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Paraneoplastic Pemphigus

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101. Paraneoplastic autoimmune multiorgan syndrome: 20 years after. (PubMed)

Paraneoplastic autoimmune multiorgan syndrome: 20 years after. The purpose of this review is to provide insight and clarification in the quandary of classification and delineate clinical and histological features and pathophysiology of paraneoplastic pemphigus. This is a paraneoplastic disease of epithelial autoimmunity and adhesion originally described by Dr. Anhalt in 1990. Paraneoplastic pemphigus represents only one manifestation of the heterogeneous autoimmune syndrome in which patients (...) , in addition to small airways occlusion, may display a spectrum of at least five clinical variants of the mucocutaneous disease [i.e. pemphigus-like, pemphigoid-like, erythema multiforme-like, graft-versus-host disease-like, and lichen planus-like, termed paraneoplastic autoimmune multiorgan syndrome (PAMS)]. There is a need for the expanded, inclusive classification of diverse mucocutaneous and respiratory presentations of PAMS. Multiple specific effectors of humoral and cellular autoimmunity mediating

2011 International Journal of Dermatology

102. Pemphigus Vulgaris

in diameter. Pemphigus vulgaris usually occurs in middle-aged patients, affecting men and women in equal numbers. Rarely, cases have been reported in children. One variant, paraneoplastic pemphigus, can occur in patients who have malignant or benign tumors, most commonly . Pemphigus vulgaris is characterized by IgG autoantibodies directed against the calcium-dependent cadherins desmoglein 1 and desmoglein 3. These transmembrane glycoproteins affect cell-cell adhesion and signaling between epidermal cells (...) Pemphigus Vulgaris Pemphigus Vulgaris - Dermatologic Disorders - MSD Manual Professional Edition Brought to you by The trusted provider of medical information since 1899 SEARCH SEARCH MEDICAL TOPICS Common Health Topics Resources QUIZZES & CASES Quizzes Cases The trusted provider of medical information since 1899 SEARCH SEARCH MEDICAL TOPICS Common Health Topics Resources QUIZZES & CASES Quizzes Cases / / / / IN THIS TOPIC OTHER TOPICS IN THIS CHAPTER Test your knowledge Diagnostic Tests

2013 Merck Manual (19th Edition)

103. Paraneoplastic syndromes in patients with primary oral cancers: a systematic review. (PubMed)

Paraneoplastic syndromes in patients with primary oral cancers: a systematic review. The aim of this study was to review all the paraneoplastic syndromes of primary tumours of the oral cavity. Metastatic tumours of the mouth and primary tumours of the oropharynx (including tonsils), and major salivary glands were excluded. The primary search was conducted on PubMed, Scopus and EMBASE, and included every paraneoplastic syndrome from a primary oral tumour described in English, French, or German (...) papers during the last 20 years. The secondary search was conducted by handpicking articles from reviews on paraneoplastic syndromes of the head and neck. The aim of the tertiary search was to identify conditions that had been reported only rarely. We then cross-referenced "mouth neoplasm" with every paraneoplastic condition cited in relevant review articles. We classified the paraneoplastic syndromes that arose from tumours of the head and neck into six categories: endocrine, dermatological

2010 The British journal of oral & maxillofacial surgery

104. Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome. (PubMed)

Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome. 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

2009 International Journal of Dermatology

105. Paraneoplastic pemphigus: an uncommon cause of chronic cicatrising conjunctivitis (PubMed)

Paraneoplastic pemphigus: an uncommon cause of chronic cicatrising conjunctivitis We report a case of paraneoplastic pemphigus (PNP) as an uncommon but severe cause of cicatrising conjunctivitis. Initially diagnosed as drug eruptions, the patient's condition did not improve despite cessation of chemotherapy. Immunohistological confirmation of PNP has led to the use of combined oral prednisolone and intravenous immunoglobulin. Her ocular conditions stabilised with complete recovery of vision

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2009 BMJ case reports

106. A case of paraneoplastic pemphigus associated with Castleman's disease presenting as erosive lichen planus. (PubMed)

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

2009 Clinical & Experimental Dermatology

107. Atypical paraneoplastic pemphigus secondary to endometrial carcinoma with no mucosal involvement. (PubMed)

Atypical paraneoplastic pemphigus secondary to endometrial carcinoma with no mucosal involvement. Paraneoplastic pemphigus (PNP) is a mucocutaneous immunobullous disease associated with several types of internal malignancy. We report a case of a 78-year-old woman presenting with an atypical form of PNP associated with a recurrence of endometrial cancer. There was no involvement of the mucous membranes. Although the macroscopic and histological appearances were typical for IgA pemphigus

