Paraneoplastic Pemphigus

Anhalt, Grant J.; Kim, Soochan; Stanley, John R.; Korman, Neil J.; Jabs, Douglas A.; Kory, Mark; Izumi, Hiroshi; Ratrie, Harry; Mutasim, Diya F.; Ariss-Abdo, Lina; Labib, Ramzy S.

doi:10.1056/nejm199012203232503

Cited by 937 publications

(188 citation statements)

References 24 publications

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“…Pemphigus or pemphigoid autoantibodies have been reported to occur specifically in pemphigus or pemphigoid patients [2, 3, 4, 19]or in bullous diseases including paraneoplastic pemphigus [20, 21, 22]and drug-induced pemphigus [23, 24]. These results were obtained by the immunofluorescent technique, immunoblotting methods and the ELISA against Dsg1 and Dsg3 using patient sera.…”

Section: Discussionmentioning

confidence: 99%

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Antidesmoglein Autoantibodies in Silicosis Patients with No Bullous Diseases

Ueki¹,

Kohda²,

Nobutoh³

et al. 2001

Dermatology

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Background: Pemphigus is an autoimmune bullous disease characterized by the presence of antidesmoglein autoantibodies. However, the mechanism of its autoantibody production remains unknown. In previous reports, we have described rare cases of pemphigus and pemphigoid associated with silicosis. It is well known that during long-term silicosis, some autoimmune diseases, such as systemic sclerosis, systemic lupus erythematosus or rheumatoid arthritis, can occur. Objective: The aim of this study was to explore the presence of pemphigus or pemphigoid autoantibodies in silicosis patients without clinical bullous diseases or collagen diseases. Method: The presence of pemphigus antibodies was examined in 54 silicosis patients with no associated bullous diseases, using immunofluorescence, the enzyme-linked immunosorbent assay (ELISA) for desmoglein 1 and 3, and immunoblotting methods. In the antibody-positive cases, HLA genotyping of peripheral lymphocytes was performed with PCR-RFLP. Results: Seven out of the 54 patients were found to be positive for pemphigus antibodies and 1 for bullous pemphigoid by immunofluorescence. In addition, by ELISA, 6 patients were found to be positive against the desmoglein 1 antigen, 2 against the desmoglein 3 antigen and 2 against both desmoglein 1 and desmoglein 3. Conclusion: The results of the present study strongly suggest the occurrence of pemphigus and pemphigoid autoantibodies in patients with silicosis. It remains unclear whether such patients will develop an autoimmune bullous disease in the future. Accordingly, long-term follow-up of antibody-positive patients is required.

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Section: Discussionmentioning

confidence: 99%

“…Certain drugs [23, 24], such as angiotensin-converting enzyme inhibitors or D -penicillamine, or internal malignant tumors [20, 21, 22]in paraneoplastic pemphigus are examples of environmental factors reported previously.…”

Section: Discussionmentioning

confidence: 99%

Antidesmoglein Autoantibodies in Silicosis Patients with No Bullous Diseases

Ueki¹,

Kohda²,

Nobutoh³

et al. 2001

Dermatology

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“…Anhalt et al [1]suggest 5 criteria to define PNP: (a) a polymorphous eruption that affects the skin (with papular lesions, progressing to blisters and erosive lesions) and mucous membranes (with extremely painful erosions) in the context of an occult or confirmed neoplasm; (b) cutaneous histopathological features that include intraepidermal acantholysis and dyskeratosis, with basal layer vacuolar changes; (c) intraepidermal and/or basement membrane zone deposition of IgG and complement on direct immunofluorescence; (d) serum autoantibodies (indirect immunofluorescence) that bind the cell surface of skin and mucosal epithelium in a pattern typical of pemphigus, but also bind to simple, columnar and transition (e.g. bladder) epithelium, and (e) immunoprecipitation of a complex of 4 proteins (250, 230, 210 and 190 kD) from keratinocytes by these autoantibodies.…”

Section: Discussionmentioning

confidence: 99%

“…Paraneoplastic pemphigus (PNP) is a paraneoplastic syndrome that may develop in patients suffering from different types of neoplasia [1]. There had been anecdotal reports of ‘pemphigus-like’ blistering of the skin and erosive mucocutaneous lesions associated with neoplasms, before Anhalt and his group found antibodies in the serum of such patients that identified a unique complex of epidermal proteins, different from the antibodies in sera of patients with pemphigus vulgaris and pemphigus foliaceus [2].…”

Section: Introductionmentioning

confidence: 99%

“…The cutaneous lesions are polymorphous with papulosquamous lesions, erythematous papules, target lesions with central vesiculation and sometimes large tense bullae, reminiscent of bullous pemphigoid. The skin lesions are usually pruritic [1]. In this article, we describe a patient fulfilling all criteria for PNP, who developed corneal melting in both eyes.…”

Section: Introductionmentioning

confidence: 99%

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Bilateral Corneal Melting in a Patient with Paraneoplastic Pemphigus

Beele

Claerhout²,

Kestelyn³

et al. 2001

Dermatology

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An 80-year-old man, with a solid abdominal tumor and multiple skin lesions, was admitted to the hospital because of a perforated right cornea and an impending perforation of the left. The clinical, histological, immunohistological and immunoprecipitation findings of the skin lesions were consistent with Anhalt’s criteria for paraneoplastic pemphigus (PNP). The underlying malignancy proved to be an incurable peripheral neuronal shaft tumor. Both conjunctivae appeared normal. The right eye revealed a flat anterior chamber, due to a spontaneous, central corneal perforation. The central part of the left cornea had severely thinned, resulting in a descemetocele, which eventually perforated. Multiple surgical interventions were needed to restore the anterior chamber in both eyes. Although a causative association between PNP and corneal perforation could not be demonstrated, we think that corneal melting should be added to the list of ocular complications in patients with PNP.

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Immunoapheresis in Paraneoplastic Pemphigus

Schoen

Foedinger²,

Derfler³

et al. 1998

Arch Dermatol

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Background: Paraneoplastic pemphigus was first described in 1990 in 5 patients with extensive mucocutaneous erosions, a distinct set of autoantibodies, and underlying neoplasia. Since then, patients described have been middle-aged, have suffered from prognostically unfavorable malignant neoplasms, and have responded poorly to immunosuppressive agents.Observation: A 16-year-old boy was examined with extensive oral erosions, halitosis that interfered with his quality of life, and rapid weight loss. The suspected clinical diagnosis of paraneoplastic pemphigus was confirmed by histopathological, immunofluorescence, and biochemical (eg, immunoblotting and immunoprecipitation) findings as well as by the demonstration of an inflammatory myofibroblastic tumor of the left retroclavicular region.Despite administration of corticosteroids, followed by excision of the neoplasm, clinical symptoms improved only slightly, and autoantibody titers decreased only marginally. We therefore initiated an immunoapheresis regimen with the use of sheep anti-human-IgG beadformed agarose gel (Sepharose; Pharmacia Biotech Comp, Vienna, Austria), which led to the disappearance of circulating autoantibodies and the patient's recovery. Conclusion:Immunoapheresis may represent a novel therapeutic option for patients with paraneoplastic pemphigus who show little improvement after curative treatment of their neoplasms.

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Paraneoplastic Pemphigus

Cited by 937 publications

References 24 publications

Antidesmoglein Autoantibodies in Silicosis Patients with No Bullous Diseases

Antidesmoglein Autoantibodies in Silicosis Patients with No Bullous Diseases

Bilateral Corneal Melting in a Patient with Paraneoplastic Pemphigus

Immunoapheresis in Paraneoplastic Pemphigus

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