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Pemphigus Vegetans

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61. Bullous Pemphigoid (Diagnosis)

, Watanabe R, et al. Serum levels of BAFF are increased in bullous pemphigoid but not in pemphigus vulgaris. Br J Dermatol . 2006 Aug. 155(2):330-6. . Iwata Y, Komura K, Kodera M, et al. Correlation of IgE autoantibody to BP180 with a severe form of bullous pemphigoid. Arch Dermatol . 2008 Jan. 144(1):41-8. . Di Zenzo G, Calabresi V, Olasz EB, Zambruno G, Yancey KB. Sequential Intramolecular Epitope Spreading of Humoral Responses to Human BPAG2 in a Transgenic Model. J Invest Dermatol . 2009 Oct 8 (...) , 2018. Kridin K, Bergman R. Association of Bullous Pemphigoid With Dipeptidyl-Peptidase 4 Inhibitors in Patients With Diabetes: Estimating the Risk of the New Agents and Characterizing the Patients. JAMA Dermatol . 2018 Aug 8. . Langan SM, Smeeth L, Hubbard R, Fleming KM, Smith CJ, West J. Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study. BMJ . 2008 Jul 9. 337:a180. . . Gajic-Veljic M, Nikolic M, Medenica L. Juvenile bullous pemphigoid:the

2014 eMedicine.com

62. Drug-Induced Bullous Disorders (Diagnosis)

. . Werchniak AE, Chaffee S, Dinulos JG. Methotrexate-induced bullous acral erythema in a child. J Am Acad Dermatol . 2005 May. 52(5 Suppl 1):S93-5. . Korman NJ, Eyre RW, Zone J, Stanley JR. Drug-induced pemphigus: autoantibodies directed against the pemphigus antigen complexes are present in penicillamine and captopril-induced pemphigus. J Invest Dermatol . 1991 Feb. 96(2):273-6. . Pinto GM, Lamarao P, Vale T. Captopril-induced pemphigus vegetans with Charcot-Leyden crystals. J Am Acad Dermatol . 1992 Aug (...) implicated in the development of EM minor in an HIV-positive patient and confirmed with patch testing. [ ] Topical mechlorethamine reportedly caused a subepidermal bullous reaction in a patient with mycosis fungoides. [ ] Methotrexate has been reported to be associated with bullous acral erythema in a child. [ ] The thiol group of drugs is the most common agent implicated in drug-induced pemphigus. Drugs known to cause pemphigus include amoxicillin, ampicillin, captopril, [ , ] cephalosporins

2014 eMedicine.com

63. Oral Manifestations of Systemic Diseases (Treatment)

coincide with exacerbations of the colonic disease. Lesions in the colon consist of areas of hemorrhage and ulceration, along with abscesses. Cutaneous involvement consists of similar ulcerations that may arise on the buttocks, abdomen, thighs, and face, although in rare cases patients may develop pyoderma vegetans. [ , ] In the oral cavity, aphthous ulcers or angular stomatitis occurs in as many as 5-10% of patients, although hemorrhagic ulcers can occur. [ ] Rarely, patients can develop pyostomatitis (...) vegetans (PSV), the oral counterpart of pyoderma vegetans. Also see . Crohn disease Crohn disease is an idiopathic inflammatory disorder that can involve the entire GI tract with transmural inflammation and noncaseating granulomas. The prevalence of Crohn disease varies significantly between populations. In North America, the incidence of Crohn disease can be as high as 20.2 cases per 100,000 population. [ ] Although formerly considered a disease of Western nations, the incidence is rising in Asia

2014 eMedicine.com

64. Bullous Pemphigoid (Treatment)

. Both Th1 and Th2 chemokines are elevated in sera of patients with autoimmune blistering diseases. Arch Dermatol Res . 2006 Jun. 298(1):38-45. . Niimi Y, Pawankar R, Kawana S. Increased expression of matrix metalloproteinase-2, matrix metalloproteinase-9 and matrix metalloproteinase-13 in lesional skin of bullous pemphigoid. Int Arch Allergy Immunol . 2006. 139(2):104-13. . Asashima N, Fujimoto M, Watanabe R, et al. Serum levels of BAFF are increased in bullous pemphigoid but not in pemphigus (...) in Patients With Diabetes: Estimating the Risk of the New Agents and Characterizing the Patients. JAMA Dermatol . 2018 Aug 8. . Langan SM, Smeeth L, Hubbard R, Fleming KM, Smith CJ, West J. Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study. BMJ . 2008 Jul 9. 337:a180. . . Gajic-Veljic M, Nikolic M, Medenica L. Juvenile bullous pemphigoid:the presentation and follow-up of six cases. J Eur Acad Dermatol Venereol . Jan/2010. 24:69-72. . Zhou XP, Liu B

