718 results match your criteria Pemphigus Paraneoplastic


Paraneoplastic Pemphigus: An Indication for Treatment in Chronic Lymphocytic Leukemia.

Cureus 2020 May 27;12(5):e8316. Epub 2020 May 27.

Oncology, Mount Sinai Medical Center, Brooklyn, USA.

Paraneoplastic disorders are rare multiorgan diseases associated with hematological malignancies such as chronic lymphocytic leukemia (CLL). Some of these paraneoplasms manifest as cutaneous lesions, appearing as a simple rash, ulcers or skin thickening. The pathogenesis for this process has been described as development of certain autoimmune reactions against cell wall antigens and proteins. Read More

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http://dx.doi.org/10.7759/cureus.8316DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7320649PMC

Erythroderma: a prospective study of 309 patients followed for 12 years in a tertiary center.

Sci Rep 2020 Jun 17;10(1):9774. Epub 2020 Jun 17.

Division of Clinical Dermatology, Hospital das Clínicas, University of São Paulo Medical School, São Paulo, Brazil.

Erythroderma is characterized by erythema and scaling affecting more than 80% of the body surface area. It is potentially life-threatening, and diagnosis of the underlying disease is a challenge. Despite laboratory improvements, many cases remain idiopathic. Read More

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http://dx.doi.org/10.1038/s41598-020-66040-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7300014PMC

Autoimmune mucocutaneous blistering diseases in the south of Italy: a 25-year retrospective study on 169 patients.

J Oral Pathol Med 2020 Jun 12. Epub 2020 Jun 12.

Department of Neurosciences, Reproductive Sciences and Dentistry, Federico II University of Naples, Naples, Italy.

Background: Autoimmune mucocutaneous blistering diseases (AMBDs) represent a heterogeneous group of organ-specific and potentially life-threatening diseases. We sought to determine the relationship between clinical remission and therapeutic regimens with clinical type and phenotype of AMBDs, as well as clinical outcomes achieved based on different therapeutic regimens.

Methods: A retrospective single-center study on 169 AMBDs patients, including pemphigus vulgaris (PV), mucous membrane pemphigoid (MMP), paraneoplastic autoimmune multiorgan syndrome (PAMS), and lichen planus pemphigoides (LPP), was performed from 1994 to 2019 in an oral medicine tertiary center, where we collected sociodemographic data, clinical type and phenotype, prescribed therapies, and related outcomes. Read More

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http://dx.doi.org/10.1111/jop.13059DOI Listing

Paraneoplastic Pemphigus Mimicking Toxic Epidermal Necrolysis Associated with Follicular Lymphoma: Possible Pathological Role of CD8 T Cells.

Acta Derm Venereol 2020 Jul 2;100(14):adv00204. Epub 2020 Jul 2.

Department of Dermatology, Shiga University of Medical Science, Setatsukinowa, Otsu, Shiga 520-2192, Japan. E-mail:

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http://dx.doi.org/10.2340/00015555-3557DOI Listing

Retrospective analysis of 45 cases of localized retroperitoneal Castleman disease from a single center.

Hepatobiliary Surg Nutr 2020 Jun;9(3):304-311

Department of General Surgery, Peking University First Hospital, Beijing 100034, China.

Background: Castleman disease (CD) is a rare lymphoproliferative disease characterized by high heterogeneity in clinical manifestation and prognosis. This study aimed to summarize clinical features of localized retroperitoneal CD and our experiences to improve the diagnosis and treatment of this disease.

Methods: Clinical data of 45 patients with localized retroperitoneal CD were retrospectively analyzed. Read More

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http://dx.doi.org/10.21037/hbsn.2019.05.05DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7262626PMC

Predominant Stroma-Rich Feature in Hyaline Vascular Variant of Castleman Disease Is Associated With Paraneoplastic Pemphigus.

Am J Clin Pathol 2020 May 27. Epub 2020 May 27.

Department of Dermatology, Peking University First Hospital, Beijing, China.

Objectives: We aimed to describe the clinical and histopathologic features of Castleman disease (CD), particularly emphasizing its associations with paraneoplastic pemphigus (PNP) and prognosis.

Methods: We retrospectively enrolled 123 CD patients at our center. Clinical, pathologic, and laboratory data were reviewed. Read More

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http://dx.doi.org/10.1093/ajcp/aqaa053DOI Listing

Detection of autoantibodies against alpha-2-macroglobulin-like 1 in paraneoplastic pemphigus sera utilizing novel green fluorescent protein-based immunoassays.

