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Paraneoplastic Pemphigus

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141. Chronic Lymphocytic Leukemia (Follow-up)

neutropenia Cold agglutinin disease Paraneoplastic pemphigus Neuropathies Evans syndrome Up to 25% of patients with CLL demonstrate autoimmune anemia, thrombocytopenia, or both. Simultaneously, immune incompetence is present, characterized by a progressive profound hypogammaglobulinemia, predisposing patients to a number of infections, the most common being bacterial pneumonias. Patients experiencing frequent bacterial infections associated with hypogammaglobulinemia are likely to benefit from monthly

2014 eMedicine.com

142. Thymoma (Diagnosis)

proliferation, autoimmunity, and/or immunodeficiency. Autoimmunity also may be caused by cross-immunity of antigens in other tissues with thymoma-associated antigens. Thymomas are usually encapsulated, locally spreading tumors. More than one system of classifying thymoma has been established (see Histologic Findings, Staging). Seventy percent of thymomas are associated with paraneoplastic syndromes such as myasthenia gravis (MG), red cell aplasia, pemphigus, and immunoglobulin (Ig) deficiency. Myasthenia (...) . [ ] Normal thymic epithelium tissue arises from the third branchial cleft and the third and fourth branchial pouches. Dendritic cells and macrophages found in large quantities at the corticomedullary junction arise from mesodermal tissues (bone marrow). The epithelial cells and these other stromal tissues of the thymus influence the selection and maturation of the T lymphocytes. Dysregulation of this system in thymoma is believed to be a cause of accompanying paraneoplastic syndromes. In the normal

2014 eMedicine Pediatrics

143. Nonneoplastic Epithelial Disorders of the Vulva (Diagnosis)

the following: Lichen sclerosus Squamous cell hyperplasia (with and without atypia) (localized neurodermatitis) Primary irritant dermatitis Fixed drug eruption Reiter disease Lupus erythematosus Darier disease Aphthosis and Behçet disease Pyoderma gangrenosum Plasma cell vulvitis Vulvar vestibulitis Blistering diseases involving the vulvar region include the following: Familial benign chronic pemphigus (Hailey-Hailey disease) Pigmentary changes involving the vulvar region include the following (...) estrogen. [ ] The use of oral contraceptives might be a contributing factor by increasing the sensitivity of the vestibular mucosa. [ ] Blistering diseases Familial benign chronic pemphigus (Hailey-Hailey disease) Hailey-Hailey disease is a rare autosomal dominant acantholytic disorder due to a mutation on band 3q21-q24 and is characterized from late adolescence or adulthood by recurrent eruptions of vesicles and blisters typically located on the neck, axillae, and groin. Intrinsic desmosomal fragility

2014 eMedicine.com

144. Intravenous Immunoglobulin (Diagnosis)

, corticosteroid-resistant extensive, GCSF-induced Sweet syndrome. [ ] Miscellaneous Adrenoleukodystrophy Acute cardiomyopathy Congenital heart block Autoimmune blistering dermatosis Acute idiopathic dysautonomia Endotoxemia Hemophagocytic syndrome Lower motor neuron syndrome Human T-cell lymphotrophic virus-1–associated myelopathy Nephritic syndrome Membranous nephropathy Euthyroid ophthalmopathy Opsoclonus-myoclonus Recurrent Paraneoplastic cerebellar degeneration Paraproteinemic neuropathy (general (...) in highly sensitized patients. Contrib Nephrol . 2009. 162:27-34. . Amagai M, Ikeda S, Shimizu H, et al. A randomized double-blind trial of intravenous immunoglobulin for pemphigus. J Am Acad Dermatol . 2009 Apr. 60(4):595-603. . Anthony RM, Wermeling F, Karlsson MC, Ravetch JV. Identification of a receptor required for the anti-inflammatory activity of IVIG. Proc Natl Acad Sci U S A . 2008 Dec 16. 105(50):19571-8. . . Bonilla FA. Intravenous immunoglobulin: adverse reactions and management. J Allergy

2014 eMedicine.com

145. Oral Manifestations of Systemic Diseases (Diagnosis)

, purpura, paraneoplastic pemphigus, Sweet syndrome) or therapy-induced lesions (eg, drug reactions, graft vs host disease). [ , ] Oral manifestations are more common in acute leukemias than in chronic leukemias. [ ] Gingival hypertrophy and hyperplasia are most commonly associated with acute myelogenous leukemia and acute promyelocytic leukemia. [ ] The gingiva are friable, edematous, and erythematous. [ , ] Thrombocytopenia commonly manifests as petechiae and ecchymoses on the mucosal surfaces (...) myeloma accounts for 1.3% of new cancer diagnoses and 1.9% of all cancer deaths. [ ] Cutaneous manifestations in multiple myeloma are rare, although multiple myeloma has been associated with certain paraneoplastic conditions, including erythema gyratum repens and pityriasis rotunda. [ ] Patients often present with osteolytic lesions, and about 30% of patients have mandibular involvement, with associated swelling, pain, paresthesias, and tooth loss. [ ] Approximately 14% of patients have oral

