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• W4237835575 abstract "IntroductionBullous pemphigoid (BP) is the most common autoimmune subepidermal vesiculobullous disease usually affecting older patients.1Schmidt E. Della Torre R. Borradori L. Clinical features and practical diagnosis of bullous pemphigoid.Immunol Allergy clin North Am. 2012; 32: 217-232Abstract Full Text Full Text PDF PubMed Scopus (35) Google Scholar In BP, there is no ethnic or racial predilection and it affects both men and women equally.2Gudi V.S. White M.I. Cruickshank N. et al.Annual incidence and mortality of bullous pemphigoid in the Grampian Region of North-east Scotland.Br J Dermatol. 2005; 153: 424-427Crossref PubMed Scopus (126) Google Scholar, 3Bernard P. Enginger V. Venot J. Bedane C. Bonnetblanc J.M. Survival prognosis in pemphigoid. A cohort analysis of 78 patients.Ann Dermatol Venereol. 1995; 122: 751-757PubMed Google Scholar, 4Zillikens D. Wever S. Roth A. Weidenthaler-Barth B. Hashimoto T. Bröcker E.B. Incidence of autoimmune sub- epidermal blistering dermatoses in a region of central Germany.Arch Dermatol. 1995; 131: 957Crossref PubMed Scopus (211) Google Scholar, 5Bernard P. Vaillant L. Labeille B. et al.Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group.Arch Dermatol. 1995; 131: 48Crossref PubMed Scopus (19) Google Scholar Based on the reported data in the Philippine Dermatological Society Health Information System, the incidence of BP for 2014 is 0.0009 per 100,000,6Philippine Dermatological Society. Health Information System Task Force Philippine Dermatological Society. Available from [email protected] . Retrieved April 9, 2015Google Scholar which is low compared with the 0.2 to 3 per 100,000 in developed countries such as the United Kingdom.7De Vega I.F. Iranzo-Fernández P. Mascaró-Galy J.M. Bullous pemphigoid: clinical practice guidelines.Actas Dermo-Sifiliográficas (English Edition). 2014; 105: 328-346Crossref Scopus (29) Google Scholar Precipitating factors include intake of drugs such as furosemide, phenacetin, enalapril, and ibuprofen; radiation therapy; ultraviolet A and B exposure; burns; surgery; and organ transplantation.8Schiavo A.L. Ruocco E. Brancaccio G. Caccavale S. Ruocco V. Wolf R. Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies.Clin Dermatol. 2013; 31: 391-399Abstract Full Text Full Text PDF PubMed Scopus (129) Google Scholar BP is typically characterized by mild to intractable pruritic macules, patches, and plaques (urticarial stage) eventually evolving into tense serous or hemorrhagic vesicles and bullae with excoriations and erosions (bullous stage) typically seen on flexural areas, lower abdomen, and lower extremities.1Schmidt E. Della Torre R. Borradori L. Clinical features and practical diagnosis of bullous pemphigoid.Immunol Allergy clin North Am. 2012; 32: 217-232Abstract Full Text Full Text PDF PubMed Scopus (35) Google Scholar Nikolsky and Asboe-Hansen signs are usually negative.9Urbano F.L. Nikolsky's sign in autoimmune skin disorders.Hospital Physician. 2001; 37: 23-24Google Scholar In some cases, BP presents clinically as atypical variants including dyshidrosiform and intertrigolike, prurigo nodularis–like, eczematous, localized, and erythrodermic forms.10Amato L. Gallerani I. Mei S. Pestelli E. Caproni M. Fabbri P. Erythrodermic bullous pemphigoid.Int J Dermatol. 2001; 40: 343Crossref PubMed Scopus (11) Google Scholar, 11Chan L.S. Dorman M.A. Agha A. Suzuki T. Cooper K.D. Hashimoto K. Pemphigoid vegetans represents a bullous pemphigoid variant. Patient's IgG autoantibodies identify the major bullous pemphigoid antigen.J Am Acad Dermatol. 