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Pemphigus Foliaceus

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141. Autoantibody Levels and Clinical Disease Severity in Patients with Pemphigus: Comparison of Aggregated Anti-desmoglein ELISA Values and Indirect Immunofluorescence Titres. (PubMed)

values and IIF titres with clinical activity over a longer treatment period are scarce. In our retrospective study, we compared aggregated repetitive intra-patient ELISA values and IIF titres with grades of clinical disease (1 = remission, 2 = moderate activity, 3 = exacerbation) in 47 patients suffering from pemphigus vulgaris (PV, n = 36) and pemphigus foliaceus (PF, n=11). We found that anti-Dsg1 ELISA values in PF and mucocutaneous PV as well as anti-Dsg3 ELISA values in PV best reflect disease (...) Autoantibody Levels and Clinical Disease Severity in Patients with Pemphigus: Comparison of Aggregated Anti-desmoglein ELISA Values and Indirect Immunofluorescence Titres. Detecting serum-autoantibodies by anti-Desmoglein-1 (anti-Dsg1) and anti-Dsg3 ELISAs as well as indirect immunofluorescence (IIF) are established complementary methods to diagnose pemphigus. Whether autoantibody levels also reflect clinical disease activity is still a matter of debate, as head-to-head comparisons of ELISA

Full Text available with Trip Pro

2014 Acta Dermato-Venereologica

142. Correlation of Conventional and Conformational Anti-desmoglein Antibodies with Phenotypes and Disease Activities in Patients with Pemphigus Vulgaris. (PubMed)

Correlation of Conventional and Conformational Anti-desmoglein Antibodies with Phenotypes and Disease Activities in Patients with Pemphigus Vulgaris. Pemphigus is an autoimmune disease characterised by anti-desmoglein (Dsg) antibodies in the serum of patients. The disease can be divided into pemphigus foliaceus and pemphigus vulgaris. Anti-Dsg1 antibody is generally related to pemphigus with cutaneous lesion, and the anti-Dsg3 antibody is related to pemphigus with mucosa lesion. Twenty-nine (...) patients with pemphigus vulgaris were selected in the clinical study. The severity of the cutaneous and mucosa lesions of these patients was evaluated using Pemphigus disease area index (PDAI). Conventional and conformational anti-Dsg index values were determined using enzyme-linked immunosorbent assay (ELISA) and ethylenediaminetetraacetic acid-treated ELISA. The relationship between clinical phenotypes and immunological profiles was analysed. In the correlation analysis, both the conventional

Full Text available with Trip Pro

2014 Acta Dermato-Venereologica

143. Prevalence of myasthenia gravis and associated autoantibodies in paraneoplastic pemphigus and their correlations with symptoms and prognosis. (PubMed)

. Serum autoantibodies against acetylcholine receptor (AChR), acetylcholinesterase (AChE), titin, ryanodine receptor (RyR) and muscle-specific kinase (MuSK) were measured by enzyme-linked immunosorbent assay. Patients with pemphigus vulgaris (PV), pemphigus foliaceus (PF), connective tissue disease (CTD) and non-PNP MG (NP-MG), and healthy donors, served as controls. These autoantibodies in PNP were also compared in the presence or absence of dyspnoea or muscle weakness. Cox regression and log-rank (...) Prevalence of myasthenia gravis and associated autoantibodies in paraneoplastic pemphigus and their correlations with symptoms and prognosis. Paraneoplastic pemphigus (PNP) involves multiple organs, but little is known about its neurological involvement.To investigate the symptoms, prognosis and profiles of associated autoantibodies in myasthenia gravis (MG), and their correlations in patients with PNP.Fifty-eight patients with PNP were assessed for myasthenic symptoms and laboratory evidence

2014 British Journal of Dermatology

144. Pemphigus, Paraneoplastic (Overview)

(desmoglein 3, 130-kd) and pemphigus foliaceus (desmoglein 1, 160-kd), as well as several others, including epiplakin. [ ] Antibodies are typically IgG, although IgA has been reported. [ ] In a 2011 review, Czernik et al . summarize the increasing role of cellular immunity in paraneoplastic pemphigus, evidenced by lesional mononuclear cells and elevated IL-6 levels in sera from paraneoplastic pemphigus patients. [ ] Patients with paraneoplastic pemphigus without identifiable autoantibodies have been (...) Pemphigus, Paraneoplastic (Overview) Paraneoplastic Pemphigus: Background, Pathophysiology, Etiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA2NDQ1Mi1vdmVydmlldw== processing > Paraneoplastic Pemphigus