2014 eMedicine.com

146. Oral Manifestations of Autoimmune Blistering Diseases (Diagnosis)

. This article discusses the oral manifestations of several well-characterized autoimmune blistering diseases, including pemphigus vulgaris, bullous pemphigoid, linear immunoglobulin A (IgA) bullous dermatosis, and paraneoplastic pemphigus (PNP). A group of autoimmune blistering diseases affecting primarily the mucous membranes is termed mucous membrane pemphigoid (MMP) (also termed ). Because this topic is discussed in a separate article, it is not described in great detail in this article. Animal models (...) Spontaneous animal homologues of human autoimmune blistering diseases have been identified in the last 2 decades. [ ] Those diseases in which oral involvement occurs include pemphigus vulgaris (dogs, cats), paraneoplastic pemphigus (dog, cat), [ ] bullous pemphigoid (dogs, cats, horses, pigs), [ , ] mucous membrane pemphigoid (dogs, cats), [ ] linear IgA bullous dermatosis (dogs), epidermolysis bullosa acquisita (dogs), and bullous systemic lupus erythematosus (1 dog). The histopathologic

2014 eMedicine.com

147. Thymoma (Overview)

proliferation, autoimmunity, and/or immunodeficiency. Autoimmunity also may be caused by cross-immunity of antigens in other tissues with thymoma-associated antigens. Thymomas are usually encapsulated, locally spreading tumors. More than one system of classifying thymoma has been established (see Histologic Findings, Staging). Seventy percent of thymomas are associated with paraneoplastic syndromes such as myasthenia gravis (MG), red cell aplasia, pemphigus, and immunoglobulin (Ig) deficiency. Myasthenia (...) . [ ] Normal thymic epithelium tissue arises from the third branchial cleft and the third and fourth branchial pouches. Dendritic cells and macrophages found in large quantities at the corticomedullary junction arise from mesodermal tissues (bone marrow). The epithelial cells and these other stromal tissues of the thymus influence the selection and maturation of the T lymphocytes. Dysregulation of this system in thymoma is believed to be a cause of accompanying paraneoplastic syndromes. In the normal

2014 eMedicine Pediatrics

148. Cutaneous manifestations of gastrointestinal disease: Part I. (PubMed)

dermatomyositis, and paraneoplastic pemphigus. Each of these disease processes have been shown to be associated with an increased risk of GI malignancy. This underscores the important role of dermatologists in the diagnosis, detection, monitoring, and treatment of these disorders while consulting and interacting with their GI colleagues.Copyright © 2012 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved. (...) Cutaneous manifestations of gastrointestinal disease: Part I. Cutaneous findings are not uncommonly a concomitant finding in patients afflicted with gastrointestinal (GI) diseases. The dermatologic manifestations may precede clinically evident GI disease. Part I of this 2-part CME review focuses on dermatologic findings as they relate to hereditary and nonhereditary polyposis disorders and paraneoplastic disorders. A number of hereditary GI disorders have an increased risk of colorectal

2013 Journal of American Academy of Dermatology

149. Clinical Relevance of Autoantibodies in Patients with Autoimmune Bullous Dermatosis (PubMed)

Clinical Relevance of Autoantibodies in Patients with Autoimmune Bullous Dermatosis The authors present their experience related to the diagnosis, treatment, and followup of 431 patients with bullous pemphigoid, 14 patients with juvenile bullous pemphigoid, and 273 patients with pemphigus. The detection of autoantibodies plays an outstanding role in the diagnosis and differential diagnosis. Paraneoplastic pemphigoid is suggested to be a distinct entity from the group of bullous pemphigoid (...) , linear IgA bullous dermatosis, and IgA pemphigus), diamino diphenyl sulfone (dapsone) therapy is preferred alone or in combination. The clinical relevance of autoantibodies in patients with autoimmune bullous dermatosis is stressed.

