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Paraneoplastic Pemphigus

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141. Drug Eruptions (Overview)

of preexisting herpes labialis, facial erythema, fixed drug eruption, hyperpigmentation, nummular eczema, paraneoplastic pemphigus, pruritus, psoriasis, sarcoidosis, SLE, urticaria, and xerostomia IFN-beta - Fatal pemphigus vulgaris (when used in combination with interleukin (IL)–2, localized reactions (common), and urticaria IFN-gamma - Increased relapses in melanoma and localized inflammation IL-1alpha - Mucositis, phlebitis, Shwartzman reaction, and xerostomia IL-1beta - Erythema at surgical wound sites (...) . Calcium channel blockers, cimetidine, griseofulvin, leflunomide, terbinafine, and TNF-alpha inhibitors have been reported. Morbilliform (exanthematous) - ACE inhibitors, allopurinol, amoxicillin, ampicillin, anticonvulsants, barbiturates, carbamazepine, cetirizine, ginkgo biloba, hydroxyzine, isoniazid, nelfinavir, NSAIDs, phenothiazine, phenytoin, quinolones, sulfonamides, thalidomide, thiazides, trimethoprim-sulfamethoxazole, and zalcitabine Pemphigus [ ] : Thiols include captopril, D-penicillamine

2014 eMedicine.com

142. Intravenous Immunoglobulin (Treatment)

, 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

143. Nonneoplastic Epithelial Disorders of the Vulva (Follow-up)

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. Oral Manifestations of Systemic Diseases (Follow-up)

, 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

145. Oral Manifestations of Autoimmune Blistering Diseases (Follow-up)

frequently is required for patients with the pemphigus group of diseases, particularly for those with pemphigus vulgaris and paraneoplastic pemphigus. Transferring patients with extremely severe disease with most of the skin denuded to a burn unit for close skin care may be indicated. Next: Surgical Care Surgical care usually is not needed in treating the oral manifestations of patients with autoimmune blistering diseases. Previous Next: Consultations Examination by pulmonary specialists is recommended (...) for patients with severe oral lesions, especially those patients with paraneoplastic pemphigus if the patients have symptoms or signs suggestive of respiratory difficulty. Respiratory failure and death have been reported in these patients. [ , ] Examination by gastroenterologists is recommended for some patients with severe oral lesions to detect possible involvement of the esophagus. Dysphagia can be an associated symptom. Examination by ophthalmologists experienced in external eye diseases is recommended

2014 eMedicine.com

146. Intravenous Immunoglobulin (Follow-up)

, 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

147. 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

148. 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

149. 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

150. 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

151. 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

152. 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

153. 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

154. 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

155. 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

156. 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

157. 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

158. 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

159. 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)

160. 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)

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