2009
DOI: 10.1111/j.1365-4632.2009.03995.x
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Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome

Abstract: Paraneoplastic pemphigus is the term used for an exclusive subset of pemphigus. The clinical lesions may resemble pemphigus, pemphigoid, erythema multiforme, graft-vs.-host disease, or lichen planus. A common denominator in all patients is the concomitant occurrence of either occult or confirmed systemic neoplasm. It is imperative to confirm the diagnosis through microscopy, where intraepidermal suprabasal cleavage, epidermal acantholysis, dyskeratotic keratinocytes and vacuolar changes in the basal epidermis,… Show more

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Cited by 93 publications
(92 citation statements)
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“…varied, typically begins with a florid, painful eruption of mucous membrane lesions, which may involve the oropharynx, nasopharynx, tongue, vermillion of the lips (Figure 1), conjunctiva, anogenital region (Figure 2), or esophagus [1,4,10,11]. Oral mucosal lesions were usually more extensive than that of pemphigus vulgaris or erythema multiforme.…”
Section: Histopathologymentioning
confidence: 99%
“…varied, typically begins with a florid, painful eruption of mucous membrane lesions, which may involve the oropharynx, nasopharynx, tongue, vermillion of the lips (Figure 1), conjunctiva, anogenital region (Figure 2), or esophagus [1,4,10,11]. Oral mucosal lesions were usually more extensive than that of pemphigus vulgaris or erythema multiforme.…”
Section: Histopathologymentioning
confidence: 99%
“…Lymphoproliferative disorders predominate, with non-Hodgkin's lymphoma, chronic lymphocytic leukemia, Castelman's tumor, and thymoma being the most common. 7 Of note, PNP with monoclonal gammopathy has been reported as well. 8 Non-hematologic neoplasms associated with PNP are liposarcoma, leiomyosarcoma, adenocarcinoma of the breast, colon, pancreas and other organs, basal cell carcinoma, and malignant melanoma.…”
Section: Discussionmentioning
confidence: 96%
“…However, PNP can affect every age group, including children and adolescents [8][9][10] . PNP appears to affect males and females equally [2] .…”
Section: Epidemiologymentioning
confidence: 94%
“…PNP is always associated with neoplasm, among which B-cell lymphomas and other hematological malignant diseases are most common [2] . In 2001, Nguyen et al suggested the term "paraneoplastic autoimmune multiorgan syndrome" (PAMS) as several organs are affected and autoantibodies bind several tissues [3] .…”
Section: Receivedmentioning
confidence: 99%