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Paraneoplastic Pemphigus (paraneoplastic + pemphigus)

Distribution by Scientific Domains
Medical Sciences100%
Distribution within Medical Sciences
Dermatology64%
Hematology14%

Selected Abstracts

Paraneoplastic Pemphigus with Bronchiolitis Obliterans in a Child

PEDIATRIC DERMATOLOGY, Issue 3 2003
M.D., Winnie A. Mar
Most cases have been reported in adults and the number of childhood cases in the current literature is limited. We describe a young patient with PNP who was initially misdiagnosed as having recurrent Stevens,Johnson syndrome. This patient had an underlying inflammatory myofibroblastic tumor and subsequently developed fatal progressive bronchiolitis obliterans. [source]

Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome

INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 2 2009
FNASC, FRAS(LOND.), Virendra N. Sehgal MD
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, interfacial dermatitis, and epidermal exocytosis can be seen. Furthermore, the deposition of immunoglobulin G (IgG) and complement in the epidermal intercellular spaces, detected by direct and/or indirect immunofluorescence, is equally crucial for confirming the diagnosis. [source]

Paraneoplastic pemphigus in association with B-cell lymphocytic leukemia and hepatitis C: favorable response to intravenous immunoglobulins and prednisolone

INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 7 2007
Mousumee Nanda MD
No abstract is available for this article. [source]

Paraneoplastic pemphigus: a review of the literature

ORAL DISEASES, Issue 4 2000
CM Allen
Paraneoplastic pemphigus is a relatively rare but highly significant acquired mucocutaneous disorder. The condition typically presents in patients with previously diagnosed lymphoreticular disease, primarily malignancies (non-Hodgkin's lymphoma; chronic lymphocytic leukemia).Multiple sites of involvement are common, with the skin affected by a polymorphous eruption that includes lichenoid plaques as well as bullae. The oral lesions occur in all cases and present as painful widespread shallow ulcers with hemorrhagic crusting of the lipS. Conjunctival involvement is also seen and may result in scarring. Pulmonary lesions are an ominous sign. Histopathologically, intraepithelial and subepithelial clefting associated with interface dermatitis are seen. A variety of autoantibodies directed against desmoplakins and desmogleins can be identified with immunoprecipit-ation studies. Treatment with immunosuppressive agents may result in some resolution of the disease, but the prognosis for paraneoplastic pemphigus is considered to be poor. [source]

Paraneoplastic pemphigus associated with systemic mastocytosis,

AMERICAN JOURNAL OF HEMATOLOGY, Issue 12 2009
Lydia R.J. Eccersley
No abstract is available for this article. [source]

Autoantibody production from a thymoma and a follicular dendritic cell sarcoma associated with paraneoplastic pemphigus

BRITISH JOURNAL OF DERMATOLOGY, Issue 3 2005
J. Wang
Summary Background, Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease. We previously reported that B cells in a Castleman tumour associated with PNP produced autoantibodies. However, it is uncertain whether the production of autoantibodies from the associated tumour is a common mechanism in PNP. Objectives, To investigate autoantibody production in a thymoma and a follicular dendritic cell sarcoma that were excised from two patients with PNP. Methods, Tumour cells were cultured, and their surface markers were identified. Indirect immunofluorescence, immunoblotting and enzyme-linked immunosorbent assay (ELISA) using culture media from the tumours were used to detect PNP autoantibodies. Results, B cells with markers (CD22+, surface membrane IgG+ and surface membrane IgM+) of mature B lymphocytes constituted a proportion of cultured tumour cells in both tumours. Western blot showed that the medium from both the thymoma and the follicular dendritic cell sarcoma cells recognized 190-kDa periplakin and 210-kDa envoplakin bands of human epithelial proteins as well as recombinant linker regions of periplakin, envoplakin, desmoplakin and bullous pemphigoid antigen 1. ELISA was positive for antidesmoglein 3 antibody. Conclusions, The presence and localization in tumours of B-lymphocyte clones against proteins of the plakin family and desmoglein 3 in skin may not be confined to PNP with Castleman disease, but is possibly a common mechanism in PNP associated with various tumours. [source]

Paraneoplastic pemphigus associated with follicular lymphoma

BRITISH JOURNAL OF HAEMATOLOGY, Issue 4 2009
Florence Lachenal
No abstract is available for this article. [source]

A case of paraneoplastic pemphigus associated with Castleman's disease presenting as erosive lichen planus

CLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 8 2009
H. L. Tey
Summary Paraneoplastic pemphigus (PNP) is a debilitating chronic blistering mucocutaneous disease associated with an underlying neoplasm and a poor prognosis. We present a patient who had been diagnosed and treated for recalcitrant erosive mucocutaneous lichen planus for 3 years with little success. Further investigations established the diagnosis of PNP with underlying Castleman's disease. This case highlights the importance of repeated investigations to exclude PNP in patients with recalcitrant mucosal erosions, especially if atypical features are present. [source]

