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Paraneoplastic Pemphigus

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122. Nonneoplastic Epithelial Disorders of the Vulva (Overview)

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

123. Benign Vulvar Lesions (Follow-up)

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: , lentiginosis, and benign (...) 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

124. Benign Vulvar Lesions (Diagnosis)

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: , lentiginosis, and benign (...) 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

125. Drug Eruptions (Diagnosis)

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

126. Oral Manifestations of Systemic Diseases (Treatment)

, 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

127. Oral Manifestations of Autoimmune Blistering Diseases (Treatment)

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

128. Chronic Lymphocytic Leukemia (Treatment)

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

129. Benign Vulvar Lesions (Treatment)

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: , lentiginosis, and benign (...) 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

130. Nonneoplastic Epithelial Disorders of the Vulva (Treatment)

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

131. Benign Vulvar Lesions (Overview)

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: , lentiginosis, and benign vulvar (...) 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 may only become evident when trigger

2014 eMedicine.com

132. Intravenous Immunoglobulin (Overview)

, 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

133. Oral Manifestations of Systemic Diseases (Overview)

, 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

134. Oral Manifestations of Autoimmune Blistering Diseases (Overview)

. 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

135. 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

136. 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

137. 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

138. 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

139. 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

140. 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

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