgery and was only transitionally controlled by plasmapheresis. Conclusion: This is the first case of paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans. Despite a benign character of the tumor the patient died, as do all patients with bronchiolitis obliterans. Massive plasmapheresis has only a transient effect. We confirmed the presence of antibodies to Dsg 3, in addition to the set of specific paraneoplastic pemphigus antibodies against various proteins of plakin family.

Original languageEnglish
Pages (from-to)393-400
Number of pages8
JournalJournal of the American Academy of Dermatology
Volume41
Issue number3
DOIs
Publication statusPublished - 1999
Externally publishedYes

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Bronchiolitis Obliterans
Giant Lymph Node Hyperplasia
Pemphigus
Myasthenia Gravis
Antibodies
Direct Fluorescent Antibody Technique
Fatal Outcome
Plasmapheresis
Indirect Fluorescent Antibody Technique
Immunoprecipitation
Proteins
Desmogleins
Desmoglein 3
Desmoplakins
Enzyme-Linked Immunosorbent Assay
Tomography
Abdominal Cavity
Intravenous Immunoglobulins
Mediastinum
Electromyography

ASJC Scopus subject areas

  • Dermatology

Cite this

Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans. / Chorzelski, T.; Hashimoto, T.; Maciejewska, B.; Amagai, Masayuki; Anhalt, G. J.; Jablonskaa, S.

In: Journal of the American Academy of Dermatology, Vol. 41, No. 3, 1999, p. 393-400.

Research output: Contribution to journalArticle

Chorzelski, T. ; Hashimoto, T. ; Maciejewska, B. ; Amagai, Masayuki ; Anhalt, G. J. ; Jablonskaa, S. / Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans. In: Journal of the American Academy of Dermatology. 1999 ; Vol. 41, No. 3. pp. 393-400.
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AU - Chorzelski, T.

AU - Hashimoto, T.

AU - Maciejewska, B.

AU - Amagai, Masayuki

AU - Anhalt, G. J.

AU - Jablonskaa, S.

PY - 1999

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