2014 eMedicine.com

145. Pemphigus, Drug-Induced (Overview)

or tissue bound. [ ] Since the 1950s, evidence has grown that drugs may cause or exacerbate pemphigus. A drug origin should be considered in every new patient with pemphigus. The most common variant of pemphigus associated with drug exposure is , although has also been described. In penicillamine-treated patients, pemphigus foliaceus is more common than pemphigus vulgaris, with an approximate ratio of 4:1. Note the images below. Early small blister filled with clear fluid arises on healthy skin. Flaccid (...) case report describes localized pemphigus foliaceus induced by topical imiquimod treatment. Imiquimod does not contain thiol, sulfur, or amide groups in its structure. The exact mechanism of acantholysis induction from this medication is unknown. Because imiquimod is known to cause a localized immune response at the site of application, the generation of antibodies to desmoglein 1 has been postulated as a mechanism of action. Previous Next: Epidemiology Frequency More than 200 cases of drug-induced

2014 eMedicine.com

146. Pemphigus Vulgaris (Overview)

of other diseases. The outlook is worse in older patients and in patients with extensive disease. Prognosis is usually better in childhood than in adulthood. A few rare cases of pemphigus vulgaris transitioning to pemphigus foliaceus have been reported. Previous Next: Patient Education Minimize trauma to the skin because the patient's skin is fragile both from the disease and from the use of topical and systemic steroids. The patient's understanding of the disease and education about pemphigus vulgaris (...) of the Pathogenesis of Pemphigus and Pemphigoid Meeting in Munich, September 2016. J Invest Dermatol . 2017 Jun. 137 (6):1199-1203. . Mentink LF, de Jong MC, Kloosterhuis GJ, Zuiderveen J, Jonkman MF, Pas HH. Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. Br J Dermatol . 2007 Apr. 156(4):635-41. . Bhol K, Mohimen A, Ahmed AR. Correlation of subclasses of IgG with disease activity in pemphigus vulgaris. Dermatology . 1994. 189 Suppl 1

2014 eMedicine.com

147. Pemphigus Erythematosus (Overview)

Updated: Mar 02, 2018 Author: Rakesh Bharti, MD, MBBS; Chief Editor: William D James, MD Share Email Print Feedback Close Sections Sections Pemphigus Erythematosus Overview Background The various types of pemphigus include pemphigus erythematosus, pemphigoid, pemphigus vegetans, pemphigus vulgaris, and . Pemphigus erythematosus, also known as Senear-Usher syndrome, is an overlap syndrome with features of lupus erythematosus (LE) and pemphigus foliaceus. Pemphigus is demonstrated by acantholysis (...) and immunoglobulin deposits in the interkeratinocyte substance (see the image below). Direct immunofluorescence microscopy performed on epithelial biopsy specimen obtained from a patient with pemphigus vulgaris detects immunoglobulin G deposits at the epithelial cell surfaces. Pérez-Pérez et al recently hypothesized that pemphigus erythematosus is a multiple autoimmune disease. [ ] High doses of UV light are suggested to be the cause of cleavage of the desmoglein-1 ectodomain. As in cases of pemphigus foliaceus

2014 eMedicine.com

148. Pemphigus Herpetiformis (Overview)

affected with pemphigus herpetiformis. At least 2 cases of pemphigus herpetiformis have been reported to occur in association with lung cancer. Whether this association was coincidental is not clear. [ , ] In addition, pemphigus herpetiformis has been associated with prostate cancer development in one case. [ ] Previous References Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol . 2011 Jul-Aug. 29(4):432-6. . Laws PM, Heelan K, Al (...) reports. Dermatologica Sinica . 2010 Mar. 28 (1):36-39. Robinson ND, Hashimoto T, Amagai M, Chan LS. The new pemphigus variants. J Am Acad Dermatol . 1999 May. 40 (5 Pt 1):649-71;672-3. . Jablonska S, Chorzelski TP, Beutner EH, Chorzelska J. Herpetiform pemphigus, a variable pattern of pemphigus. Int J Dermatol . 1975 Jun. 14 (5):353-9. . Cunha PR, Jiao D, Bystryn JC. Simultaneous occurrence of herpetiform pemphigus and endemic pemphigus foliaceus (fogo selvagem). Int J Dermatol . 1997 Nov. 36 (11

