Paraneoplastic pemphigus without an underlying neoplasm

Park, G.T.; Lee, J.H.; Yun, Sook Jung; Lee, S.C.

doi:10.1111/j.1365-2133.2006.07605.x

Cited by 29 publications

(15 citation statements)

References 25 publications

(47 reference statements)

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“…However, a defining feature of PNP is underlying malignancy and rapid disease progression with treatment resistance. There are several case reports of PNP without identifiable malignancy, however, these are consistently characterised by the rapid demise of the patient before complete malignancy screening can be performed and occult malignancy proven . Our patient had no malignancy on screening, responded rapidly and completely to treatment, and remains stable 2 years later without occult malignancy declaring itself.…”

Section: Discussionmentioning

confidence: 65%

Circulating plakin autoantibodies in a patient with erythema multiforme major: Are they pathogenic or a manifestation of epitope spreading?

Ellis

Sidhu

2014

Australasian Journal of Dermatology

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Erythema multiforme is a well-recognised entity but its pathogenesis remains elusive. Theories hypothesise a cell-mediated immune pathogenesis, however recent case reports have observed autoantibodies to the plakin family of proteins, suggesting a role for the humoral immune system. We present a case of erythema multiforme major with circulating desmoplakin autoantibodies in a 36-year old woman who was previously diagnosed with pemphigoid gestationis. The close correlation between the concentration of these autoantibodies and the severity of clinical disease strongly suggests a pathogenic role in her disease. As previous case reports have proposed, these autoantibodies may be directly pathogenic. Alternatively, the epiphenomenon of epitope spreading must be considered in the subset of patients with erythema multiforme major.

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Section: Discussionmentioning

confidence: 65%

Circulating plakin autoantibodies in a patient with erythema multiforme major: Are they pathogenic or a manifestation of epitope spreading?

Ellis

Sidhu

2014

Australasian Journal of Dermatology

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“…The medical records of 12 Korean patients with PNP diagnosed between 1993 and 2011 were retrospectively reviewed to evaluate the clinical manifestations, histological and immunopathological features, autoantibody profiles, associated neoplasia and prognosis. Seven of 12 cases have been reported previously …”

Section: Methodsmentioning

confidence: 99%

“…Seven of 12 cases have been reported previously. [6][7][8][9][10][11][12] The 12 cases were diagnosed by the following criteria: (i) clinical features, including the presence of severe mucosal involvement or polymorphous cutaneous eruption; (ii) characteristic histological features of the skin or mucosal eruption; (iii) the presence of autoantibodies in DIF or IIF studies; (iv) detection of anti-plakin or anti-Dsg autoantibodies in immunoblotting or immunoprecipitation; and (v) the presence of associated neoplasm. Diagnosis of PNP required four of five criteria, including criteria (i) and (ii) ( Table 1).…”

Section: Methodsmentioning

confidence: 99%

“…Surprisingly, no underlying neoplasm was detected in one of our cases (patient 7) over the 8-year follow-up period. 9 Despite the lack of an association with a neoplasm, the case was diagnosed as PNP based on the mucocutaneous manifestations and results of immunofluorescence, immunoblotting and immunoprecipitation studies. In approximately two-thirds of PNP patients, the underlying neoplasms precede the clinical manifestations of PNP.…”

Section: Associated Neoplasmsmentioning

confidence: 99%

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Retrospective analysis of 12 Korean patients with paraneoplastic pemphigus

Youngik

Nam

Lee

et al. 2012

The Journal of Dermatology

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Paraneoplastic pemphigus (PNP) is a rare, life-threatening, autoimmune, mucocutaneous blistering disease associated with neoplasia. Both humoral and cellular immunity are involved in the pathogenesis of PNP. Characteristically, PNP has a diverse spectrum of clinical and immunopathological features. We retrospectively analyzed 12 Korean patients with PNP who were diagnosed between 1993 and 2011. We performed analysis of the clinical features, clinical outcomes, underlying neoplasia, histological features and laboratory findings. All of the patients except one had severe mucosal involvement. Two patients had only mucosal lesions but no cutaneous involvement was observed. Erythema multiforme or lichen planus-like eruptions rather than bullous lesions were more commonly observed skin rashes. The most common histological features were interface dermatitis and apoptotic keratinocytes. There were associated hematological-related neoplasms in 11 patients, with Castleman's disease (n = 4) as the most frequent. Twelve patients were followed for 5-148 months (mean, 43.0). The prognosis depended on the nature of the underlying neoplasm. Six patients died due to respiratory failure (n = 3), postoperative septicemia (n = 1), lymphoma (n = 1) and sarcomatosis (n = 1). The 2-year survival rate was 50.0%, and the median survival period after diagnosis was 21.0 months. Immunoblotting was performed in 12 patients and autoantibodies to plakins were detected in 11 patients. The results of this study demonstrated the clinical, histological and immunological diversity of PNP. Widely accepted diagnostic criteria that account for the diversity of PNP are needed.

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“…There are reports of carcinomas of the lung, colon, hepatocellular, pancreas, and cervix [12][13][14]. In one case no underlying neoplasm was found over 8 years of follow-up [15]. The absence of common neoplasms is notable.…”

Section: Discussionmentioning

confidence: 98%