Paraneoplastic Pemphigus With Anti–Laminin-332 Autoantibodies in a Patient With Follicular Dendritic Cell Sarcoma

Sugiura, Kazumitsu; Koga, Hiroshi; Ishikawa, Rei; Matsumoto, Takaaki; M, Matsubara; Hagiwara, Rika; Muro, Yoshinao; Hashimoto, Takashi; Akiyama, Masashi

doi:10.1001/2013.jamadermatol.512

Cited by 7 publications

(6 citation statements)

References 5 publications

(2 reference statements)

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“…One of the 11 cases of paraneoplastic pemphigus also demonstrated anti‐Ln‐332 IgG. A similar case of paraneoplastic pemphigus with additional anti‐Ln‐332 IgG has been described before …”

Section: Discussionmentioning

confidence: 65%

Keratinocyte footprint assay discriminates antilaminin‐332 pemphigoid from all other forms of pemphigoid diseases

et al. 2019

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Summary Background Antilaminin‐332 mucous membrane pemphigoid is a chronic severe pemphigoid disease characterized by autoantibodies to laminin‐332. At present no commercial assay is available to demonstrate antilaminin‐332 antibodies, and diagnosis relies on in‐house techniques with limited sensitivities. Objectives In order to move, keratinocytes cultured in vitro secrete laminin‐332 to attach to the culture dish. In that way, they leave behind a unique footprint trail of laminin‐332. We aimed to develop a sensitive and specific laboratory assay to determine antilaminin‐332 autoantibodies in patient serum based on binding of patient IgG to these unique footprints. Methods Normal human keratinocytes were grown on glass coverslips and incubated with patient or control serum for 1 h. The binding of IgG was then investigated by immunofluorescence. After validating the test for its ability to identify antilaminin‐332 autoantibodies it was converted into a daily available test based on binding of IgG to dried coverslips that can be stored frozen. The staining patterns of sera from patients with antilaminin‐332 pemphigoid were then compared with those of sera from patients with other autoimmune bullous diseases and normal human sera. Results IgG of all antilaminin‐332 pemphigoid sera (n = 16) bound to laminin‐332 footprints, while all normal human controls (n = 55) were negative. From the sera of patients with other diseases (n = 72) four sera tested positive. The footprint assay was also positive for sera that were negative by salt‐split skin analysis, demonstrating that it is a very sensitive technique. Conclusions The keratinocyte footprint assay is a fast and specific assay to confirm or rule out the presence of antilaminin‐332 autoantibodies. What's already known about this topic? Antilaminin‐332 mucous membrane pemphigoid is a severe form of pemphigoid, and patients may have an increased risk of malignancies. The diagnosis of antilaminin‐332 mucous membrane pemphigoid is complicated by the lack of specific commercial tests for antilaminin‐332 antibodies and can be confirmed only in specialized laboratories. Keratinocytes in culture need laminin‐332 for adhesion and migration and therefore deposit it on the bottom of the culture dish. What does this study add? The keratinocyte footprint assay detects antilaminin‐332 autoantibodies in patient serum using the native laminin‐332 produced by cultured keratinocytes. What is the translational message? The keratinocyte footprint assay is a fast and specific assay to confirm or rule out the presence of antilaminin‐332 autoantibodies.

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Section: Discussionmentioning

confidence: 65%

Keratinocyte footprint assay discriminates antilaminin‐332 pemphigoid from all other forms of pemphigoid diseases

et al. 2019

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“…This characteristic suggests that intra-abdominal FDC sarcomas may indicate a poor prognosis. Furthermore, in six cases, FDC sarcomas caused PNP were coexisting with myasthenia gravis (MG)[ 4 , 5 , 13 , 14 , 19 , 21 ]. Paraneoplastic neurologic syndromes are even rarer than other syndromes, occurring in approximately 0.01% of patients with cancer[ 33 ].…”

