The occurrence of cancer in immune deficiencies

A statistically increased incidence of malignancy has been observed in patients with pemphigus. A review of the literature reveals 42 cases of nonthymic malignancies and 18 cases of thymic malignancies. A significant predominence of men, with mean age at onset of 50 years, was observed. Pemphigus vulgaris is more common in patients with nonthymic neoplasms, whereas pemphigus foliaceus or pemphipus erythematosus and pemphigus vulgaris are equally common in patients with thymic neoplasms. Lymphoreticular malignancies, especially Kaposi's sarcoma, are most frequently observed. The majority of patients with nonthymic neoplasms have pemphigus before the detection of the malignancy and have a favorable 5-year survival rate after tumor resection. The majority of the patients with thymic neoplasms have a thymoma before the development of pemphigus. In some patients pemphigus develops after thymectomy and myasthenia gravis is often associated. Overall, 37 of the 60 patients, or 61%, had a neoplasm of the immune system.

The relationship of pemphigus to neoplasia.

Current evidence strongly supports the theory that the lesions of pemphigus are due to binding of pemphigus antibody to an antigen in or near the epidermal cell membrane, which causes a release of at least one enzyme which results in dissolution of the intercellular attachments and acantholysis. Similarly, strong evidence supports the hypothesis that pemphigoid blisters are due to binding of antibody at the basement membrane, followed by activation of complement and release of anaphylatoxins which activate tissue mast cells to release eosinophil chemotactic factor. These eosinophils then release tissue-destructive enzymes and reactive oxygen intermediates directly onto the basement membrane zone, with loss of dermoepidermal adherence and formation of blisters.

Mechanism of lesion production in pemphigus and pemphigoid

• Background.— Paraneoplastic pemphigus refers to a specific disease entity in which neoplasia is associated with severe mucosal ulceration and polymorphous cutaneous eruptions, sometimes resembling erythema multiforme. Direct immunofluorescence and indirect immunofluorescence on standard substrates are similar to pemphigus vulgaris; however, paraneoplastic pemphigus serum uniquely binds to all epithelia and has distinctive immunoprecipitation characteristics. Observations.— A 63-year-old woman with chronic lymphocytic leukemia developed a severe, mucocutaneous, vesiculobullous eruption with the clinical and histopathologic characteristics of lichen planus pemphigoides evolving into a Stevens-Johnson—like presentation and the immunofluorescence findings of pemphigus vulgaris. Evaluation of her serum confirmed the presence of autoantibodies specific for paraneoplastic pemphigus by indirect immunofluorescence and immunoprecipitation criteria. Conclusion.— The spectrum of paraneoplastic pemphigus must now be expanded to include cases that present clinically and histologically as a lichen planus pemphigoides— like eruption. ( Arch Dermatol. 1993;129:866-869)

Paraneoplastic Pemphigus Presenting as a Lichen Planus Pemphigoides—like Eruption

CMV retinitis develops in approximately 28-35% of all AIDS patients at later stages of disease, often leading to blindness. To determine whether the subset of AIDS patients who developed CMV retinitis (CMV-R) were immunologically predisposed, T cell proliferation responses to CMV were examined prospectively in an HIV infected, HLA typed, longitudinal study population. Individuals who developed CMV-R had significantly lower T cell proliferation responses to CMV, both early and late in disease, compared to CD4 matched controls who have not developed CMV-R. Since HLA proteins influence T-cell recognition, phenotypes of 21 CMV-R patients were examined to determine whether certain HLA alleles were associated with low immune response and predisposed AIDS patients to CMV-R. HLA DR7 and B44 were at increased (nearly twice the expected) frequency in those with CMV-R. The combined association of either B44, 51 or DR7 with CMV-R was highly significant (P = .008, relative risk of CMV-R = 15) with correction for multiple comparisons. Low immune responses were twice as frequent in those with (61%) compared to those without (30%) predisposing alleles. Thus, AIDS patients with immunogenetically related hyporesponsiveness to CMV antigens may be at increased risk of retinitis.

/pdf/immune-predispositions-for-cytomegalovirus-retinitis-in-aids-4i13nunizu.pdf

Immune predispositions for cytomegalovirus retinitis in AIDS. The HNRC Group.

Five patients with pemphigus vulgaris representing 12% of the total cases seen at the University of California at Los Angeles Center for the Health Sciences were found to have associated internal malignancies. Three patients developed internal malignancy after receiving corticosteroid therapy for periods of 2-108 months. In approximately 54% of patients surveyed in the literature with this association, the malignancy involved the lymphoid or reticuloendothelial systems. Pemphigus and internal malignancies or thymomas may be related through some intervening variables such as immunosuppressive therapy, the antigenic and cross-reacting nature of the primary tumor, or as an immunologic consequence of associated autoimmune disease, which occurred in 48% of the cases reviewed. Our observation of five patients with pemphigus and internal malignancies greatly exceeds chance expectations for the Los Angeles area. A review of the literature indicates that pemphigus and internal malignancies are causally related by the criteria employed by Curth.12 Cancer 33:1091-1099,1974.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/1097-0142%28197404%2933%3A4%3C1091%3A%3AAID-CNCR2820330430%3E3.0.CO%3B2-Y

Pemphigus vulgaris and internal malignancy.

In this preliminary study the phenotypic distribution of HLA and DRw antigens was investigated in patients with recurrent herpes progenitalis. Ninety-three white men between the ages of 20 and 53 years, with at least a 2-year history of recurrent herpes progenitalis infection, and 558 age-matched white men from the same area of Los Angeles were studied by means of a microcytotoxicity assay. Statistical analysis of data indicated that there is no increased incidence of any HLA antigen. The B cell antigen DRw7 is present in 37% of the patients and 22% of controls, with a p value of 0.009 and a relative risk of 2.1. These results suggest that there may be genetic and immunologic factors that tend to predispose certain individuals to the development of recurrent herpes progenitalis when they are exposed to the virus.

A study of HLA and DRw antigens in severe recurrent herpes progenitalis (HS V-2) infection

In this preliminary study the phenotypic distribution of HLA and DRw antigens was investigated in patients with recurrent herpes progenitalis. Ninety-three white men between the ages of 20 and 53 years, with at least a 2-year history of recurrent herpes progenitalis infection, and 558 age-matched white men from the same area of Los Angeles were studied by means of a microcytotoxicity assay. Statistical analysis of data indicated that there is no increased incidence of any HLA antigen. The B cell antigen DRw7 is present in 37% of the patients and 22% of controls, with a p value of 0.009 and a relative risk of 2.1. These results suggest that there may be genetic and immunologic factors that tend to predispose certain individuals to the development of recurrent herpes progenitalis when they are exposed to the virus. (J AM ACAD DERMATOL 6:898-901, 1982.)

https://www.jaad.org/article/S0190-9622(82)70079-9/pdf

A study of HLA and DRw antigens in recurrent herpes progenitalis (HSV-2) infection severe

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Pemphigus vulgaris and internal malignancy.

A study of HLA and DRw antigens in severe recurrent herpes progenitalis (HS V-2) infection

A study of HLA and DRw antigens in recurrent herpes progenitalis (HSV-2) infection severe