Coexistence of Peculiar Pemphigus, Myasthenia gravis and Malignant Thymoma

Abstract

An unusual vesiculobullous disease and myasthenia gravis occurred concurrently with a malignant thymoma in a Japanese male. The bullous dermatosis had combined clinical and histological features of both dermatitis herpetiformis and pemphigus. Serum showed striated muscle antibodies and intercellular epithelial autoantibodies; the latter had been demonstrated even prior to the development of the skin lesions. Thus, immunologically, this dermatosis was confirmed to be a variety of pemphigus.

Exacerbation of Pemphigus Foliaceus After Electron-beam Radiation

“…None of the other cases had a history of blistering disease before irradiation but, in our case, preexisting, well-controlled PF drastically worsened (8)(9)(10)(11). In PV, 3 cases which had history of pemphigus were reported to show exacerbation or recurrence after irradiation (4)(5)(6).…”

“…The first case of PF after radiation was described by Tagami et al (9) in 1976. The case was a 41-year-old Japanese man who underwent cobalt therapy for a thymic tumour.…”

“…It is unclear how radiation therapy acts as a trigger to induce or exacerbate pemphigus (8)(9)(10)(11). Pemphigus is induced by the autoantibodies for Dsg (1); therefore, enhancement of the antigen-antibody reaction may be a key to explaining the aggravation of pemphigus by irradiation.…”

Clinical, pathologic, and immunologic features of pemphigus herpetiformis: a literature review and proposed diagnostic criteria

“…Pemphigus herpetiformis associated with neoplasia is rare (6%). Among reported cases were three patients with lung cancer; 1, esophageal carcinoma; 1, thymic tumor; 1, lymphoma; 2, prostate cancer; 1, cutaneous angiosarcoma; and 1, extramammary Paget disease . In most cases, because of the limited descriptions and variable immunologic profiles among affected patients, the correlation between PH and neoplasia was unclear.…”

Pemphigus foliaceus induced by radiotherapy and responsive to dapsone

“…Six months later he developed a pruritic vesicular eruption diagnosed as pemphigus. The author did not describe the type, but some reviewers interpreted it as consistent with pemphigus foliaceus (6). No more cases have been described in the literature.…”