J Dermatol Sci 2020 Jun 7;98(3):173-178. Epub 2020 May 7.

Department of Dermatology, Inselspital, Hospital and University of Bern, Bern, Switzerland. Electronic address:

Background: Paraneoplastic pemphigus (PNP) is a devastating autoimmune multiorgan syndrome associated with autoantibodies against several autoantigens, including the alpha-2-macroglobulin-like-1 (A2ML1). A2ML1 is recognized by up to 70 % of PNP sera. The currently recommended techniques for serological diagnosis of PNP are inadequate to detect anti-A2ML1 antibodies. Read More

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http://dx.doi.org/10.1016/j.jdermsci.2020.04.005DOI Listing

18F-FDG PET/CT in Follicular Dendritic Cell Sarcoma With Paraneoplastic Pemphigus as the First Manifestation.

Clin Nucl Med 2020 Jul;45(7):572-574

Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA.

A 48-year-old woman presented with refractory oral ulcers and skin rashes on the palms and trunk, diagnosed as paraneoplastic pemphigus. The chest x-ray revealed a mass in the right lower chest, and the F-FDG PET/CT scan showed the lesion in the right anterior-inferior mediastinum with intense F-FDG uptake, accompanied by right parasternal adenopathy and pleural effusion. The surgical pathology proved a follicular dendritic cell sarcoma, with right parasternal lymph node metastasis. Read More

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http://dx.doi.org/10.1097/RLU.0000000000003065DOI Listing
July 2020
3.931 Impact Factor

Exfoliative Erythroderma: An Unusual Presentation of Paraneoplastic Pemphigus Associated with Castleman's Disease.

Case Rep Dermatol 2020 Jan-Apr;12(1):76-81. Epub 2020 Apr 14.

Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

We report a 23-year-old woman who presented with generalized scaly erythematous rash predominately on the upper trunk and hemorrhagic stomatitis. The histopathologic and immunopathologic findings were consistent with the diagnosis of paraneoplastic pemphigus. Castleman's tumor was diagnosed with computed tomography and exploratory laparotomy. Read More

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http://dx.doi.org/10.1159/000507357DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7184796PMC

A case in which paraneoplastic pemphigus and bronchiolitis obliterans are the main manifestations of inflammatory pseudotumour-like follicular dendritic cell sarcoma.

Authors:
Changlin Zhao

Australas J Dermatol 2020 Apr 21. Epub 2020 Apr 21.

Health Science College, Guangdong Pharmaceutical University, Guangzhou, Guangdong, China.

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http://dx.doi.org/10.1111/ajd.13287DOI Listing

Paraneoplastic Dermatoses: A Brief General Review and an Extensive Analysis of Paraneoplastic Pemphigus and Paraneoplastic Dermatomyositis.

Int J Mol Sci 2020 Mar 21;21(6). Epub 2020 Mar 21.

Molecular and Cell Biology Laboratory, IDI-IRCCS, 00167 Rome, Italy.

Skin manifestations of systemic disease and malignancy are extremely polymorphous. Clinicians should be familiarized with paraneoplastic dermatoses in order to perform an early diagnosis of the underlying neoplasm. Lack of familiarity with cutaneous clues of internal malignancy may delay diagnosis and treatment of cancer. Read More

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http://dx.doi.org/10.3390/ijms21062178DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7139382PMC

Pemphigus in Sarcomatoid Renal Cell Carcinoma: A Rare Paraneoplastic Manifestation.

Niger J Surg 2020 Jan-Jun;26(1):81-83. Epub 2020 Feb 10.

Department of Surgery, AIIMS, Rishikesh, Uttarakhand, India.

Renal cell carcinoma with sarcomatoid features is a rare presentation with a 5% incidence. Sarcomatoid renal cell carcinoma is usually associated with poor prognosis. It commonly metastasizes to the lungs, bones, and liver. Read More

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http://dx.doi.org/10.4103/njs.NJS_4_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7041359PMC
February 2020

Paraneoplastic pemphigus associated with Waldenström's macroglobulinemia.

J Dermatol 2020 May 27;47(5):e200-e201. Epub 2020 Feb 27.

Department of Dermatology, Shinshu University School of Medicine, Matsumoto, Japan.