Section: Discussionmentioning

confidence: 99%

“…Previous reports have revealed that diseases associated with PNP predominantly underlie B-cell lymphoproliferative diseases, for example, non-Hodgkin lymphomas, chronic lymphocytic leukemia, and Castleman disease, such that 84% of neoplasms are associated with PNP[ 31 ]. Interestingly, we found that Castleman disease was involved in eight cases of PNP-associated FDC sarcoma (Table 1 )[ 4 , 8 , 19 , 21 , 24 , 26 , 29 ]. In China, PNP is frequently found in association with Castleman’s tumors[ 36 ].…”

Section: Discussionmentioning

confidence: 99%

Intra-abdominal inflammatory pseudotumor-like follicular dendritic cell sarcoma associated with paraneoplastic pemphigus: A case report and review of the literature

Zhuang¹,

Zhang²,

Qing-wen³

et al. 2020

WJCC

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BACKGROUD Follicular dendritic cell (FDC) sarcomas are rare neoplasms that occur predominantly in the lymph nodes. They can also occur extranodally. Extranodal FDC sarcomas most commonly present as solitary masses. Inflammatory pseudotumor (IPT)-like FDC sarcomas, a subcategory of FDC sarcomas, are rarer than other sarcoma subtypes. They are composed of spindle or ovoid neoplastic cells and exhibit an admixture of plasma cells and prominent lymphoplasmacytic infiltration. Paraneoplastic pemphigus (PNP), also known as paraneoplastic autoimmune multiorgan syndrome, is a rare autoimmune bullous disease that is associated with underlying neoplasms. PNP has a high mortality, and its early diagnosis is usually difficult. CASE SUMMARY We describe a 27-year-old woman who presented with stomatitis, conjunctivitis, and skin blisters and erosions as her first symptoms of PNP with an intra-abdominal IPT-like FDC sarcoma. The patient underwent surgical tumor resection and received tapering oral corticosteroid treatment. She showed no recurrence at the 1-year follow-up. CONCLUSION IPT-like FDC sarcomas are rare underlying neoplasms that have an uncommon association with PNP. PNP-associated FDC sarcomas predominantly occur in intra-abdominal sites and suggest a poor prognosis. Surgical resection is an essential and effective treatment for PNP and primary and recurrent FDC sarcomas.

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“…(Table ) . In recent years, new autoantigens including laminin 332, BP180, epiplakin and protein P200 have been identified . To detect anti‐envoplakin and anti‐periplakin antibodies, using full‐length recombinant proteins in IP and ELISA is highly effective .…”

Section: Detection Results Of Immunological Methodsmentioning

confidence: 99%

“…[74][75][76] In recent years, new autoantigens including laminin 332, BP180, epiplakin and protein P200 have been identified. [77][78][79][80] To detect anti-envoplakin and antiperiplakin antibodies, using full-length recombinant proteins in IP and ELISA is highly effective. 81 Nagata et al found autoantibodies in a majority of patients with PNP showed a strong reactivity with multiple recombinant proteins of envoplakin and periplakin, but few cases for the C-terminal homologous domain of periplakin (amino acid residues 1646-1756), which was detected by IB.…”

Section: Paraneoplastic Pemphigusmentioning

confidence: 99%

Immunological methods for the diagnosis of oral mucosal diseases

Sun

Zhong

et al. 2019

Br J Dermatol

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Paraneoplastic Pemphigus With Anti–Laminin-332 Autoantibodies in a Patient With Follicular Dendritic Cell Sarcoma

Cited by 7 publications

References 5 publications

Keratinocyte footprint assay discriminates antilaminin‐332 pemphigoid from all other forms of pemphigoid diseases

Keratinocyte footprint assay discriminates antilaminin‐332 pemphigoid from all other forms of pemphigoid diseases

Intra-abdominal inflammatory pseudotumor-like follicular dendritic cell sarcoma associated with paraneoplastic pemphigus: A case report and review of the literature

Immunological methods for the diagnosis of oral mucosal diseases

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