2014 eMedicine.com

65. 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

66. 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

67. 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

68. Oral Manifestations of Systemic Diseases (Overview)

coincide with exacerbations of the colonic disease. Lesions in the colon consist of areas of hemorrhage and ulceration, along with abscesses. Cutaneous involvement consists of similar ulcerations that may arise on the buttocks, abdomen, thighs, and face, although in rare cases patients may develop pyoderma vegetans. [ , ] In the oral cavity, aphthous ulcers or angular stomatitis occurs in as many as 5-10% of patients, although hemorrhagic ulcers can occur. [ ] Rarely, patients can develop pyostomatitis (...) vegetans (PSV), the oral counterpart of pyoderma vegetans. Also see . Crohn disease Crohn disease is an idiopathic inflammatory disorder that can involve the entire GI tract with transmural inflammation and noncaseating granulomas. The prevalence of Crohn disease varies significantly between populations. In North America, the incidence of Crohn disease can be as high as 20.2 cases per 100,000 population. [ ] Although formerly considered a disease of Western nations, the incidence is rising in Asia

2014 eMedicine.com

69. Drug-Induced Bullous Disorders (Overview)

. . Werchniak AE, Chaffee S, Dinulos JG. Methotrexate-induced bullous acral erythema in a child. J Am Acad Dermatol . 2005 May. 52(5 Suppl 1):S93-5. . Korman NJ, Eyre RW, Zone J, Stanley JR. Drug-induced pemphigus: autoantibodies directed against the pemphigus antigen complexes are present in penicillamine and captopril-induced pemphigus. J Invest Dermatol . 1991 Feb. 96(2):273-6. . Pinto GM, Lamarao P, Vale T. Captopril-induced pemphigus vegetans with Charcot-Leyden crystals. J Am Acad Dermatol . 1992 Aug (...) implicated in the development of EM minor in an HIV-positive patient and confirmed with patch testing. [ ] Topical mechlorethamine reportedly caused a subepidermal bullous reaction in a patient with mycosis fungoides. [ ] Methotrexate has been reported to be associated with bullous acral erythema in a child. [ ] The thiol group of drugs is the most common agent implicated in drug-induced pemphigus. Drugs known to cause pemphigus include amoxicillin, ampicillin, captopril, [ , ] cephalosporins

2014 eMedicine.com

70. Dermatologic Manifestations of Gastrointestinal Disease (Overview)

[ ] Esophagitis Scleroderma GI bleeding Hereditary hemorrhagic telangiectasia Cirrhosis Liver disease secondary to alcohol or other factors Vesicles/blisters/erosions Esophageal webs Epidermolysis bullosa Esophageal erosion Pemphigus vulgaris Pyloric atresia Junctional epidermolysis bullosa Hepatitis Porphyria cutanea tarda Malabsorption Dermatitis herpetiformis and celiac sprue Velvety hyperpigmented plaques, tripe palms, mucosal hyperplasia Gastric cancer Malignant acanthosis nigricans Yellowish papules (...) , and rectal prolapse. Neuropathy plays a role in the fecal incontinence. Liver and biliary involvement in SSc is relatively rare; however, primary biliary cirrhosis (PBC) is the most common hepatobiliary manifestation in SSc patients. The onset of PBC may precede, occur concomitantly with, or, more commonly, follow SSc onset. [ ] Pemphigus vulgaris is a rare autoimmune blistering disorder primarily affecting the skin and mucosa. It is characterized by loss of cell-to-cell adhesion in the epithelial