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http://dx.doi.org/10.1111/1346-8138.15289DOI Listing

Cutaneous paraneoplastic pemphigus syndrome associated with undifferentiated uterine sarcoma.

Gynecol Oncol Rep 2020 May 18;32:100534. Epub 2020 Jan 18.

University of Virginia Health System, 1215 Lee St, Charlottesville, VA 22903, United States.

Pemphigus is a group of autoimmune intraepidermal blistering diseases caused by immunoglobulins directed against keratinocyte cell surface components. In this case report, we identify a non-classical paraneoplastic pemphigus (PNP) foliaceous related to an undifferentiated uterine sarcoma. The patient is a 54-year-old Chinese female with a past medical history of arthritis who presented with worsening fatigue in November 2017 and an itchy, blistering, erythematous annular plaque that first appeared on her chest in February 2018. Read More

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http://dx.doi.org/10.1016/j.gore.2019.100534DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7000789PMC

Radiation-Associated Pemphigus Vulgaris in a Patient With Preceding Malignancy: Treatment With Rituximab as a Valuable Option.

Front Immunol 2019 21;10:3116. Epub 2020 Jan 21.

Department of Dermatology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

Pemphigus is a chronic autoimmune blistering disorder, characterized by (muco-)cutaneous erosions due to autoantibodies against desmoglein 3 and/or 1. Pemphigus induction might be associated with drugs, malignancy or radiation therapy (RT); the latter being only rarely described. A rigorous literature review revealed around 30 cases of RT-associated pemphigus, which had been primarily treated with topical and/or systemic steroids, in some cases also dapsone or few other immunosuppressive agents were given. Read More

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http://dx.doi.org/10.3389/fimmu.2019.03116DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6985440PMC
January 2020

Follicular dendritic cell sarcoma aggravated by hyaline-vascular Castleman's disease in association with paraneoplastic pemphigus: study of the tumor and successful treatment.

An Bras Dermatol 2019 Sep - Oct;94(5):578-581. Epub 2019 Sep 30.

Department of Dermatology, The Eighth Affiliated Hospital of Sun Yat-sen University, Shenzhen, Guangdong, China.

The authors have successfully treated and monitored a case of paraneoplastic pemphigus in association with follicular dendritic cell sarcoma aggravated by hyaline-vascular Castleman's disease. The patient was a 56-year-old female who presented with recalcitrant erosive lichen planus of the oral cavity, tongue, and genital mucosa, along with polymorphous eruptions throughout her body. Histological examination of the cutaneous lesions, indirect immunofluorescence on rat bladder epithelium, and western blot of human keratinocyte proteins identified anti-epidermal antibodies in the patient's serum. Read More

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http://dx.doi.org/10.1016/j.abd.2019.09.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6857577PMC
December 2019

Paraneoplastic Pemphigus Revealed by Anti-programmed Death-1 Pembrolizumab Therapy for Cutaneous Squamous Cell Carcinoma Complicating Hidradenitis Suppurativa.

Front Med (Lausanne) 2019 5;6:249. Epub 2019 Nov 5.

Department of Dermatology and Referral Center for Autoimmune Bullous Diseases MALIBUL, Avicenne Hospital, AP-HP, University Paris 13, Bobigny, France.

A 64-year-old patient developed a widespread autoimmune mucocutaneous blistering disease 3 weeks after the initiation of the anti-programmed death-1 (anti-PD-1) pembrolizumab therapy administered for a locally advanced cutaneous squamous cell carcinoma (SCC) of the buttocks arising from hidradenitis suppurativa. A diagnosis of paraneoplastic pemphigus (PNP) was made based on the presence of a suprabasal acantholysis associated with intercellular deposits of immunoglobulin G and C3 on basement membrane zone. Analysis of the patient's sera was positive on monkey bladder and detected circulating antibodies against desmoglein 3 and desmoplakin I prior to the initiation of pembrolizumab. Read More

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http://dx.doi.org/10.3389/fmed.2019.00249DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6848154PMC
November 2019
4 Reads

Dermatomyositis in patients with autoimmune blistering diseases.

Int J Womens Dermatol 2019 Sep 4;5(4):256-260. Epub 2019 Jun 4.

Corporal Michael J. Crescenz VAMC, Philadelphia, Pennsylvania.