2009 Clinical & Experimental Dermatology

108. Assessment of vesicular-bullous rash

) Incontinentia pigmenti Bullous ichthyosiform erythroderma (epidermolytic hyper-keratosis) Mastocytosis Bullous arthropod bite reaction Erythema multiforme Stevens-Johnson syndrome Toxic epidermal necrolysis Amyloidosis Hand-foot-mouth disease Congenital syphilis Linear IgA disease Chronic bullous disease of childhood Pemphigus vulgaris Pemphigus foliaceus (fogo selvagem, pemphigus erythematosus) Paraneoplastic pemphigus Bullous pemphigoid Pemphigoid gestationis Mucous membrane pemphigoid (cicatricial (...) eruption with resultant shearing off of the epithelium) or the Asboe-Hansen sign (extension of blister into unblistered skin when pressure is applied to the top of the blister). Berger TG, James WD, Elston DM. Andrew's diseases of the skin, clinical dermatology, 10th edition. WB Saunders; 2008. These signs serve to demonstrate that in some diseases (e.g., pemphigus vulgaris and severe bullous drug eruptions) the extent of blistering is greater than observed by simple inspection. Bullae located

2018 BMJ Best Practice

109. Management of Immune-Related Adverse Events in Patients Treated With Immune Checkpoint Inhibitor Therapy

drug, or a skin condition linked to another systemic disease. A biologic checkup, including a CBC with differential test, and liver and kidney function tests, including urinalysis, in addition to the blood work. If the patient is febrile, blood cultures should be considered as well. Skin biopsies to assess for full-thickness epidermal necrosis, as is seen in SJS/TEN, as well as other possible etiologies like paraneoplastic pemphigus or other autoimmune blistering dermatoses, or other drug reactions (...) in detached or sloughing epidermis, demonstrating poor attachment of the epidermis to the dermis, which is the case in some autoimmune disorders (eg, pemphigus) and Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN). It is recommended that clinicians manage grade 3 toxicities as follows: Should hold ICPi therapy and consult with dermatology to determine appropriateness of resuming. Should administer IV (methyl)prednisolone (or equivalent) at 1 to 2 mg/kg dosing tapered over at least 4 weeks

2018 American Society of Clinical Oncology Guidelines

111. Paraneoplastic Conjunctival Cicatrization: Two Different Pathogenic Types. (PubMed)

Paraneoplastic Conjunctival Cicatrization: Two Different Pathogenic Types. To describe the clinical and immunopathologic features of patients with 2 different types of paraneoplastic conjunctival cicatrization.Retrospective observational case analyses with a review of the literature.One patient with paraneoplastic ocular cicatricial pemphigoid (POCP) and 1 patient with paraneoplastic pemphigus (PNP) with ocular involvement.Critical review of clinical history, diagnostic studies (...) , and immunopathologic results of biopsies in the 2 cases, together with a review of the literature.Ability to recognize paraneoplastic conjunctival cicatrization and to diagnose the conditions accurately.The first patient, 46 years of age, presented with conjunctival scarring and symblephara, cough, oral lesions, and chest rash. Concurrently, a diagnosis of pulmonary squamous cell carcinoma was made. Conjunctival biopsy revealed a subepithelial bulla, an inflammatory infiltrate of T and B lymphocytes, and basement

2009 Ophthalmology

112. Diagnosis of Autoimmune Blistering Diseases (PubMed)

the disease course which can be helpful for treatment decisions. Exact diagnosis is essential for both treatment and prognosis, since some AIBD are associated with malign tumors such as paraneoplastic pemphigus and anti-laminin 332 mucous membrane pemphigoid. This review presents clinical and immunopathological features of AIBD for the state-of the art diagnosis of these disorders.

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2018 Frontiers in medicine

113. Lip ulceration in an immunocompromised patient (PubMed)

, direct immunofluorescence HSV, herpes simplex virus PNP, paraneoplastic pemphigus 2018 3 13 6 0 2018 3 13 6 0 2018 3 13 6 1 epublish 29527548 10.1016/j.jdcr.2017.07.011 S2352-5126(17)30160-1 PMC5842327 Clin Microbiol Rev. 2003 Jan;16(1):114-28 12525428 Adv Pharmacol. 2013;67:1-38 23885997 Br J Dermatol. 2015 Dec;173(6):1447-52 26358412 BMC Res Notes. 2016 Apr 23;9:232 27107717

