Paraneoplastic pemphigus: A report of three cases including one long-term survivor

C. Camisa, T. N. Helm, Y. C. Liu, R. Valenzuela, C. Allen, S. Bona, N. Larrimer, N. J. Korman

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Abstract

Background: Paraneoplastic pemphigus is a newly described autoimmune disease characterized by painful mucosal ulcerations and polymorphous skin lesions in association with an underlying neoplasm. All reported patients with an associated malignant neoplasm have had a poor prognosis. Objective: We present three new cases of paraneoplastic pemphigus associated with a malignant neoplasm and further characterize this disease. Methods: We used clinical criteria, histologic and immunopathologic examinations, and immunophenotyping to characterize this disease. In addition, we performed immunoprecipitation studies with extracts of radiolabeled human keratinocytes to characterize the antigens to which patient serum binds. Results: All three patients had clinical, histologic, and immunopathologic findings that were strongly suggestive of paraneoplastic pemphigus. Their sera immunoprecipitated a complex of four polypeptides from human keratinocyte extracts with molecular weights of 250, 230, 210, and 190 kd, confirming the diagnosis of paraneoplastic pemphigus. The 250, 230, and 210 kd antigens comigrated with desmoplakin I, the 230 kd bullous pemphigoid antigen, and desmoplakin II, respectively. Lymphocyte immunophenotyping revealed large populations of monoclonal CD19+, CD5+ B cells in two cases. Although two of the patients died, one patient is alive and well 2 years after the diagnosis of paraneoplastic pemphigus. Conclusion: We report three cases of paraneoplastic pemphigus. One patient is alive and well 2 years after diagnosis, which suggests that a subgroup of patients may have a more benign course.

Original languageEnglish
Pages (from-to)547-553
Number of pages7
JournalJournal of the American Academy of Dermatology
Volume27
Issue number4
StatePublished - Jan 1 1992
Externally publishedYes

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Pemphigus
Survivors
Desmoplakins
Immunophenotyping
Keratinocytes
Antigens
Bullous Pemphigoid
Neoplasms
Serum
Immunoprecipitation
Autoimmune Diseases
B-Lymphocytes
Molecular Weight
Skin
Peptides
Population

ASJC Scopus subject areas

  • Dermatology

Cite this

Camisa, C., Helm, T. N., Liu, Y. C., Valenzuela, R., Allen, C., Bona, S., ... Korman, N. J. (1992). Paraneoplastic pemphigus: A report of three cases including one long-term survivor. Journal of the American Academy of Dermatology, 27(4), 547-553.

Paraneoplastic pemphigus : A report of three cases including one long-term survivor. / Camisa, C.; Helm, T. N.; Liu, Y. C.; Valenzuela, R.; Allen, C.; Bona, S.; Larrimer, N.; Korman, N. J.

In: Journal of the American Academy of Dermatology, Vol. 27, No. 4, 01.01.1992, p. 547-553.

Research output: Contribution to journalArticle

Camisa, C, Helm, TN, Liu, YC, Valenzuela, R, Allen, C, Bona, S, Larrimer, N & Korman, NJ 1992, 'Paraneoplastic pemphigus: A report of three cases including one long-term survivor', Journal of the American Academy of Dermatology, vol. 27, no. 4, pp. 547-553.
Camisa C, Helm TN, Liu YC, Valenzuela R, Allen C, Bona S et al. Paraneoplastic pemphigus: A report of three cases including one long-term survivor. Journal of the American Academy of Dermatology. 1992 Jan 1;27(4):547-553.
Camisa, C. ; Helm, T. N. ; Liu, Y. C. ; Valenzuela, R. ; Allen, C. ; Bona, S. ; Larrimer, N. ; Korman, N. J. / Paraneoplastic pemphigus : A report of three cases including one long-term survivor. In: Journal of the American Academy of Dermatology. 1992 ; Vol. 27, No. 4. pp. 547-553.
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abstract = "Background: Paraneoplastic pemphigus is a newly described autoimmune disease characterized by painful mucosal ulcerations and polymorphous skin lesions in association with an underlying neoplasm. All reported patients with an associated malignant neoplasm have had a poor prognosis. Objective: We present three new cases of paraneoplastic pemphigus associated with a malignant neoplasm and further characterize this disease. Methods: We used clinical criteria, histologic and immunopathologic examinations, and immunophenotyping to characterize this disease. In addition, we performed immunoprecipitation studies with extracts of radiolabeled human keratinocytes to characterize the antigens to which patient serum binds. Results: All three patients had clinical, histologic, and immunopathologic findings that were strongly suggestive of paraneoplastic pemphigus. Their sera immunoprecipitated a complex of four polypeptides from human keratinocyte extracts with molecular weights of 250, 230, 210, and 190 kd, confirming the diagnosis of paraneoplastic pemphigus. The 250, 230, and 210 kd antigens comigrated with desmoplakin I, the 230 kd bullous pemphigoid antigen, and desmoplakin II, respectively. Lymphocyte immunophenotyping revealed large populations of monoclonal CD19+, CD5+ B cells in two cases. Although two of the patients died, one patient is alive and well 2 years after the diagnosis of paraneoplastic pemphigus. Conclusion: We report three cases of paraneoplastic pemphigus. One patient is alive and well 2 years after diagnosis, which suggests that a subgroup of patients may have a more benign course.",
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N2 - Background: Paraneoplastic pemphigus is a newly described autoimmune disease characterized by painful mucosal ulcerations and polymorphous skin lesions in association with an underlying neoplasm. All reported patients with an associated malignant neoplasm have had a poor prognosis. Objective: We present three new cases of paraneoplastic pemphigus associated with a malignant neoplasm and further characterize this disease. Methods: We used clinical criteria, histologic and immunopathologic examinations, and immunophenotyping to characterize this disease. In addition, we performed immunoprecipitation studies with extracts of radiolabeled human keratinocytes to characterize the antigens to which patient serum binds. Results: All three patients had clinical, histologic, and immunopathologic findings that were strongly suggestive of paraneoplastic pemphigus. Their sera immunoprecipitated a complex of four polypeptides from human keratinocyte extracts with molecular weights of 250, 230, 210, and 190 kd, confirming the diagnosis of paraneoplastic pemphigus. The 250, 230, and 210 kd antigens comigrated with desmoplakin I, the 230 kd bullous pemphigoid antigen, and desmoplakin II, respectively. Lymphocyte immunophenotyping revealed large populations of monoclonal CD19+, CD5+ B cells in two cases. Although two of the patients died, one patient is alive and well 2 years after the diagnosis of paraneoplastic pemphigus. Conclusion: We report three cases of paraneoplastic pemphigus. One patient is alive and well 2 years after diagnosis, which suggests that a subgroup of patients may have a more benign course.

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