It is common for multiple autoimmune diseases to occur in the same patient. However, autoimmune blistering diseases (AIBD) do not commonly associate with dermatomyositis (DM). We performed a literature review and found 12 previous reports that may be attributed to misdiagnosis, underreporting, or true rarity of association. Read More

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http://dx.doi.org/10.1016/j.ijwd.2019.05.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6831793PMC
September 2019

Pemphigus erythematosus and cutaneous epitheliotropic lymphoma in a Labrador Retriever dog.

Iran J Vet Res 2019 ;20(3):221-224

Department of Morphology, Veterinary Faculty, University of Las Palmas de Gran Canaria, Spain.

Background: Paraneoplastic pemphigus is an autoimmune blistering skin disease associated with concurrent neoplasia that is rarely observed in veterinary medicine.

Case Description: This case report presents a ten-year-old female Labrador Retriever dog with generalized seborrhea and alopecic, crusting and ulcerative lesions in the periocular and perioral regions, the lips and the groins.

Findings/treatment And Outcome: Hematology analysis showed a marked increase in the number of lymphocytes, while the rest of the values were normal. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6811711PMC
January 2019

Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview.

An Bras Dermatol 2019 17;94(4):388-398. Epub 2019 Oct 17.

Department of Dermatology, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.

Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and α-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Read More

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http://dx.doi.org/10.1590/abd1806-4841.20199165DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7007015PMC
October 2019

Castleman's disease. Large retroperitoneal masses and multiple calcifications detected by F-FDG PET/CT.

Hell J Nucl Med 2019 Sep-Dec;22(3):220-221. Epub 2019 Oct 7.

Department of Nuclear Medicine, West China Hospital of Sichuan University, No. 37. Guoxue Alley, 610041 Chengdu, Sichuan province, P.R. China.

An 18 years old man was admitted to our hospital for intractable oral ulcer and abdominal discomfort. Bulky soft-tissue masses were found in pathology speciments in the retroperitoneum from the twelfth thoracic vertebra to the fifth lumbar vertebra during an abdominal computed tomography (CT) scan. Then he was referred for fluorine-18-fluorodeoxyglucose positron emission tomography/CT (F-FDG PET/CT) due to suspicion of paraneoplastic pemphigus. Read More

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http://dx.doi.org/10.1967/s002449911057DOI Listing
May 2020
3 Reads

Persistence of antienvoplakin and antiperiplakin antibodies in a patient with paraneoplastic pemphigus 20 years after remission.

Br J Dermatol 2020 Mar 8;182(3):797-798. Epub 2019 Nov 8.

Department of Dermatology, Hospital Clínic de Barcelona, University of Barcelona, Barcelona, Spain.

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http://dx.doi.org/10.1111/bjd.18534DOI Listing
March 2020
2 Reads

Synchronous retroperitoneal Castleman's disease with clear cell renal cell carcinoma.

BMJ Case Rep 2019 Sep 16;12(9). Epub 2019 Sep 16.

Urology, PGIMER, Chandigarh, India.

Castleman's disease is a rare non-clonal lymphoproliferative disorder and known to be associated with a number of disorders such as polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes syndrome, Kaposi sarcoma, paraneoplastic pemphigus and plasma cell dyscrasias. The association of Castleman's disease with epithelial malignancy is not clear and limited to few case reports. We describe a case of synchronous Castleman's disease with renal cell carcinoma in a 53-year-old woman. Read More

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http://dx.doi.org/10.1136/bcr-2019-230919DOI Listing
September 2019
4 Reads

Bullous Diseases in Children: A Review of Clinical Features and Treatment Options.

Paediatr Drugs 2019 Oct;21(5):345-356

Department of Dermatology, University of Minnesota, 240 Phillips-Wangensteen Building, 516 Delaware Street Southeast, Minneapolis, MN, 55455, USA.

Bullous diseases are uncommon in children; however, as they have the potential to affect quality of life, occasionally have long-term side effects in the setting of scarring processes, and carry a rare risk of underlying malignancy [e.g., with paraneoplastic pemphigus (PNP)], knowledge of their clinical presentation and treatment options is essential. Read More

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http://dx.doi.org/10.1007/s40272-019-00349-3DOI Listing
October 2019
5 Reads

Paraneoplastic Pemphigus with Underlying Castleman's Disorder: A Rare Report with Literature Review.

Cureus 2019 Jun 28;11(6):e5022. Epub 2019 Jun 28.

Internal Medicine, Dow University of Health Sciences, Karachi, PAK.