2014 eMedicine.com

71. Cutaneous Manifestations of HIV Disease (Overview)

Multiple dermatofibromas [ ] Recurrent neutrophilic eccrine hidradenitis Pemphigus vegetans Lichen scrofulosorum Cutaneous mucinosis Papulonecrotic tuberculide Kawasaki disease Eruptive dysplastic nevi Dissemination of vaccinia Ofuji disease [ ] Angiomyolipomas Glucan-induced keratoderma [ ] Disseminated superficial porokeratosis [ ] Previous Next: Workup Histopathologic examination is useful to diagnose cutaneous manifestations of HIV disease with atypical clinical features and Kaposi sarcoma (KS

2014 eMedicine.com

72. Drug-Induced Bullous Disorders (Treatment)

. J Am Acad Dermatol . 2005 May. 52(5 Suppl 1):S93-5. . Korman NJ, Eyre RW, Zone J, Stanley JR. Drug-induced pemphigus: autoantibodies directed against the pemphigus antigen complexes are present in penicillamine and captopril-induced pemphigus. J Invest Dermatol . 1991 Feb. 96(2):273-6. . Pinto GM, Lamarao P, Vale T. Captopril-induced pemphigus vegetans with Charcot-Leyden crystals. J Am Acad Dermatol . 1992 Aug. 27(2 Pt 2):281-4. . Ben Salem C, Chenguel L, Ghariani N, Denguezli M, Hmouda H (...) pemphigus). Small vesicle at edge of urticarial plaque (drug-induced pemphigoid). Tense vesicles in annular array (linear immunoglobulin A dermatosis). Erosions, scars, milia, and vesicle (pseudoporphyria). of 9 Tables Contributor Information and Disclosures Author David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic David F

2014 eMedicine.com

73. Cutaneous Manifestations of HIV Disease (Treatment)

Multiple dermatofibromas [ ] Recurrent neutrophilic eccrine hidradenitis Pemphigus vegetans Lichen scrofulosorum Cutaneous mucinosis Papulonecrotic tuberculide Kawasaki disease Eruptive dysplastic nevi Dissemination of vaccinia Ofuji disease [ ] Angiomyolipomas Glucan-induced keratoderma [ ] Disseminated superficial porokeratosis [ ] Previous Next: Workup Histopathologic examination is useful to diagnose cutaneous manifestations of HIV disease with atypical clinical features and Kaposi sarcoma (KS

2014 eMedicine.com

74. Dermatologic Manifestations of Gastrointestinal Disease (Treatment)

[ ] Esophagitis Scleroderma GI bleeding Hereditary hemorrhagic telangiectasia Cirrhosis Liver disease secondary to alcohol or other factors Vesicles/blisters/erosions Esophageal webs Epidermolysis bullosa Esophageal erosion Pemphigus vulgaris Pyloric atresia Junctional epidermolysis bullosa Hepatitis Porphyria cutanea tarda Malabsorption Dermatitis herpetiformis and celiac sprue Velvety hyperpigmented plaques, tripe palms, mucosal hyperplasia Gastric cancer Malignant acanthosis nigricans Yellowish papules (...) , and rectal prolapse. Neuropathy plays a role in the fecal incontinence. Liver and biliary involvement in SSc is relatively rare; however, primary biliary cirrhosis (PBC) is the most common hepatobiliary manifestation in SSc patients. The onset of PBC may precede, occur concomitantly with, or, more commonly, follow SSc onset. [ ] Pemphigus vulgaris is a rare autoimmune blistering disorder primarily affecting the skin and mucosa. It is characterized by loss of cell-to-cell adhesion in the epithelial

2014 eMedicine.com

75. 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

76. Oral Manifestations of Systemic Diseases (Follow-up)

coincide with exacerbations of the colonic disease. Lesions in the colon consist of areas of hemorrhage and ulceration, along with abscesses. Cutaneous involvement consists of similar ulcerations that may arise on the buttocks, abdomen, thighs, and face, although in rare cases patients may develop pyoderma vegetans. [ , ] In the oral cavity, aphthous ulcers or angular stomatitis occurs in as many as 5-10% of patients, although hemorrhagic ulcers can occur. [ ] Rarely, patients can develop pyostomatitis (...) vegetans (PSV), the oral counterpart of pyoderma vegetans. Also see . Crohn disease Crohn disease is an idiopathic inflammatory disorder that can involve the entire GI tract with transmural inflammation and noncaseating granulomas. The prevalence of Crohn disease varies significantly between populations. In North America, the incidence of Crohn disease can be as high as 20.2 cases per 100,000 population. [ ] Although formerly considered a disease of Western nations, the incidence is rising in Asia