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2018 JAAD Case Reports

114. Autoantibodies against desmocollins in European patients with pemphigus. (PubMed)

, Dsc2 and Dsc3 were investigated by ELISA and immunoblotting analysis in a cohort of European patients with pemphigus vulgaris (PV; n = 74), IgA pemphigus (n = 3), paraneoplastic pemphigus (PNP; n = 3) and two cases of atypical pemphigus (n = 2).Of the two cases with atypical pemphigus, one showed IgA reactivity against Dsc1 and Dsc3 and weak reactivity against Dsc2, and the other showed both IgG and IgA reactivity against Dsc1. One patient with IgA pemphigus had IgA autoantibodies against Dsc1 (...) Autoantibodies against desmocollins in European patients with pemphigus. Autoimmune bullous disorders of the pemphigus group are characterized by autoantibodies targeting desmoglein (Dsg)1, Dsg3 and Dsg4 and occasionally, desmocollin (Dsc)1, Dsc2 and Dsc3. Both Dsg and Dsc are components of desmosomal adhesion complexes.To investigate the presence of IgG and IgA autoantibodies against Dsc1, Dsc2 and Dsc3 in a cohort of patients with bullous disorders.IgG and IgA autoantibodies against Dsc1

2009 Clinical & Experimental Dermatology

116. Prevalence of Cutaneous Autoimmune Phenomena in HIV Infected Patients

reports have been published, though the overall frequency appears to be low. The aim of this project is to investigate the frequency of various autoantibodies specific for various AIBD such as bullous pemphigoid, epidermolysis bullosa acquisita and paraneoplastic pemphigus using commercially available ELISA (BPAG1/2, desmoglein 1/3, collagen type VII, envoplakin) as well as indirect immunofluorescence. The knowledge of the frequency of these auto-antibodies in HIV infected patients may elucidate

2017 Clinical Trials

117. Autoimmune Blistering Diseases in the Elderly: Clinical Presentations and Management. (PubMed)

Autoimmune Blistering Diseases in the Elderly: Clinical Presentations and Management. Elderly patients are more susceptible to the development of autoimmune blistering disorders such as bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and paraneoplastic pemphigus. This article focuses on the clinical aspects of the aforementioned autoimmune blistering diseases and highlights the important factors involved in treating elderly patients. It is essential

2016 Drugs & Aging

118. Multicentric Castleman¿s disease with voltage-gated potassium channel antibody-positive limbic encephalitis: a case report. (PubMed)

Multicentric Castleman¿s disease with voltage-gated potassium channel antibody-positive limbic encephalitis: a case report. Castleman's disease is a rare lymphoproliferative disorder which occurs in localized and multicentric forms and can mimic lymphoma. Despite its well-known association with certain autoimmune diseases, including paraneoplastic pemphigus and myasthenia gravis, Castleman's disease has not previously been associated with limbic encephalitis.We report the case of a 47-year old (...) revealed multicentric Castleman's disease. His symptoms improved with antiepileptic drugs and immunotherapy.This case highlights the clinical diversity of voltage-gated potassium channel autoimmunity and expands the association of Castleman's disease and autoimmune syndromes to include limbic encephalitis. Clinicians should be aware that paraneoplastic disorders of the central nervous system can be related to underlying hematologic disorders such as Castleman's disease.

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2015 BMC Neurology

119. Treatment of severe autoimmune blistering skin diseases with combination of protein A immunoadsorption and rituximab: a protocol without initial high dose or pulse steroid medication. (PubMed)

weeks and decline of circulating autoantibody levels. Complete/partial remission was 88%/12% in pemphigus and 71%/29% in subepidermal blistering diseases. Overall relapse rate was 13% with an average follow-up of 22 months. In the AL332-MMP pt. the PAIA/rituximab treatment was stopped because of an oesophagus cancer considered as the paraneoplastic cause of the skin disease.Combined treatment with PAIA and rituximab showed rapid and long-lasting response, thereby allowing substantial reduction (...) .) with such diseases: 6 pts. with pemphigus vulgaris, 3 pts. with bullous pemphigoid, 3 pts. with mucous membrane pemphigoid (MMP), one being anti-laminin-332-MMP (AL332-MMP), 2 pts. with pemphigus foliaceus and 1 pt. with epidermolysis bullosa acquisita (EBA). Patients were treated with a combination of protein A immunoadsorption (PAIA, 3-21 treatments) and rituximab (3-6 treatments) in addition to low dose conventional immunosuppression.All patients showed rapid clinical improvement starting within the first 4

2014 Journal of the European Academy of Dermatology and Venereology

120. A review of the current literature and a guide to the early diagnosis of autoimmune disorders associated with neuromyelitis optica. (PubMed)

with NMO and identified 22 autoimmune conditions (myasthenia gravis, coeliac disease, ulcerative colitis, sclerosing cholangitis, systemic lupus erythematosus, rheumatoid arthritis, antiphospholipid antibody syndrome, Sjogren's syndrome, autoimmune hypothyroidism, immune thrombocytopenic purpura, pernicious anaemia, narcolepsy, pemphigus foliaceus, alopecia areata, psoriasis, scleroderma, dermatitis herpetiformis, polymyositis, chronic inflammatory demyelinating polyneuropathy, paraneoplastic disorders

2014 Autoimmunity

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