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recognized in the setting of paraneoplastic autoimmunity can be diverse, and the number of determinants recognized within a single antigen can be numerous. This review uses prototypic examples of paraneoplastic immune-mediated diseases and their associated malignancies to describe the mechanisms by which immune dysregulation can occur in the setting of cancer. Specific diseases covered include paraneoplasticpemphigus, Sweet's syndrome, pyoderma gangrenosum, thymoma-associated multiorgan autoimmunity (...) The Etiology of Paraneoplastic Autoimmunity. Although they may sometimes appear similar, paraneoplastic autoimmunity has a unique pathogenesis, different from the classical autoimmune diseases not associated with cancer. When distinguished clinically, paraneoplastic autoimmunity is more severe and often presents with a broader range of clinical signs and symptoms. Management of these patients is difficult and is usually centered in part on treatment of the underlying malignancy. Self-antigens
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 paraneoplasticpemphigus. This is a paraneoplastic disease of epithelial autoimmunity and adhesion originally described by Dr. Anhalt in 1990. Paraneoplasticpemphigus 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
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, paraneoplasticpemphigus, 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
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
Paraneoplasticpemphigus/paraneoplastic autoimmune multiorgan syndrome. Paraneoplasticpemphigus 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
Paraneoplasticpemphigus: an uncommon cause of chronic cicatrising conjunctivitis We report a case of paraneoplasticpemphigus (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
A case of paraneoplasticpemphigus associated with Castleman's disease presenting as erosive lichen planus. Paraneoplasticpemphigus (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
Atypical paraneoplasticpemphigus secondary to endometrial carcinoma with no mucosal involvement. Paraneoplasticpemphigus (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
) 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) Paraneoplasticpemphigus 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
acute graft‐versus‐host disease Bullous lupus erythematosus Bullous pemphigoid Epidermolysis bullosa acquisita Kawasaki disease (early‐stage erythema no blisters) Behҫet disease Generalized bullous fixed‐drug eruption Pemphigus vulgaris Paraneoplasticpemphigus 7 Management and long‐term complications 7.1 How should causality be determined? In the paediatric population, both infections and drugs are important triggers of SJS/TEN. The most commonly implicated medications in children
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 paraneoplasticpemphigus 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
of patients have an acquired disorder. These include the rare autoimmune blistering diseases mucous membrane pemphigoid (MMP), pemphigus vulgaris (PV), linear IgA disease, epidermolysis bullosa acquisita and paraneoplasticpemphigus. Important clinical differential diagnoses include erythema multiforme, which may be mistaken for PV in appearance, while oral lichen planus may be indistinguishable from MMP. Angina bullosa haemorrhagica may also present with tense haemorrhagic bullae, and in the absence
, and paraneoplasticpemphigus. 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. Therefore, a quick and proper diagnosis with adequate treatment is needed. Clinical presentations of autoimmune bullous diseases often overlap and diagnosis cannot be made based on clinical features alone. Immunodiagnostic tests are of great importance (...) in differentiating between the different diseases. Direct immunofluorescence microscopy shows depositions of autoantibodies along the epithelial basement membrane zone in mucous membrane pemphigoid subtypes, or depositions on the epithelial cell surface in pemphigus variants. Additional immunoserological tests are useful to discriminate between the different subtypes of pemphigoid, and are essential to differentiate between pemphigus and paraneoplasticpemphigus. This review gives an overview of the clinical
(paraneoplasticpemphigus, xanthogranulomas), cytokine-related (vasculitis-like lesion, cherry angioma, hyperpigmentation), and non-specific (pruritus). This study builds on previous case reports of cutaneous disorders in Castleman's disease and proposes a new classification system.
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 paraneoplasticpemphigus (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
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 paraneoplasticpemphigus 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.
, 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), paraneoplasticpemphigus (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