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?

Beutner, Ernst H.; Pelton, Stephen I.; Hashimoto, Takashi; Xu, Yuelin; Plunkett, Richard W.; Korman, Neil J.; Helm, Thomas N.; Jabłońska, Stefania

doi:10.1067/mjd.2002.123486

Cited by 11 publications

(10 citation statements)

References 23 publications

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“…From the literature search, we included an additional 124 articles, reporting 208 patients with PAMS published between January 1, 1996, and August 1, 2012 . A single article reporting patients from our institution was excluded from analysis to avoid duplicate reporting .…”

Section: Resultsmentioning

confidence: 99%

Diagnostic imaging in paraneoplastic autoimmune multiorgan syndrome: retrospective single site study and literature review of 225 patients

et al. 2014

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Diagnostic imaging is frequently utilized in PAMS with unknown malignancy. Both conventional CT and 18F-FDG PET/CT are likely to detect the typical underlying neoplasms. Relative to conventional CT, 18F-FDG PET/CT may provide additional useful information regarding prognosis for the likely underlying malignancies, although there is a paucity of reports describing the use of this modality for this purpose.

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Section: Resultsmentioning

confidence: 99%

Diagnostic imaging in paraneoplastic autoimmune multiorgan syndrome: retrospective single site study and literature review of 225 patients

et al. 2014

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“…The majority of patients succumb to sepsis, respiratory failure, or their underlying neoplasm. Beutner et al 68 showed that the presence of cell surface antibodies detectable by complement fixing IIF was associated with fatal disease. These antibodies were absent in PAMS cases with long-term survival.…”

Section: Managementmentioning

confidence: 99%

Paraneoplastic autoimmune multiorgan syndrome: 20 years after

et al. 2011

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The purpose of this review is to provide insight and clarification in the quandary of classification and delineate clinical and histological features and pathophysiology of paraneoplastic pemphigus. This is a paraneoplastic disease of epithelial autoimmunity and adhesion originally described by Dr. Anhalt in 1990. Paraneoplastic pemphigus represents only one manifestation of the heterogeneous autoimmune syndrome in which patients, in addition to small airways occlusion, may display a spectrum of at least five clinical variants of the mucocutaneous disease [i.e. pemphigus-like, pemphigoid-like, erythema multiforme-like, graft-versus-host disease-like, and lichen planus-like, termed paraneoplastic autoimmune multiorgan syndrome (PAMS)]. There is a need for the expanded, inclusive classification of diverse mucocutaneous and respiratory presentations of PAMS. Multiple specific effectors of humoral and cellular autoimmunity mediating epithelial damage have been identified. An update of advances in clinical and basic research on PAMS and in management and overall prognosis of PAMS is provided.

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“…It is not uncommon to ¢nd in some myasthenic patients clinical manifestations of pemphigus (reviewed in Kaplan and Callen, 1983). In such patients, autoantibodies can be directed against both desmosomal and neuromuscular antigens (McKee et al, 1978;Beutner et al, 2002). Likewise, AChR accumulate at both desmosomal and neuromuscular junctions (Engel et al, 1977;Grando et al, 1995b).…”

Section: Ach As a Cytotransmitter Regulating Keratinocyte Adhesionmentioning

confidence: 97%

“…Likewise, AChR accumulate at both desmosomal and neuromuscular junctions (Engel et al, 1977;Grando et al, 1995b). Recently, Dr. Beutner's group proposed that in pemphigus associated with malignancies, autoimmunity may serve primarily as a defense mechanism against such systemic complications, although some forms of it, such as the autoantibodies to AChR, can cause the death of patients with PAMS (Beutner et al, 2002). Approximately 70% of MG patients have thymitis, and approximately 10% develop thymoma (reviewed in Marx et al, 1992).…”

Section: Ach As a Cytotransmitter Regulating Keratinocyte Adhesionmentioning

confidence: 98%

New Approaches to the Treatment of Pemphigus

Grando

2004

Journal of Investigative Dermatology Symposium Proceedings

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In pemphigus vulgaris, treatment with systemic glucocorticosteroids is life saving; it may, however, cause severe side effects, including death. A patient with pemphigus vulgaris and myasthenia gravis was treated for approximately five years with the cholinomimetic Mestinon (pyridostigmine bromide), Imuran (azathioprine), and a topical corticosteroid gel before the need to introduce systemic glucocorticosteroids. Because activation of keratinocyte acetylcholine receptors also has been shown to abolish pemphigus IgG-induced acantholysis in cultured keratinocyte monolayers, a clinical trial of Mestinon was initiated in patients with active pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic autoimmune multiorgan syndrome (also known as paraneoplastic pemphigus). First results indicate that nonsteroidal treatment of pemphigus is possible. Mestinon may be used to slow down progression of the disease and to treat mild cases with chronic lesions on limited areas. Stimulation of the keratinocyte- acetylcholine axis may lead to a therapeutic effect through any of the following mechanisms: (1) stimulating keratinocyte cell-to-cell attachment; (2) accelerating reepithelialization; and (3) competing with the disease-causing pemphigus antibodies, preventing them from attachment to keratinocytes. Glucocorticosteroids and various types of steroid-sparing drugs used to treat pemphigus exhibit cholinergic side effects, including effects on expression and function of keratinocyte adhesion molecules, that are very similar to those produced by the cholinomimetic drugs. Further elucidation of the mechanisms underlying therapeutic efficacy of antiacantholytics may shed light on the immunopharmacological mechanisms of pemphigus antibody-induced acantholysis.

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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?

Cited by 11 publications

References 23 publications

Diagnostic imaging in paraneoplastic autoimmune multiorgan syndrome: retrospective single site study and literature review of 225 patients

Diagnostic imaging in paraneoplastic autoimmune multiorgan syndrome: retrospective single site study and literature review of 225 patients

Paraneoplastic autoimmune multiorgan syndrome: 20 years after

New Approaches to the Treatment of Pemphigus

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