Rituximab: Applications in dermatology

INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 10 2006
Mitra M. Fatourechi
Rituximab is a chimeric anti-CD20 monoclonal antibody which has been used extensively for B-lymphocytic malignancies. In addition, applications for autoimmune diseases have emerged in recent years. Case reports support the use of rituximab in certain dermatologic conditions, including paraneoplastic pemphigus, pemphigus vulgaris, graft versus host disease, and cutaneous B-cell malignancies. Clinical trials are lacking and would be an appropriate next step. [source]

Paraneoplastic pemphigus: a review of the literature

ORAL DISEASES, Issue 4 2000
CM Allen
Paraneoplastic pemphigus is a relatively rare but highly significant acquired mucocutaneous disorder. The condition typically presents in patients with previously diagnosed lymphoreticular disease, primarily malignancies (non-Hodgkin's lymphoma; chronic lymphocytic leukemia).Multiple sites of involvement are common, with the skin affected by a polymorphous eruption that includes lichenoid plaques as well as bullae. The oral lesions occur in all cases and present as painful widespread shallow ulcers with hemorrhagic crusting of the lipS. Conjunctival involvement is also seen and may result in scarring. Pulmonary lesions are an ominous sign. Histopathologically, intraepithelial and subepithelial clefting associated with interface dermatitis are seen. A variety of autoantibodies directed against desmoplakins and desmogleins can be identified with immunoprecipit-ation studies. Treatment with immunosuppressive agents may result in some resolution of the disease, but the prognosis for paraneoplastic pemphigus is considered to be poor. [source]

Autoantibody production from a thymoma and a follicular dendritic cell sarcoma associated with paraneoplastic pemphigus

BRITISH JOURNAL OF DERMATOLOGY, Issue 3 2005
J. Wang
Summary Background, Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease. We previously reported that B cells in a Castleman tumour associated with PNP produced autoantibodies. However, it is uncertain whether the production of autoantibodies from the associated tumour is a common mechanism in PNP. Objectives, To investigate autoantibody production in a thymoma and a follicular dendritic cell sarcoma that were excised from two patients with PNP. Methods, Tumour cells were cultured, and their surface markers were identified. Indirect immunofluorescence, immunoblotting and enzyme-linked immunosorbent assay (ELISA) using culture media from the tumours were used to detect PNP autoantibodies. Results, B cells with markers (CD22+, surface membrane IgG+ and surface membrane IgM+) of mature B lymphocytes constituted a proportion of cultured tumour cells in both tumours. Western blot showed that the medium from both the thymoma and the follicular dendritic cell sarcoma cells recognized 190-kDa periplakin and 210-kDa envoplakin bands of human epithelial proteins as well as recombinant linker regions of periplakin, envoplakin, desmoplakin and bullous pemphigoid antigen 1. ELISA was positive for antidesmoglein 3 antibody. Conclusions, The presence and localization in tumours of B-lymphocyte clones against proteins of the plakin family and desmoglein 3 in skin may not be confined to PNP with Castleman disease, but is possibly a common mechanism in PNP associated with various tumours. [source]

An evaluation of the usefulness of mycophenolate mofetil in pemphigus

BRITISH JOURNAL OF DERMATOLOGY, Issue 1 2003
A.M. Powell
SummaryBackground Pemphigus is a group of autoimmune blistering diseases of the skin and/or mucous membranes requiring management with immunosuppressive therapy. The optimal therapeutic regimen would rapidly induce remission and maintain effectiveness with minimal adverse effects in the long term. Objectives The present study describes our experience of the addition of mycophenolate mofetil (MMF) to prednisolone in the management of severe, refractory pemphigus. Methods Patients with active, refractory pemphigus were treated with MMF. Our series included 12 cases of pemphigus vulgaris, four cases of pemphigus foliaceous and one case of paraneoplastic pemphigus. All patients were monitored to assess disease control and mycophenolate toxicity. Results Of the 17 cases, MMF has been of benefit to 12. MMF was well tolerated and there were no treatment withdrawals because of safety concerns. Conclusions We found that MMF permitted a reduction in prednisolone dosage without disease relapse. [source]

A case of paraneoplastic pemphigus associated with Castleman's disease presenting as erosive lichen planus

CLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 8 2009
H. L. Tey
Summary Paraneoplastic pemphigus (PNP) is a debilitating chronic blistering mucocutaneous disease associated with an underlying neoplasm and a poor prognosis. We present a patient who had been diagnosed and treated for recalcitrant erosive mucocutaneous lichen planus for 3 years with little success. Further investigations established the diagnosis of PNP with underlying Castleman's disease. This case highlights the importance of repeated investigations to exclude PNP in patients with recalcitrant mucosal erosions, especially if atypical features are present. [source]