Paraneoplastic pemphigus (PNP), also known as paraneoplastic autoimmune multisystem syndrome (PAMS), is an autoimmune blistering disorder of the skin associated with various hematological and nonhematological malignancies. In most of the cases, it can be a harbinger of a concealed benign or malignant neoplasm. We report the case of a 23-year-old female patient who presented to the dermatology consult service with a previously known diagnosis of refractory pemphigus vulgaris but she failed to reach remission for her oral and skin lesions on steroid and intravenous immunoglobulin (IVIG). Read More

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http://dx.doi.org/10.7759/cureus.5022DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6721887PMC
June 2019
3 Reads

Dermatology in the Diagnosis of Noncutaneous Malignancy: Paraneoplastic Diseases.

Dermatol Clin 2019 Oct 10;37(4):537-544. Epub 2019 Jul 10.

Oregon Health & Science University, 3303 Southwest Bond Avenue CH16D, Portland, OR 97239, USA.

It is important to recognize paraneoplastic dermatoses because they allow the practitioner to begin an early, directed workup to detect an underlying malignant neoplasm. In this review, several paraneoplastic dermatoses are outlined using existing data to detail each one's association with underlying malignancy, demographics, prognosis, and treatment considerations. Read More

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http://dx.doi.org/10.1016/j.det.2019.05.011DOI Listing
October 2019
5 Reads

Development of an electronic medical record-based algorithm to identify patients with Stevens-Johnson syndrome and toxic epidermal necrolysis in Japan.

PLoS One 2019 13;14(8):e0221130. Epub 2019 Aug 13.

Division of Drug Development and Regulatory Science, Graduate School of Pharmaceutical Sciences, Keio University, Shibakoen, Minato-ku, Tokyo, Japan.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), severe drug reactions, are often misdiagnosed due to their rarity and lack of information on differential diagnosis. The objective of the study was to develop an electronic medical record (EMR)-based algorithm to identify patients with SJS/TEN for future application in database studies. From the EMRs of a university hospital, two dermatologists identified all 13 patients with SJS/TEN seen at the Department of Dermatology as the case group. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0221130PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6692049PMC
March 2020
4 Reads

Oral Lesions in Autoimmune Bullous Diseases: An Overview of Clinical Characteristics and Diagnostic Algorithm.

Am J Clin Dermatol 2019 Dec;20(6):847-861

Department of Dermatology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, 9700 RB, Groningen, The Netherlands.

Autoimmune bullous diseases are a group of chronic inflammatory disorders caused by autoantibodies targeted against structural proteins of the desmosomal and hemidesmosomal plaques in the skin and mucosa, leading to intra-epithelial or subepithelial blistering. The oral mucosa is frequently affected in these diseases, in particular, in mucous membrane pemphigoid, pemphigus vulgaris, and paraneoplastic pemphigus. The clinical symptoms are heterogeneous and may present with erythema, blisters, erosions, and ulcers localized anywhere on the oral mucosa, and lead to severe complaints for the patients including pain, dysphagia, and foetor. Read More

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http://dx.doi.org/10.1007/s40257-019-00461-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6872602PMC
December 2019
9 Reads

Thymoma-Associated Paraneoplastic Autoimmune Multiorgan Syndrome-From Pemphigus to Lichenoid Dermatitis.

Front Immunol 2019 21;10:1413. Epub 2019 Jun 21.

Department of Dermatology and Allergology, Philipps-University, Marburg, Germany.

Paraneoplastic autoimmune multi-organ syndrome (PAMS) is a rare clinical condition characterized by variable and heterogeneous clinical phenotypes in the presence of neoplasias which largely depend on the activation of humoral and cellular immune responses. Clinically, these patients present with a spectrum of antibody-driven pemphigus-like lesions to graft-vs.-host-disease-like exanthemas with a lichenoid inflammatory infiltrate in the skin. Read More

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http://dx.doi.org/10.3389/fimmu.2019.01413DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6598597PMC
June 2019
15 Reads

[Paraneoplastic syndromes].

Ned Tijdschr Geneeskd 2019 06 17;163. Epub 2019 Jun 17.

Amsterdam UMC, locatie VUmc, afd. Interne Geneeskunde, Amsterdam.