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2012 Clinical and Developmental Immunology

150. Spectrum of autoimmune bullous diseases in Iran: a 10-year review. (PubMed)

with dermatitis herpetiformis were not included.A total of 1402 patients were diagnosed with ABD during the study period. Pemphigus vulgaris (PV) was the most common ABD (81.2%), followed by bullous pemphigoid (BP) (11.6%), pemphigus foliaceus (PF) (4.4%), pemphigoid gestationis (0.7%), mucous membrane pemphigoid (0.7%), epidermolysis bullosa acquisita (0.5%), linear immunoglobulin A (IgA) disease (0.4%), paraneoplastic pemphigus (0.2%), IgA pemphigus (0.2%), and pemphigus erythematosus (0.1%). The mean age (...) Spectrum of autoimmune bullous diseases in Iran: a 10-year review. Autoimmune bullous diseases (ABDs) are potentially devastating bullous dermatoses of the skin and mucosae characterized by the presence of tissue-bound and circulating antibodies directed against disease-specific target antigens. These diseases comprise two major subgroups of subepidermal autoimmune bullous disorders and pemphigus, based on the level of blister formation. Although they occur worldwide, the relative frequencies

2012 International Journal of Dermatology

151. Bullous mycosis fungoides associated with an extensive ulcer and a severe leukemoid reaction (PubMed)

Bullous mycosis fungoides associated with an extensive ulcer and a severe leukemoid reaction This report presents a case of bullous mycosis fungoides associated with an extensive ulcer and a severe leukemoid reaction. The rash began as indurated erythema which was always followed by ulceration. The rashes initially responded to radiation therapy, but multiple recurrences appeared. Several bullae appeared on the trunk during the course of the illness, without any evidence of paraneoplastic (...) pemphigus. Finally, the ulcer covered a large part of the trunk, and the patient died of sepsis with an extreme leukocyte count of 118,000/µL. A bone marrow analysis revealed a leukemoid reaction and an autopsy revealed pseudomembranous colitis.

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2011 Dermatology Reports

152. Can deep radiation therapy lead to Erythema Multiforme (Stevens-Johnson Syndrome)?

of EM, SJS, or TEN. The major entity in the differential diagnosis of a cutaneous eruption limited to a radiation port is radiation recall phenomenon, which occurs subsequent to the administration of particular chemotherapeutic drugs. In addition, there are scattered case reports of bullous pemphigoid, paraneoplastic pemphigus, and pemphigus vulgaris beginning within sites of radiation therapy. See references below: Other references: 1. J DAVIS, GT PACK - Archives of Dermatology, 1952 - Am Med Assoc

2010 TRIP Answers

153. Skin Manifestations of Internal Disease

of seborrheic keratoses in healthy adults, this sign may be overdiagnosed. Leser-Trélat Sign © Springer Science+Business Media (Sweet syndrome) is sometimes associated with hematologic cancer. Acanthosis nigricans that is associated with cancer can be of rapid onset and particularly widespread. Acquired ichthyosis or pruritus without a clearly associated dermatitis may indicate occult cancer, often lymphoma. Acanthosis Nigricans Images provided by Thomas Habif, MD. Paraneoplastic pemphigus is a relatively

2013 Merck Manual (19th Edition)

154. Bullous Pemphigoid

: ). Table Distinguishing Bullous Pemphigoid From Pemphigus Vulgaris Disorder Appearance of Lesion Oral Involvement Itching Nikolsky Sign Prognosis Bullous pemphigoid Tense bullae that developed on normal-appearing or erythematous skin or urticarial-appearing Rare, with small blisters Common Generally negative Usually good; occasionally fatal in the elderly Pemphigus vulgaris Flaccid bullae of various sizes Often shearing off of skin or mucosa, leaving painful erosions Typically starts in the mouth (...) Absent Positive Mortality ≤ 10% with treatment; higher without treatment Test results help differentiate bullous pemphigoid from , , , , , paraneoplastic pemphigoid, , and . If bullous pemphigoid is suspected, skin biopsy is done for histology and direct immunofluorescence testing. Samples from in and around the lesion itself are often used for histology, but samples of uninvolved skin (often about 3 mm from the edge of a lesion) are used for direct immunofluorescence. The blister in bullous

2013 Merck Manual (19th Edition)

155. Modern diagnosis of autoimmune blistering skin diseases. (PubMed)

a dozen entities is increasingly important since more diverse treatment options are employed. Exact diagnosis is also pivotal for the prognosis, since some autoimmune bullous diseases may indicate an underlying tumor. Association with a malignancy has been shown in paraneoplastic pemphigus (in 100%) and anti-laminin 332 mucous pemphigoid (in 25%) In pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid, autoantibodies to desmoglein 3, desmoglein 1, and BP180, respectively, have been shown

