Laboratory diagnosis of paraneoplastic pemphigus

Poot, Andreas A.; Diercks, Gilles F.H.; Kramer, Duco; Schepens, Isabelle; Klunder, G.; Hashimoto, Takashi; Borradori, Luca; Jonkman, Marcel F.; Pas, Hendrikus

doi:10.1111/bjd.12479

Cited by 96 publications

(111 citation statements)

References 40 publications

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“…Immunoprecipitation is a serum-based test that demonstrates the presence of multiple autoantibodies quickly with high sensitivity and specificity. 7 It is a favorable technique to confirm the diagnosis of PNP in patients with severe stomatitis when performing a skin biopsy may be difficult. 6 Classically, DIF reveals IgG and C3 deposits in an intercellular and linear basement membrane zone pattern, differentiating PNP from other types of pemphigus.…”

Section: Discussionmentioning

confidence: 99%

“…4 Indirect immunofluorescence is performed on rat bladder epithelium because plakins are strongly expressed in rodent epithelium and autoantibodies to plakins are most specific to PNP. 7 The histological findings of PNP are variable, ranging from suprabasal acantholysis to lichenoid dermatitis depending on the clinical morphologic characteristics of the lesions. For this reason, a combination of clinical and histopathological criteria are used for diagnosis.…”

Section: Discussionmentioning

confidence: 99%

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Polymorphic Blistering Eruption and Stomatitis in a Patient With Non-Hodgkin Lymphoma

2015

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A man in his 70s with a history of T2N2 melanoma, locally metastatic squamous cell carcinoma, and non-Hodgkin follicular lymphoma presented with weakness, a blistering erythematous eruption, and painful oral lesions of 1 month's duration. On physical examination, the patient was in severe discomfort with limited movement owing to cutaneous pain. He was cachectic and had widespread erosions and tense and flaccid bullae with overlying crust affecting his trunk and extremities (including the palms and soles) but sparing his scalp (Figure, A). He had severe stomatitis with localized hemorrhagic lesions, shaggy erosions on the buccal and gingival mucosa, and erythematous macules on his hard and soft palates (Figure, B). Findings from laboratory tests, including a complete blood cell count and a comprehensive metabolic panel, were unremarkable except for mild anemia. Results from a computed tomographic scan of the chest and neck showed adenopathy suspicious for lymphoproliferative disorder or potentially metastatic disease. A punch biopsy for histopathologic examination (Figure, C) and direct immunofluorescence (DIF) was performed, and serum was tested for indirect immunofluorescence (IIF) on rodent epithelium (Figure, D).

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Polymorphic Blistering Eruption and Stomatitis in a Patient With Non-Hodgkin Lymphoma

2015

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“…Western blotting of normal human epidermal extracts using the patient's serum revealed protein bands corresponding to 190-kDa periplakin and 210-kDa envoplakin. [9][10][11][12] On the basis of these findings, he was diagnosed with PNP.…”

Section: Case Reportmentioning

confidence: 99%

Rituximab Monotherapy and Rituximab-Containing Chemotherapy Were Effective for Paraneoplastic Pemphigus Accompanying Follicular Lymphoma, but not for Subsequent Bronchiolitis Obliterans

Hirano

Higuchi

Yuki

et al. 2015

J Clin Exp Hematopathol

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A 60-year-old male patient suffered from mild exertional dyspnea, wheezing, and systemic blisters. He was diagnosed with paraneoplastic pemphigus (PNP) with follicular lymphoma in the pancreas head and pelvic cavity. He was first treated with eight cycles of rituximab; his blisters and erosions gradually improved and highly elevated levels of auto-antibodies related to PNP gradually decreased to normal levels. However, obstructive and restrictive respiratory failure still progressed. Computed tomography of the inspiratory and expiratory phases revealed obstructive pulmonary disorder, leading to a diagnosis of bronchiolitis obliterans (BO). The patient underwent plasma exchange and was repeatedly treated with rituximab monotherapy and rituximab-containing chemotherapies, but died 7 months after the diagnosis of BO. Early introduction of rituximabcontaining regimens may be necessary to prevent the development of BO accompanying PNP. However, when a diagnosis of PNP-related BO is made, lung transplantation may also be considered for patients in whom rituximab-containing regimens are effective for PNP. 〔J Clin Exp Hematop 55(2) : 83-88, 2015〕

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“…However, rat bladder IIF in combination with IB offers an easier and more accurate alternative to IP [59] . Furthermore, the laboratory data should be related to the clinical features [38][39][40] .…”

Section: Diagnosismentioning

confidence: 99%

Paraneoplastic pemphigus: A trait d’union between dermatology and oncology

Didona¹,

Didona²,

Richetta³

et al. 2015

Adv Mod Oncol Res

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Paraneoplastic pemphigus is a rare autoimmune disease of the skin associated with neoplasm. Nowadays, the pathogenesis of paraneoplastic pemphigus is not fully understood. Due to its rarity, various criteria have been proposed for the diagnosis. For this reason, several diagnostic methods have been considered useful for the diagnosis of paraneoplastic pemphigus including indirect immunofluorescence, direct immune of fluorescence, immunoprecipitation, immunoblotting, and enzyme-linked immunosorbent assay (ELISA). However, the polymorphic clinical features and the various results of laboratory tests and pathological evaluation present a challenge for the clinicians.

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Laboratory diagnosis of paraneoplastic pemphigus

Cited by 96 publications

References 40 publications

Polymorphic Blistering Eruption and Stomatitis in a Patient With Non-Hodgkin Lymphoma

Polymorphic Blistering Eruption and Stomatitis in a Patient With Non-Hodgkin Lymphoma

Rituximab Monotherapy and Rituximab-Containing Chemotherapy Were Effective for Paraneoplastic Pemphigus Accompanying Follicular Lymphoma, but not for Subsequent Bronchiolitis Obliterans

Paraneoplastic pemphigus: A trait d’union between dermatology and oncology

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