A paraneoplastic syndrome is a phenomenon or complex of symptoms that can occur with malignancy, without this being the result of tumour cells in the affected area. In this article, we describe the following paraneoplastic syndromes: thrombophlebitis migrans, clubbing, pemphigus, acanthosis nigricans, blue fingers, dermatomyositis, and myasthenia gravis. Read More

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June 2019
21 Reads

Cutaneous Disorders Associated with Castleman's Disease.

Acta Derm Venereol 2019 Oct;99(11):984-989

Department of Dermatology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.

Castleman's disease is a rare disease of the lymph nodes and related tissues, presenting as angiofollicular or giant lymph node hyperplasia. Although various skin manifestations have been reported to occur in Castleman's disease, a comprehensive study of cutaneous disorders in Castleman's disease is lacking. Therefore, the aim of this study was to investigate Castleman's disease-associated cutaneous disorders. Read More

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http://dx.doi.org/10.2340/00015555-3253DOI Listing
October 2019
5 Reads

Development of a Desmocollin-3 Active Mouse Model Recapitulating Human Atypical Pemphigus.

Front Immunol 2019 19;10:1387. Epub 2019 Jun 19.

Laboratory of Cutaneous Biology, Department of Surgical, Medical, Dental and Morphological Sciences, University of Modena and Reggio Emilia, Modena, Italy.

Pemphigus vulgaris (PV) is a life-threatening mucocutaneous autoimmune blistering disease. It is often associated with autoantibodies to the desmosomal adhesion proteins Desmoglein 3 (DSG3) and Desmoglein 1 (DSG1). Recently, auto-antigens, such as desmocollins and others have been described in PV and in atypical pemphigus forms such as Pemphigus Herpetiformis (PH), Pemphigus Vegetans (PVeg), and Paraneoplastic Pemphigus (PP). Read More

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http://dx.doi.org/10.3389/fimmu.2019.01387DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6593104PMC
June 2019
4 Reads

[Paraneoplastic skin diseases].

Internist (Berl) 2019 Aug;60(8):775-782

Hautklinik und Hauttumorzentrum Rheinpfalz, Klinikum der Stadt Ludwigshafen, Bremserstraße 79, 67063, Ludwigshafen am Rhein, Deutschland.

Various specific skin alterations can occur in patients with malignant diseases. If these skin diseases occur as associated symptoms of a malignant process, they are called paraneoplastic. In this overview, obligate and frequent facultative paraneoplastic skin diseases are assigned according to the triggering type of malignancy. Read More

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http://dx.doi.org/10.1007/s00108-019-0636-1DOI Listing
August 2019
9 Reads

Paraneoplastic Pemphigus: Paraneoplastic Autoimmune Disease of the Skin and Mucosa.

Front Immunol 2019 4;10:1259. Epub 2019 Jun 4.

Department of Dermatology and Cutaneous Biology Research Institute, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea.

Paraneoplastic pemphigus (PNP) is a rare but life-threatening mucocutaneous disease mediated by paraneoplastic autoimmunity. Various neoplasms are associated with PNP. Intractable stomatitis and polymorphous cutaneous eruptions, including blisters and lichenoid dermatitis, are characteristic clinical features caused by humoral and cell-mediated autoimmune reactions. Read More

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http://dx.doi.org/10.3389/fimmu.2019.01259DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6558011PMC
June 2019
9 Reads

Paraneoplastic pemphigus and myasthenia gravis as the first manifestations of a rare case of pancreatic follicular dendritic cell sarcoma: CT findings and review of literature.

BMC Gastroenterol 2019 Jun 14;19(1):92. Epub 2019 Jun 14.

Department of Organ transplantation, Sichuan Academy of Medical Science and Sichuan Provincial People's Hospital, 32 West Second Section, First Ring Road, Chengdu, 610072, Sichuan, China.

Background: Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm that originates from follicular dendritic cells in lymphoid tissue while paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms. Pancreatic FDCS associated with PNP and myasthenia gravis (MG) is even rarer and highly malignant. We present the clinical data, pathological materials and computed tomography (CT) features of a rare case of this disease. Read More

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http://dx.doi.org/10.1186/s12876-019-1008-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6570917PMC
June 2019
18 Reads

Ocular Manifestations and Management of Autoimmune Bullous Diseases.