2010 Autoimmunity reviews

156. Place of human amniotic membrane immunoblotting in the diagnosis of autoimmune bullous dermatoses. (PubMed)

immunofluorescence (IIF), and enzyme-linked immunosorbent assay (ELISA) quantification of anti-BP180-NC16a and anti-BP230, or antidesmoglein (Dsg) 1 and 3 antibodies. There were 56 cases of bullous pemphigoid (BP), 22 cases of mucous membrane pemphigoid (MMP), eight cases of epidermolysis bullosa acquisita (EBA), two cases of bullous systemic lupus erythematosus (BSLE), 17 cases of pemphigus vulgaris (PV), and four cases each of pemphigus foliaceus (PF) and paraneoplastic pemphigus (PNP).In BP, the three methods (...) had similar sensitivity (84-89%) for both anti-BP180-NC16a and anti-BP230 antibody detection. In MMP, autoantibodies (mainly directed against BP180 or laminin 332 subunits) were detected in 77% of patients by IB, compared with only 9% by IIF on rat and monkey oesophagus and 36% on salt-split skin, and 14% by anti-BP180-NC16a and anti-BP230 ELISA. In patients with pemphigus, ELISA had 92% sensitivity for anti-Dsg1 and 3, but IB and rat bladder IIF were necessary to confirm PNP by revealing specific

2009 British Journal of Dermatology

157. Stevens-Johnson Syndrome

. . Bullous phototoxic reactions. Chemical or thermal burns. . . Maculopapular drug rashes. Paraneoplastic pemphigus acantholysis. . . . Investigations Serum electrolytes, glucose and bicarbonate are essential to assess clinical severity and level of dehydration. [ ] Diagnosis is based on clinical classification - as in the table, above - and on histopathology. Skin biopsy: demonstrates that the bullae are subepidermal. Epidermal cell necrolysis may be seen and perivascular areas are infiltrated

2008 Mentor

158. Pemphigus (PubMed)

bullae are subepidermal. Three major variants of the disorder have been described, each with characteristic clinical and immunological features: Pemphigus vulgaris (PV) is the most common subset or variant and accounts for 70% of cases of pemphigus. Pemphigus foliaceus (PF) is characterised by lesions which occur only in the skin and associated with antibodies to desmoglein 1 (DSG1). Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms (...) lesions. Pemphigus foliaceus (PF): Presents with lesions on the skin only, and these patients will not go on to develop oral blisters. Paraneoplastic pemphigus (PNP): It may present in patients who are already known to have an underlying neoplasm such as the B-cell lymphoproliferative disorders (eg, non-Hodgkin's lymphoma and chronic lymphocytic leukaemia). It may present with mucocutaneous lesions before any underlying tumour has been diagnosed. Patients present with painful ulcers on their mucous

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2008 Mentor

159. Benign Chronic Pemphigus (BCPM)

pemphigoid where bullae are subepidermal. Three major variants of the disorder have been described, each with characteristic clinical and immunological features: Pemphigus vulgaris (PV) is the most common subset or variant and accounts for 70% of cases of pemphigus. Pemphigus foliaceus (PF) is characterised by lesions which occur only in the skin and associated with antibodies to desmoglein 1 (DSG1). Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms (...) lesions. Pemphigus foliaceus (PF): Presents with lesions on the skin only, and these patients will not go on to develop oral blisters. Paraneoplastic pemphigus (PNP): It may present in patients who are already known to have an underlying neoplasm such as the B-cell lymphoproliferative disorders (eg, non-Hodgkin's lymphoma and chronic lymphocytic leukaemia). It may present with mucocutaneous lesions before any underlying tumour has been diagnosed. Patients present with painful ulcers on their mucous

2008 Mentor

160. Retroperitoneal liposarcoma associated with small plaque parapsoriasis (PubMed)

Retroperitoneal liposarcoma associated with small plaque parapsoriasis Extremely rare cases of paraneoplastic syndromes or ectopic production of proteins associated with liposarcoma are reported in literature. Production of Granulocyte-Colony Stimulating Factor, alpha-fetoprotein, paraneoplastic pemphigus and leucocytosis, Acrokeratosis paraneoplastica (Bazex's syndrome) are reported. The present report describes a case of retroperitoneal liposarcoma associated with small plaque parapsoriasis (...) syndrome was done.Parallel disappearing of skin eruptions after surgery, typical clinical picture and not specific histology of the cutaneous lesions suggest the diagnosis of small plaque parapsoriasis. Therefore we propose to add Small Plaque Parapsoriasis to the list of paraneoplastic syndromes associated to liposarcoma.

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2007 World journal of surgical oncology

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