2014 eMedicine.com

149. Pemphigus, IgA (Treatment)

pemphigus foliaceus with a clinical presentation of pemphigus herpetiformis. J Am Acad Dermatol . 1991 May. 24(5 Pt 2):839-44. . Erdag G, Qureshi HS, Greer KE, Patterson JW. Immunoglobulin A pemphigus involving the perianal skin and oral mucosa: an unusual presentation. Cutis . 2007 Sep. 80(3):218-20. . Neethu KC, Rao R, Balachandran C, Pai S. Juvenile IgA pemphigus: A case report and review of literature. Indian J Dermatol Venereol Leprol . 2016 Jul-Aug. 82 (4):439-42. . Porro AM, Caetano Lde V (...) Pemphigus, IgA (Treatment) IgA Pemphigus Treatment & Management: Medical Care, Activity, Complications Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA2Mzc3Ni10cmVhdG1lbnQ= processing > IgA Pemphigus Treatment

2014 eMedicine.com

150. Pemphigus, Drug-Induced (Treatment)

: changes in anti-32-2B immunostaining patterns]. Ann Dermatol Venereol . 2013 Aug-Sep. 140(8-9):531-4. . Wolf R, Brenner S. An active amide group in the molecule of drugs that induce pemphigus: a casual or causal relationship?. Dermatology . 1994. 189(1):1-4. . Brenner S, Bialy-Golan A, Anhalt GJ. Recognition of pemphigus antigens in drug-induced pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol . 1997 Jun. 36(6 Pt 1):919-23. . Ramseur WL, Richards F 2nd, Duggan DB. A case of fatal (...) pemphigus vulgaris in association with beta interferon and interleukin-2 therapy. Cancer . 1989 May 15. 63(10):2005-7. . Nagao K, Tanikawa A, Yamamoto N, Amagai M. Decline of anti-desmoglein 1 IgG ELISA scores by withdrawal of D-penicillamine in drug-induced pemphigus foliaceus. Clin Exp Dermatol . 2005 Jan. 30(1):43-5. . Hur JW, Lee CW, Yoo DH. Bucillamine-induced pemphigus vulgaris in a patient with rheumatoid arthritis and polymyositis overlap syndrome. J Korean Med Sci . 2006 Jun. 21(3):585-7

2014 eMedicine.com

151. Pemphigus Vulgaris (Treatment)

of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. Br J Dermatol . 2007 Apr. 156(4):635-41. . Bhol K, Mohimen A, Ahmed AR. Correlation of subclasses of IgG with disease activity in pemphigus vulgaris. Dermatology . 1994. 189 Suppl 1:85-9. . Wilson CL, Wojnarowska F, Dean D, Pasricha JS. IgG subclasses in pemphigus in Indian and UK populations. Clin Exp Dermatol . 1993 May. 18(3):226-30. . Fitzpatrick RE, Newcomer VD. The correlation (...) lesion. Oral Surg Oral Med Oral Pathol . 1974 Sep. 38(3):382-7. . Simon DG, Krutchkoff D, Kaslow RA, Zarbo R. Pemphigus in Hartford County, Connecticut, from 1972 to 1977. Arch Dermatol . 1980 Sep. 116(9):1035-7. . Bastuji-Garin S, Souissi R, Blum L, et al. Comparative epidemiology of pemphigus in Tunisia and France: unusual incidence of pemphigus foliaceus in young Tunisian women. J Invest Dermatol . 1995 Feb. 104(2):302-5. . Hietanen J, Salo OP. Pemphigus: an epidemiological study of patients

2014 eMedicine.com

152. Pemphigus Herpetiformis (Treatment)

) patients are responding clinically to prescribed medications and (2) patients experience no serious adverse effects that require a change of medications. Typically, follow-up visits are not needed for patients in clinical remission and not on treatment. Previous References Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol . 2011 Jul-Aug. 29(4):432-6. . Laws PM, Heelan K, Al-Mohammedi F, Walsh S, Shear NH. Pemphigus herpetiformis: a case (...) . Robinson ND, Hashimoto T, Amagai M, Chan LS. The new pemphigus variants. J Am Acad Dermatol . 1999 May. 40 (5 Pt 1):649-71;672-3. . Jablonska S, Chorzelski TP, Beutner EH, Chorzelska J. Herpetiform pemphigus, a variable pattern of pemphigus. Int J Dermatol . 1975 Jun. 14 (5):353-9. . Cunha PR, Jiao D, Bystryn JC. Simultaneous occurrence of herpetiform pemphigus and endemic pemphigus foliaceus (fogo selvagem). Int J Dermatol . 1997 Nov. 36 (11):850-4. . Faria PCP, Cruz CC, Abulafia LA, Maceira JMP