J Ophthalmic Vis Res 2019 Apr-Jun;14(2):195-210

Ocular Tissue Engineering Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Autoimmune bullous diseases with ocular involvement consist of a group of systemic entities that are characterized by formation of autoantibodies against the proteins of the epithelial basement membrane zone of the conjunctiva. Mostly, the elderly are affected by these diseases. The characteristic patterns of mucocutaneous involvement and the specific tissue components targeted by these autoantibodies are differentiating features of these diseases. Read More

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http://dx.doi.org/10.4103/jovr.jovr_86_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6504727PMC
May 2019
8 Reads

Mucous Membrane Pemphigoid-Associated Malignancies: Case Series and a Brief Overview of the Literature.

Dermatol Pract Concept 2019 Apr 30;9(2):119-125. Epub 2019 Apr 30.

Dermatology Division, Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy.

Background: Mucous membrane pemphigoid (MMP) is a heterogeneous group of blistering disorders affecting the mucosae with or without skin involvement, characterized by the presence of autoantibodies to components of the basement membrane zone, including the bullous pemphigoid antigen BP180 and β4 integrin. Current literature has shown that a minority of patients present circulating antibodies to laminin-332 and this population seems to be associated with a relatively high risk of malignancy.

Objective: To present our personal case series of patients with MMP-associated malignancy from a dermatology university hospital. Read More

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https://dpcj.org/index.php/dpc/article/view/dermatol-pract-c
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http://dx.doi.org/10.5826/dpc.0902a07DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6502303PMC
April 2019
7 Reads

Mucous membrane pemphigoid and oral blistering diseases.

Clin Exp Dermatol 2019 Oct 18;44(7):732-739. Epub 2019 May 18.

Oral Medicine, Dental Institute, Guy's and St Thomas' NHS Foundation Trust, London, UK.

The autoimmune blistering disorders present with variable frequency in the oral cavity. Recognition of their key clinical features at presentation is important, as there are many causes of oral ulceration. Careful history-taking, clinical examination, an understanding of pathogenesis and appropriate investigations are essential. Read More

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http://dx.doi.org/10.1111/ced.13996DOI Listing
October 2019
13 Reads

Oral lichen planus: A disease or a spectrum of tissue reactions? Types, causes, diagnostic algorhythms, prognosis, management strategies.

Periodontol 2000 2019 06;80(1):105-125

UCL Eastman Dental Institute, University College London, London, UK.

Oral lichen planus and lichenoid lesions comprise a group of disorders of the oral mucosa that likely represent a common reaction pattern to 1 or more unknown antigens. The coexistence of hyperkeratotic striation/reticulation, varying degrees of mucosal inflammation from mild erythema to severe widespread ulceration, and a band-like infiltrate of mononuclear inflammatory cells including activated T lymphocytes, macrophages, and dendritic cells, are considered suggestive of oral lichen planus and lichenoid lesions. Several classification systems of oral lichen planus and lichenoid lesions have been attempted, although none seem to be comprehensive. Read More

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http://dx.doi.org/10.1111/prd.12260DOI Listing
June 2019
21 Reads
3.632 Impact Factor

Rituximab as a dual therapeutic option for pemphigus and primary cutaneous B-cell lymphomas: Two case reports.

Dermatol Ther 2019 07 7;32(4):e12948. Epub 2019 May 7.

Department of Clinical-Surgical, Diagnostic and Pediatric Science, Institute of Dermatology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.

It is known that individuals with immune dysregulation have an increased risk of non-Hodgkin lymphoma. This association has been proven for pemphigus as well as for other autoimmune disease. We describe the development of cutaneous B-cell lymphoma in two patients affected by long-standing pemphigus vulgaris and pemphigus foliaceus (i. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/dth.12948
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http://dx.doi.org/10.1111/dth.12948DOI Listing
July 2019
16 Reads

Risk factors for death and survival in paraneoplastic pemphigus associated with hematologic malignancies in adults.

J Am Acad Dermatol 2019 Jun 10;80(6):1544-1549. Epub 2019 Apr 10.

Assistance Publique-Hôpitaux de Paris, Département de Dermatologie, Hôpital Saint-Louis, Paris, France; Université Paris-Diderot, Sorbonne Paris Cité, Paris, France. Electronic address:

Background: Paraneoplastic pemphigus (PNP) occurs more often in patients with hematologic malignancies (HMs) than in patients with solid cancer. Lung bronchiolitis obliterans (BO) is a severe complication of PNP.

