A nonfatal case and 2 fatal cases of paraneoplastic pemphigus: can a complement indirect immunofluorescent test help to identify fatal "group A" paraneoplastic pemphigus cases?

We studied 3 recent cases of paraneoplastic pemphigus (PNP) in detail. Two patients died despite concerted management efforts. One patient received no treatment after the appearance of PNP and recovered completely from both PNP and lymphoma. Multiple serum studies of these 3 patients plus 9 other proven PNP cases revealed that 8 of 9 fatal PNP cases (referred to here as "group A") had distinctive cell surface antibodies detected by complement indirect immunofluorescent (CIIF) tests on monkey esophagus sections. By contrast, none of the sera from 3 patients with PNP who experienced long-term survival (referred to here as "group B") and none of 20 pemphigus vulgaris or 10 pemphigus foliaceus control sera revealed similar beaded cell surface CIIF reaction patterns, a difference that is statistically significant (P <.0001). Cell surface CIIF reaction patterns of group A PNP antibodies resemble the pattern of pemphigus antibody reactions in indirect immunofluorescent tests on the same substrate; however, the latter tend to be thinner and more linear, whereas the cell surface CIIF pattern tends to be more beaded, suggesting possible desmosomal reactions. We believe this test is useful in identifying an aggressive group A form of PNP.

[1]  J. Roujeau,et al.  Paraneoplastic pemphigus with circulating antibodies directed exclusively against the pemphigus vulgaris antigen desmoglein 3. , 2000, Journal of the American Academy of Dermatology.

[2]  P. Courville,et al.  Sensitivity and specificity of clinical, histologic, and immunologic features in the diagnosis of paraneoplastic pemphigus. , 2000, Journal of the American Academy of Dermatology.

[3]  A. Grañena,et al.  Feasibility of ESHAP + G-CSF as peripheral blood hematopoietic progenitor cell mobilisation regimen in resistant and relapsed lymphoma: a single-center study of 22 patients. , 1999, Leukemia & lymphoma.

[4]  J. Binet,et al.  Intensive chemotherapy with hematopoietic cell transplantation after ESHAP therapy for relapsed or refractory non-Hodgkin's lymphoma. Results of a single-centre study of 65 patients. , 1999, Leukemia & lymphoma.

[5]  M. Grever,et al.  Immunoablative high-dose cyclophosphamide without stem cell rescue in paraneoplastic pemphigus: report of a case and review of this new therapy for severe autoimmune disease. , 1999, Journal of the American Academy of Dermatology.

[6]  A. Santoro,et al.  Dose-escalation of CHOP in non-Hodgkin's lymphoma. , 1999, Annals of oncology : official journal of the European Society for Medical Oncology.

[7]  E. Beutner,et al.  Relevance of complement fixing antinuclear antibodies , 1999, International journal of dermatology.

[8]  E. Bröcker,et al.  Paraneoplastic pemphigus treated with dexamethasone/ cyclophosphamide pulse therapy. , 1999, European journal of dermatology : EJD.

[9]  A. Sklavounou,et al.  Paraneoplastic pemphigus: a review. , 1998, Oral oncology.

[10]  T. Miller.  Management of intermediate-grade lymphomas. , 1998, Oncology.

[11]  K. Wolff,et al.  Erythema multiforme associated human autoantibodies against desmoplakin I and II: biochemical characterization and passive transfer studies into newborn mice. , 1998, The Journal of investigative dermatology.

[12]  T. Nishikawa,et al.  Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice. , 1998, The Journal of clinical investigation.

[13]  J. Uitto,et al.  The members of the plakin family of proteins recognized by paraneoplastic pemphigus antibodies include periplakin. , 1998, The Journal of investigative dermatology.

[14]  E. Beutner,et al.  Antinuclear antibodies (ANA) and complement fixing ANA in systemic connective tissue diseases. , 1998, Immunological investigations.

[15]  T. Nishikawa,et al.  A pemphigus case with long term survival, implicating the spectrum between paraneoplastic pemphigus and pemphigus vulgaris , 1997, International journal of dermatology.

[16]  K. Wolff,et al.  Autoantibodies against desmoplakin I and II define a subset of patients with erythema multiforme major. , 1996, The Journal of investigative dermatology.

[17]  T. Nishikawa,et al.  Antigen-specific immunoadsorption of pathogenic autoantibodies in pemphigus foliaceus. , 1995, The Journal of investigative dermatology.

[18]  M. Amagai,et al.  Characterization of paraneoplastic pemphigus autoantigens by immunoblot analysis. , 1994, The Journal of investigative dermatology.

[19]  C. Lebbé,et al.  Overlapping distribution of autoantibody specificities in paraneoplastic pemphigus and pemphigus vulgaris. , 1994, The Journal of investigative dermatology.

[20]  J. Tabernero,et al.  CHOP chemotherapy of intermediate and high-grade non-Hodgkin's lymphoma. , 1994, Acta oncologica.

[21]  N. Pelc,et al.  Paraneoplastic Pemphigus: An Autoimmune Mucocutaneous Disease Associated with Neoplasia , 1990 .

[22]  Y. C. Liu,et al.  Paraneoplastic pemphigus: a report of three cases including one long-term survivor. , 1992, Journal of the American Academy of Dermatology.

[23]  M. Yaar,et al.  Pemphigus antibodies identify a cell surface glycoprotein synthesized by human and mouse keratinocytes. , 1982, The Journal of clinical investigation.

[24]  W. F. Lever.  Pemphigus and pemphigoid: A review of the advances made since 1964 , 1979 .

[25]  G. Friou,et al.  Lupus Nephritis: Varying Complement-Fixing Properties of Immuvnoglobulin G Antibodies to Antigens of Cel Nuclei , 1968, Science.

[26]  E. Beutner,et al.  Quantitative studies of immunofluorescent staining. Relationships of characteristics of unabsorbed antihuman IgG conjugates to their specific and non-specific staining properties in an indirect test for antinuclear factors. , 1968, Bulletin of the World Health Organization.