2014 eMedicine.com

153. Pemphigus Erythematosus (Treatment)

, Anandan S, Magesh K. Current concepts of immunofluorescence in oral mucocutaneous diseases. J Oral Maxillofac Pathol . 2011 Sep. 15(3):261-6. . . Singh S. Evidence-based treatments for pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid: a systematic review. Indian J Dermatol Venereol Leprol . 2011 Jul-Aug. 77(4):456-69. . Griffies JD, Mendelsohn CL, Rosenkrantz WS, et al. Topical 0.1% tacrolimus for the treatment of discoid lupus erythematosus and pemphigus erythematosus in dogs. J Am (...) and bullous pemphigoid. Arch Dermatol . 2003 Feb. 139(2):225-6. . Cohen MA, Cohen JJ, Kerdel FA. Immunoablative high-dose cyclophosphamide without stem cell rescue in pemphigus foliaceus. Int J Dermatol . 2002 Jun. 41(6):340-4. . Hayag MV, Cohen JA, Kerdel FA. Immunoablative high-dose cyclophosphamide without stem cell rescue in a patient with pemphigus vulgaris. J Am Acad Dermatol . 2000 Dec. 43(6):1065-9. . Nousari HC, Brodsky RA, Jones RJ, Grever MR, Anhalt GJ. Immunoablative high-dose cyclophosphamide

2014 eMedicine.com

154. Pemphigus, IgA (Overview)

with having IgA-pemphigus foliaceus (IgA-PF) or IgA-pemphigus vulgaris (IgA-PV), respectively. All other cases not meeting these criteria are diagnosed as unclassified IAD. IgA pemphigus has not been described in animals. Although the term IgA pemphigus has not been established in the veterinary literature, IgA deposition around epidermal cells has been detected by direct immunofluorescence in animals affected with pemphigus foliaceus. Medscape articles on other variants of pemphigus include (...) , Hashimoto T, et al. Autoantibodies against desmocollins in European patients with pemphigus. Clin Exp Dermatol . 2009 Dec. 34(8):898-903. . Chan LS, Vanderlugt CJ, Hashimoto T, et al. Epitope spreading: lessons from autoimmune skin diseases. J Invest Dermatol . 1998 Feb. 110(2):103-9. . Chorzelski TP, Beutner EH, Kowalewski C, et al. IgA pemphigus foliaceus with a clinical presentation of pemphigus herpetiformis. J Am Acad Dermatol . 1991 May. 24(5 Pt 2):839-44. . Erdag G, Qureshi HS, Greer KE

2014 eMedicine.com

155. Pemphigus, Drug-Induced (Follow-up)

: changes in anti-32-2B immunostaining patterns]. Ann Dermatol Venereol . 2013 Aug-Sep. 140(8-9):531-4. . Wolf R, Brenner S. An active amide group in the molecule of drugs that induce pemphigus: a casual or causal relationship?. Dermatology . 1994. 189(1):1-4. . Brenner S, Bialy-Golan A, Anhalt GJ. Recognition of pemphigus antigens in drug-induced pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol . 1997 Jun. 36(6 Pt 1):919-23. . Ramseur WL, Richards F 2nd, Duggan DB. A case of fatal (...) pemphigus vulgaris in association with beta interferon and interleukin-2 therapy. Cancer . 1989 May 15. 63(10):2005-7. . Nagao K, Tanikawa A, Yamamoto N, Amagai M. Decline of anti-desmoglein 1 IgG ELISA scores by withdrawal of D-penicillamine in drug-induced pemphigus foliaceus. Clin Exp Dermatol . 2005 Jan. 30(1):43-5. . Hur JW, Lee CW, Yoo DH. Bucillamine-induced pemphigus vulgaris in a patient with rheumatoid arthritis and polymyositis overlap syndrome. J Korean Med Sci . 2006 Jun. 21(3):585-7

2014 eMedicine.com

156. Pemphigus, IgA (Follow-up)

pemphigus foliaceus with a clinical presentation of pemphigus herpetiformis. J Am Acad Dermatol . 1991 May. 24(5 Pt 2):839-44. . Erdag G, Qureshi HS, Greer KE, Patterson JW. Immunoglobulin A pemphigus involving the perianal skin and oral mucosa: an unusual presentation. Cutis . 2007 Sep. 80(3):218-20. . Neethu KC, Rao R, Balachandran C, Pai S. Juvenile IgA pemphigus: A case report and review of literature. Indian J Dermatol Venereol Leprol . 2016 Jul-Aug. 82 (4):439-42. . Porro AM, Caetano Lde V (...) Pemphigus, IgA (Follow-up) IgA Pemphigus Treatment & Management: Medical Care, Activity, Complications Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA2Mzc3Ni10cmVhdG1lbnQ= processing > IgA Pemphigus Treatment