2014 eMedicine.com

77. Drug-Induced Bullous Disorders (Follow-up)

. J Am Acad Dermatol . 2005 May. 52(5 Suppl 1):S93-5. . Korman NJ, Eyre RW, Zone J, Stanley JR. Drug-induced pemphigus: autoantibodies directed against the pemphigus antigen complexes are present in penicillamine and captopril-induced pemphigus. J Invest Dermatol . 1991 Feb. 96(2):273-6. . Pinto GM, Lamarao P, Vale T. Captopril-induced pemphigus vegetans with Charcot-Leyden crystals. J Am Acad Dermatol . 1992 Aug. 27(2 Pt 2):281-4. . Ben Salem C, Chenguel L, Ghariani N, Denguezli M, Hmouda H (...) pemphigus). Small vesicle at edge of urticarial plaque (drug-induced pemphigoid). Tense vesicles in annular array (linear immunoglobulin A dermatosis). Erosions, scars, milia, and vesicle (pseudoporphyria). of 9 Tables Contributor Information and Disclosures Author David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic David F

2014 eMedicine.com

78. Bullous Pemphigoid (Follow-up)

. Both Th1 and Th2 chemokines are elevated in sera of patients with autoimmune blistering diseases. Arch Dermatol Res . 2006 Jun. 298(1):38-45. . Niimi Y, Pawankar R, Kawana S. Increased expression of matrix metalloproteinase-2, matrix metalloproteinase-9 and matrix metalloproteinase-13 in lesional skin of bullous pemphigoid. Int Arch Allergy Immunol . 2006. 139(2):104-13. . Asashima N, Fujimoto M, Watanabe R, et al. Serum levels of BAFF are increased in bullous pemphigoid but not in pemphigus (...) in Patients With Diabetes: Estimating the Risk of the New Agents and Characterizing the Patients. JAMA Dermatol . 2018 Aug 8. . Langan SM, Smeeth L, Hubbard R, Fleming KM, Smith CJ, West J. Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study. BMJ . 2008 Jul 9. 337:a180. . . Gajic-Veljic M, Nikolic M, Medenica L. Juvenile bullous pemphigoid:the presentation and follow-up of six cases. J Eur Acad Dermatol Venereol . Jan/2010. 24:69-72. . Zhou XP, Liu B

2014 eMedicine.com

79. Dermatologic Manifestations of Gastrointestinal Disease (Follow-up)

[ ] Esophagitis Scleroderma GI bleeding Hereditary hemorrhagic telangiectasia Cirrhosis Liver disease secondary to alcohol or other factors Vesicles/blisters/erosions Esophageal webs Epidermolysis bullosa Esophageal erosion Pemphigus vulgaris Pyloric atresia Junctional epidermolysis bullosa Hepatitis Porphyria cutanea tarda Malabsorption Dermatitis herpetiformis and celiac sprue Velvety hyperpigmented plaques, tripe palms, mucosal hyperplasia Gastric cancer Malignant acanthosis nigricans Yellowish papules (...) , and rectal prolapse. Neuropathy plays a role in the fecal incontinence. Liver and biliary involvement in SSc is relatively rare; however, primary biliary cirrhosis (PBC) is the most common hepatobiliary manifestation in SSc patients. The onset of PBC may precede, occur concomitantly with, or, more commonly, follow SSc onset. [ ] Pemphigus vulgaris is a rare autoimmune blistering disorder primarily affecting the skin and mucosa. It is characterized by loss of cell-to-cell adhesion in the epithelial

2014 eMedicine.com

80. Cutaneous Manifestations of HIV Disease (Follow-up)

Multiple dermatofibromas [ ] Recurrent neutrophilic eccrine hidradenitis Pemphigus vegetans Lichen scrofulosorum Cutaneous mucinosis Papulonecrotic tuberculide Kawasaki disease Eruptive dysplastic nevi Dissemination of vaccinia Ofuji disease [ ] Angiomyolipomas Glucan-induced keratoderma [ ] Disseminated superficial porokeratosis [ ] Previous Next: Workup Histopathologic examination is useful to diagnose cutaneous manifestations of HIV disease with atypical clinical features and Kaposi sarcoma (KS

2014 eMedicine.com

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