Graft-versus-Host-Like Mucocutaneous Eruptions with Serological Features of Paraneoplastic Pemphigus and Systemic Lupus erythematosus in a Patient with Non-Hodgkin’s Lymphoma

Mahler, Vera; Antoni, Christian; Anhalt, Grant J.; Koch, Hans‐Joachim; Peters, K.‐P.; Manger, Bernhard; Kalden, J. R.; Hornstein, O. P.

doi:10.1159/000017964

Cited by 18 publications

(14 citation statements)

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“…Subsequent reports delineated a wider spectrum of pathologic findings associated with this syndrome. Although a subset of patients showed acantholysis and intraepithelial blister formation classically associated with pemphigus vulgaris [3,42] ( Figure 5), others showed interface vacuolar changes [3,34,43], lichenoid infiltrates, and occasionally dyskeratotic keratinocytes consistent with lichen planus [5,9,32,35,43], erythema multiforme [44], or GVHD [34,45] (Figure 6). …”

Section: Histologic Findingsmentioning

confidence: 99%

Paraneoplastic autoimmune multiorgan syndrome: Review of the literature and support for a cytotoxic role in pathogenesis

2006

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Paraneoplastic autoimmune multiorgan syndrome (PAMS), first described as paraneoplastic pemphigus in 1990, is an autoimmune blistering disease associated with neoplasia. Patients with this rare disorder have severe blistering and painful erosions of the oral cavity and various other cutaneous findings ranging from classic pemphigus vulgaris-like erosions to targetoid lesions resembling erythema multiforme and papular to more confluent lichenoid eruptions. This syndrome involves multiple organ systems, and its high rate of mortality often stems from constrictive bronchiolitis obliterans. The histologic findings are as diverse as the clinical presentation, often making diagnosis difficult initially. Immunodermatologic and serologic laboratory findings typically establish the diagnosis. These results can be confirmed with immunoprecipitation profiling of specific molecular weight protein markers. The proposed pathogenesis of PAMS continues to evolve, and recent reports implicate the involvement of cell-mediated, cytotoxic immunity, in addition to humoral autoantibodies. This review characterizes and summarizes the clinical, pathologic, and immunohistologic features of PAMS and outlines the possible role of cytotoxic T lymphocytes in the pathogenesis of this syndrome.

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Section: Histologic Findingsmentioning

confidence: 99%

Paraneoplastic autoimmune multiorgan syndrome: Review of the literature and support for a cytotoxic role in pathogenesis

2006

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“…The patient also showed good response to oral corticotherapy, with a prolonged remission. The deep vein thrombosis was, in our opinion, another paraneoplastic manifestation and the presence of antinuclear antibodies an evidence of a patent dysimmunoreactivity 23 …”

Section: Discussionmentioning

confidence: 59%

“…The deep vein thrombosis was, in our opinion, another paraneoplastic manifestation and the presence of antinuclear antibodies an evidence of a patent dysimmunoreactivity. 23 PNP manifested has lichenoid eruptions is more common then previously accepted and the potential role of cell-mediated immunity at the DEJ as a participant in the pathogenesis of PNP can't be underestimated.…”

Section: Immunoprecipitationmentioning

confidence: 97%

Paraneoplastic pemphigus with clinical features of lichen planus associated with low‐grade B cell lymphoma

et al. 2005

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Background Neoplasia-induced lichen planus is described as a cell-mediated reaction to unknown epithelial antigens. Paraneoplastic pemphigus (PNP), characterized by the presence of a specific array of autoantibodies, probably represents a different form of presentation of the same autoimmune syndrome where the mucocutaneous expression depends on the dominant pathologic mechanism. Conclusion PNP has different forms of presentation and the lack of a consensus about diagnostic criteria may contribute to underdiagnosed cases. Advances on the knowledge of the sensitivity and specificity of diagnostic criteria have allowed a better accuracy of diagnosis. Methods

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“…The palmar involvement is usually observed [4]. Lichen planus-like lesions localized on skin, nails and/or mucosa resemble lichen planus, target-like lesions resemble erythema multiforme, and bullous lesions and erosive lesions resemble PV and bullous pemphigoid [4,15,66,76,77,[83][84][85][86][87]. Cutaneous lesions mimicking graft versus host disease or Stevens-Johnson syndrome may also be observed [86,87].…”

Section: Clinical Featuresmentioning

confidence: 99%

Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

Kılıç¹

2018

Autoimmune Bullous Diseases

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Pemphigus is a group of autoimmune blistering disorders associated with autoantibodies against the keratinocyte cell surface. Pemphigus has three major variants: pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP) which all have further subtypes. The variants of pemphigus are classified depending upon the clinical and histological features, immunofluorescence staining pattern, and autoantibody profile of the disease. The onset and course of pemphigus appear on the basis of interaction between genetic predisposition and various triggering factors. Pemphigus vulgaris is the most commonly seen and representative clinical form of pemphigus. Together with clinical manifestations, the histopathological and immunopathological data support the diagnosis. As though some pemphigus variants, particularly pemphigus vulgaris and paraneoplastic pemphigus, have a mortality risk, early diagnosis is necessary and onset of treatment should be promptly initiated. In this chapter, firstly, classification of pemphigus is described. After then, clinical features, histopathological and immunopathological findings, target antigens, etiopathogenesis and comorbidities of each pemphigus variant are discussed briefly.Keywords: pemphigus, pemphigus variants, clinical manifestations, pathogenesis, histopathology, immunopathology IntroductionPemphigus is a distinct organ-specific autoimmune blistering disorder involving skin and mucous membranes associated with autoantibodies directed against desmosomes-intercellular adhesive molecule complex localized on the keratinocyte cell surface [1][2][3][4].© 2018 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Pemphigus has three major variants, which are classified depending on the basis of the clinical, histological features, immunofluorescence staining pattern and autoantibody profile of the disease including pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP), which all have further subtypes. Less frequently seen and newer variants of pemphigus include IgA pemphigus (IGAP) and pemphigus herpetiformis (PH) [1-6].The term "pemphigus" origins from the Greek word "pemphix", which has a meaning of "blister" [1]. It is a chronic potentially life-threating bullous disorder if not treated on time [4,7,8]. The phenotypes of pemphigus represent a complex spectrum with multiple genetic and environmental factors playing a role in disease pathogenesis [9,10].Together with clinical manifestations, the histopathological and immunopathological data support the diagnosis of the disease. The best site for the cutaneous biopsy for the appropriate histopathological examination is a fresh (< 24 h) small vesicle or 1/3 of the peripheral portion of the blister including the perilesional normal appearing s...

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Graft-versus-Host-Like Mucocutaneous Eruptions with Serological Features of Paraneoplastic Pemphigus and Systemic Lupus erythematosus in a Patient with Non-Hodgkin’s Lymphoma

Cited by 18 publications

References 22 publications

Paraneoplastic autoimmune multiorgan syndrome: Review of the literature and support for a cytotoxic role in pathogenesis

Paraneoplastic autoimmune multiorgan syndrome: Review of the literature and support for a cytotoxic role in pathogenesis

Paraneoplastic pemphigus with clinical features of lichen planus associated with low‐grade B cell lymphoma

Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

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