2014 eMedicine.com

157. Pemphigus Erythematosus (Follow-up)

, Anandan S, Magesh K. Current concepts of immunofluorescence in oral mucocutaneous diseases. J Oral Maxillofac Pathol . 2011 Sep. 15(3):261-6. . . Singh S. Evidence-based treatments for pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid: a systematic review. Indian J Dermatol Venereol Leprol . 2011 Jul-Aug. 77(4):456-69. . Griffies JD, Mendelsohn CL, Rosenkrantz WS, et al. Topical 0.1% tacrolimus for the treatment of discoid lupus erythematosus and pemphigus erythematosus in dogs. J Am (...) and bullous pemphigoid. Arch Dermatol . 2003 Feb. 139(2):225-6. . Cohen MA, Cohen JJ, Kerdel FA. Immunoablative high-dose cyclophosphamide without stem cell rescue in pemphigus foliaceus. Int J Dermatol . 2002 Jun. 41(6):340-4. . Hayag MV, Cohen JA, Kerdel FA. Immunoablative high-dose cyclophosphamide without stem cell rescue in a patient with pemphigus vulgaris. J Am Acad Dermatol . 2000 Dec. 43(6):1065-9. . Nousari HC, Brodsky RA, Jones RJ, Grever MR, Anhalt GJ. Immunoablative high-dose cyclophosphamide

2014 eMedicine.com

158. Pemphigus Herpetiformis (Follow-up)

) patients are responding clinically to prescribed medications and (2) patients experience no serious adverse effects that require a change of medications. Typically, follow-up visits are not needed for patients in clinical remission and not on treatment. Previous References Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol . 2011 Jul-Aug. 29(4):432-6. . Laws PM, Heelan K, Al-Mohammedi F, Walsh S, Shear NH. Pemphigus herpetiformis: a case (...) . Robinson ND, Hashimoto T, Amagai M, Chan LS. The new pemphigus variants. J Am Acad Dermatol . 1999 May. 40 (5 Pt 1):649-71;672-3. . Jablonska S, Chorzelski TP, Beutner EH, Chorzelska J. Herpetiform pemphigus, a variable pattern of pemphigus. Int J Dermatol . 1975 Jun. 14 (5):353-9. . Cunha PR, Jiao D, Bystryn JC. Simultaneous occurrence of herpetiform pemphigus and endemic pemphigus foliaceus (fogo selvagem). Int J Dermatol . 1997 Nov. 36 (11):850-4. . Faria PCP, Cruz CC, Abulafia LA, Maceira JMP

2014 eMedicine.com

159. Pemphigus Vulgaris (Follow-up)

of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. Br J Dermatol . 2007 Apr. 156(4):635-41. . Bhol K, Mohimen A, Ahmed AR. Correlation of subclasses of IgG with disease activity in pemphigus vulgaris. Dermatology . 1994. 189 Suppl 1:85-9. . Wilson CL, Wojnarowska F, Dean D, Pasricha JS. IgG subclasses in pemphigus in Indian and UK populations. Clin Exp Dermatol . 1993 May. 18(3):226-30. . Fitzpatrick RE, Newcomer VD. The correlation (...) lesion. Oral Surg Oral Med Oral Pathol . 1974 Sep. 38(3):382-7. . Simon DG, Krutchkoff D, Kaslow RA, Zarbo R. Pemphigus in Hartford County, Connecticut, from 1972 to 1977. Arch Dermatol . 1980 Sep. 116(9):1035-7. . Bastuji-Garin S, Souissi R, Blum L, et al. Comparative epidemiology of pemphigus in Tunisia and France: unusual incidence of pemphigus foliaceus in young Tunisian women. J Invest Dermatol . 1995 Feb. 104(2):302-5. . Hietanen J, Salo OP. Pemphigus: an epidemiological study of patients

2014 eMedicine.com

160. Pemphigus, Drug-Induced (Diagnosis)

or tissue bound. [ ] Since the 1950s, evidence has grown that drugs may cause or exacerbate pemphigus. A drug origin should be considered in every new patient with pemphigus. The most common variant of pemphigus associated with drug exposure is , although has also been described. In penicillamine-treated patients, pemphigus foliaceus is more common than pemphigus vulgaris, with an approximate ratio of 4:1. Note the images below. Early small blister filled with clear fluid arises on healthy skin. Flaccid (...) case report describes localized pemphigus foliaceus induced by topical imiquimod treatment. Imiquimod does not contain thiol, sulfur, or amide groups in its structure. The exact mechanism of acantholysis induction from this medication is unknown. Because imiquimod is known to cause a localized immune response at the site of application, the generation of antibodies to desmoglein 1 has been postulated as a mechanism of action. Previous Next: Epidemiology Frequency More than 200 cases of drug-induced

2014 eMedicine.com

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