Objective: To determine the precise clinical and biologic features of HM-associated PNP and identify factors associated with mortality and survival. Read More

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http://dx.doi.org/10.1016/j.jaad.2018.03.043DOI Listing
June 2019
19 Reads

Review of paraneoplastic syndromes in children.

Pediatr Radiol 2019 04 16;49(4):534-550. Epub 2019 Mar 16.

Department of Radiology, Boston Children's Hospital, 300 Longwood Ave., Boston, MA, 02215, USA.

Paraneoplastic syndromes are defined as clinical syndromes that are not related to direct tumor invasion or compression but are secondary to tumor secretion of functional peptides/hormones or related to immune cross-reactivity with normal host tissue. Paraneoplastic syndromes have a wide range of presentations and can present before the primary malignancy or tumor recurrence is diagnosed. They can mimic non-neoplastic processes, making detection, diagnosis and treatment difficult. Read More

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http://dx.doi.org/10.1007/s00247-019-04371-yDOI Listing
April 2019
40 Reads

Cutaneous paraneoplastic syndromes.

Semin Diagn Pathol 2019 Jul 31;36(4):211-228. Epub 2019 Jan 31.

PRW Laboratories, Charlottesville, VA, United State.

A variety of cutaneous abnormalities can be seen in patients with malignant diseases, some of which are infectious, with others representing direct involvement of the skin by the underlying disorder. Yet another group of lesions can be regarded as associated markers of the malignant process, and, as such, are termed "paraneoplastic." This review considers the latter collection of conditions, grouping them by the generic type of malignancy that is usually linked to the paraneoplasia. Read More

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http://dx.doi.org/10.1053/j.semdp.2019.01.001DOI Listing
July 2019
44 Reads

[Follicular lymphoma accompanied by paraneoplastic pemphigus and bronchiolitis obliterans: a case report].

Rinsho Ketsueki 2019;60(1):7-11

Department of Hematology, Tokyo Medical and Dental University.

A 54-year-old female complained of oral erosion. A flaccid blister appeared on the trunk 2 months after the onset. The high titer of the anti-desmoglein 1 antibody in the absence of Nikolsky's sign led to the diagnosis of pemphigus vulgaris. Read More

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http://dx.doi.org/10.11406/rinketsu.60.7DOI Listing
August 2019
6 Reads

Decreased recognition of paraneoplastic pemphigus in patients previously treated with anti-CD 20 monoclonal antibodies.

Br J Dermatol 2019 05 15;180(5):1238-1239. Epub 2019 Jan 15.

Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, MD, U.S.A.

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http://dx.doi.org/10.1111/bjd.17577DOI Listing
May 2019
5 Reads

A rare association of paraneoplastic pemphigus with gastric signet cell ring carcinoma.

Australas J Dermatol 2019 May 4;60(2):e168-e169. Epub 2018 Dec 4.

Division of Immune-Mediated Skin Diseases, I.M. Sechenov First Moscow State Medical University, Moscow, Russia.

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http://dx.doi.org/10.1111/ajd.12963DOI Listing
May 2019
14 Reads

Diagnosis of Autoimmune Blistering Diseases.

Front Med (Lausanne) 2018 2;5:296. Epub 2018 Nov 2.

Department of Dermatology, University of Lübeck, Lübeck, Germany.

Autoimmune skin blistering diseases (AIBD) are characterized by autoantibodies that are directed against structural proteins in the skin and adjacent mucous membranes. Some clinical signs are typical for a specific AIBD, however, correct diagnosis requires the detection of tissue-bound or circulating autoantibodies. The gold standard for diagnosis of AIBD is the detection of autoantibodies or complement component 3 by direct immunofluorescence (DIF) microscopy of a perilesional biopsy. Read More

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http://dx.doi.org/10.3389/fmed.2018.00296DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6224342PMC
November 2018
39 Reads

Masked pemphigus among pediatric patients with Castleman's disease.

Int J Rheum Dis 2019 Jan 8;22(1):121-131. Epub 2018 Nov 8.

Department of Pathology, Taichung Veterans General Hospital, Taichung, Taiwan.

Aim: Paraneoplastic pemphigus (PNP) is a mucocutaneous autoimmune disorder accompanied with a neoplasm. Castleman's disease (CD), although rare, is the most common cause of PNP in children. It can be life-threatening when pulmonary involvement occurs. Read More

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http://dx.doi.org/10.1111/1756-185X.13407DOI Listing
January 2019
49 Reads