Pemphigus vulgaris and internal malignancy

Abstract

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.

Pemphigus

“…The blisters result from acantholysis. PNP was recognized as a distinct entity in 1990 1 after various reports 2–6 associated pemphigus and malignancy, usually of lymphoid tissue.…”

Pemphigus vegetans in a patient with colonic cancer

“…[6] A statistically increased incidence of malignancy is observed in patients with pemphigus, mainly pemphigus vulgaris and pemphigus erythematosus. [7,8] However, the coexistence of pemphigus vegetans and internal neoplasm has been rarely reported in the literature, being described in four cases, two cases of coexistence with lung cancer, [2,3] one with a lymphosarcoma, [4] and another associated with a gastric cancer. [5] In the case described by Bastiaens et al, [3] the removal of the tumor, a lung cancer, caused the disappearance of the lesions and circulating antibodies of pemphigus, proving the inducing role of the tumor.…”

Thymoma with Pemphigus Foliaceus.

“…Pemphigus is an immunologically mediated skin disease characterized by epidermal blisters that may occur in association with thymic or nonthymic neoplasms (2,3). Since some patients with dermatological complaints consult an internist first, it is important to recognize skin disorders as possible sequelae of thymoma,the most commonprimary neoplasm in the mediastinum (1).…”