Paraneoplastic pemphigus. A distinct autoimmune vesiculobullous disorder associated with neoplasia

Thomas N. Helm, Charles Camisa, Rafael Valenzuela, Carl M. Allen

Research output: Contribution to journalArticle

31 Citations (Scopus)

Abstract

A vesiculobullous disease termed paraneoplastic pemphigus with distinct autoantibodies was newly described in 1990. All reported cases have occurred in patients with a history of neoplasia, including lymphoma, chronic lymphocytic leukemia, poorly differentiated sarcoma, and benign thymoma. As in pemphigus vulgaris, intraepithelial clefts with acantholysis are noted hisopathologically, and intercellular binding of immunoreactants is seen with direct immunofluorescence studies of mucous membrane and skin biopsies. However, immunoreactants may also be found along the basement membrane zone in paraneoplastic pemphigus. Indirect immunofluorescence using rat bladder epithelium as substrate shows an intercellular pattern that appears to be highly specific for paraneoplastic pemphigus. We report a patient with non-Hodgkins lymphoma of 8 years duration who developed severe erosive stomatitis and lichenoid dermatitis after receiving chemotherapy for a relapse of lymphoma. Her case illustrates the typical features of the disorder described as paraneoplastic pemphigus. Neoplasia-associated pemphigus may be a more precise term for this disorder because the course of the blistering eruption does not always parallel the course of the underlying cancer. The clinical features, histopathologic findings, and immunofluorescence findings of this unique syndrome are reviewed.

Original languageEnglish
Pages (from-to)209-213
Number of pages5
JournalOral Surgery, Oral Medicine, Oral Pathology
Volume75
Issue number2
DOIs
StatePublished - Jan 1 1993
Externally publishedYes

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Pemphigus
Neoplasms
Lymphoma
Acantholysis
Direct Fluorescent Antibody Technique
Stomatitis
Thymoma
Dermatitis
B-Cell Chronic Lymphocytic Leukemia
Indirect Fluorescent Antibody Technique
Basement Membrane
Sarcoma
Autoantibodies
Non-Hodgkin's Lymphoma
Fluorescent Antibody Technique
Mucous Membrane
Urinary Bladder
Epithelium
Biopsy
Recurrence

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Dentistry(all)

Cite this

Paraneoplastic pemphigus. A distinct autoimmune vesiculobullous disorder associated with neoplasia. / Helm, Thomas N.; Camisa, Charles; Valenzuela, Rafael; Allen, Carl M.

In: Oral Surgery, Oral Medicine, Oral Pathology, Vol. 75, No. 2, 01.01.1993, p. 209-213.

Research output: Contribution to journalArticle

Helm, Thomas N. ; Camisa, Charles ; Valenzuela, Rafael ; Allen, Carl M. / Paraneoplastic pemphigus. A distinct autoimmune vesiculobullous disorder associated with neoplasia. In: Oral Surgery, Oral Medicine, Oral Pathology. 1993 ; Vol. 75, No. 2. pp. 209-213.
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