Histologic Features of Paraneoplastic Pemphigus

Thomas D. Horn, Grant James Anhalt

Research output: Contribution to journalArticle

Abstract

Background.—We describe the histopathologic features of paraneoplastic pemphigus, a recently described autoimmune mucocutaneous disease associated with neoplasia. Complete evaluation for paraneoplastic pemphigus requires identification of the characteristic mucocutaneous eruption, tissue specimens for routine histologic and direct immunofluorescence evaluation, and identification of circulating autoantibodies with a unique specificity. Immunoprecipitation from keratinocytes reveals a characteristic complex of four proteins with the circulating antibodies. Various neoplasms have been identified in patients with paraneoplastic pemphigus. Observations.—We reviewed 16 skin and oral mucous membrane biopsy specimens from six patients with paraneoplastic pemphigus confirmed by fulfillment of all criteria. Major features include epidermal acantholysis, suprabasal cleft formation, dyskeratotic keratinocytes, vacuolar change of the basilar epidermis, and epidermal exocytosis of inflammatory cells. Seven (44%) of 16 specimens displayed a unique combination of suprabasal acantholysis and dyskeratotic keratinocytes throughout the epidermis. These histologic findings correspond to those of the characteristic clinical lesions that are described as having features of pemphigus and erythema multiforme. Conclusions.—Paraneoplastic pemphigus represents a unique clinical, histologic, and immunologic disease characterized by autoantibody production to desmoplakin I and desmoplakin II, bullous pemphigoid antigen, and, possibly, other antigens in the desmosomal complex. Recognition of the histologic features should prompt immunopathologic confirmation and evaluation for an occult neoplasm.

Original languageEnglish (US)
Pages (from-to)1091-1095
Number of pages5
JournalArchives of Dermatology
Volume128
Issue number8
DOIs
StatePublished - 1992

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Pemphigus
Desmoplakins
Acantholysis
Keratinocytes
Epidermis
Autoantibodies
Erythema Multiforme
Antigens
Bullous Pemphigoid
Neoplasms
Direct Fluorescent Antibody Technique
Immune System Diseases
Exocytosis
Immunoprecipitation
Autoimmune Diseases
Mucous Membrane
Biopsy
Skin
Antibodies
Proteins

ASJC Scopus subject areas

  • Dermatology

Cite this

Histologic Features of Paraneoplastic Pemphigus. / Horn, Thomas D.; Anhalt, Grant James.

In: Archives of Dermatology, Vol. 128, No. 8, 1992, p. 1091-1095.

Research output: Contribution to journalArticle

Horn, Thomas D. ; Anhalt, Grant James. / Histologic Features of Paraneoplastic Pemphigus. In: Archives of Dermatology. 1992 ; Vol. 128, No. 8. pp. 1091-1095.
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