1993; 28: 331Abstract Full Text PDF PubMed Scopus (27) Google Scholar, 12Kim J. Chavel S. Girardi M. McNiff J.M. Pemphigoid vegetans: A case report and review of the literature.J Cutan Pathol. 2008; 35: 1144Crossref PubMed Scopus (16) Google Scholar, 13Kim Y.J. Kim M.Y. Kim H.O. Park Y.M. Dyshidrosiform bullous pemphigoid.Acta Derm Venereol. 2004; 84: 253Crossref PubMed Scopus (12) Google Scholar, 14Korman N.J. Woods S.G. Erythrodermic bullous pemphigoid is a clinical variant of bullous pem- phigoid.Br J Dermatol. 1995; 133: 967Crossref PubMed Scopus (38) Google Scholar, 15Scrivener Y. Cribier B. Le Coz C. et al.Erythrodermic bullous pemphigoid.J Am Acad Dermatol. 1999; 41: 658Abstract Full Text PDF PubMed Scopus (4) Google Scholar, 16Sugimura C. Katsuura J. Moriue T. Matsuoka Y. Kubota Y. Dyshidrosiform pemphigoid: Report of a case.J Dermatol. 2003; 30: 525Crossref PubMed Scopus (19) Google Scholar, 17Tashiro H. Arai H. Hashimoto T. Takezaki S. Kawana S. Pemphigoid nodularis: Two case studies and analysis of autoantibodies before and after the development of generalized blistering.J Nippon Med Sch. 2005; 72: 60Crossref PubMed Scopus (12) Google Scholar, 18Yasuda M. Miyachi Y. Utani A. Two cases of dyshi- drosiform pemphigoid with di erent presenta- tions.Clin Exp Dermatol. 2009; 34: e151Crossref PubMed Scopus (10) Google Scholar In addition to these atypical presentations, BP may be seen on seborrheic areas (scalp, pre- and postauricular, auricular, chest) and is referred to as seborrheic pemphigoid, which has very few reported cases.19Errichetti E. Stinco G. Pegolo E. di Meo N. Trevisan G. Patrone P. Seborrheic pemphigoid.Case Rep Dermatol Med. 2014; 2014: 768217PubMed Google Scholar Here, we report a case of seborrheic pemphigoid in a 67-year-old Filipino man.Case reportWe present a case of a 67-year-old Filipino man who is hypertensive, diabetic, and dyslipidemic who is on the following maintenance medications: olmesartan plus hydrochlorothiazide, metformin, aspirin, and simvastatin for more than 5 years. He had no history of seborrheic dermatitis. He presented with a 4-week history of erythematous, severely pruritic, recurrent macules and patches on the cheeks and chest topped with clear fluid-filled vesicles and bullae spreading to the pre- and postauricular areas, mandibular areas, and scalp. A few scattered lesions also appeared on the arms and thighs. Persistence of the lesions prompted a visit to the outpatient department of a tertiary hospital in Makati City, Philippines. Physical examination found a few tense, clear fluid-filled vesicles and bullae on the right preauricular area, chest, and sole of the right foot (Fig 1). Several erythematous erosions were also noted on the scalp, pre- and post-auricular areas, malar areas, mandible, and chest, with a few on the arms and thighs (Fig 2). Nikolsky and Asboe-Hansen signs were negative. No other significant skin or mucosal lesions were appreciated.Fig 2Erythematous erosions on the (A) scalp, (B) malar areas and mandible, (C) pre- and postauricular areas, and (D and E) a few on the thighs.View Large Image Figure ViewerDownload (PPT)A 4-mm skin punch biopsy specimen from the edge of the blister on the chest was submitted for histologic examination (hematoxylin-eosin stain), which found a subepidermal blister with a superficial perivascular lymphohistiocytic and eosinophilic infiltrate (Fig 3). Direct immunofluorescence of perilesional skin from the chest showed linear deposits of IgG (+1) and C3 (+2) in the basement membrane zone (Fig 4). Serum enzyme-linked immunosorbent assay to BP180 was positive at 73.45 (cutoff value for positivity, 9 U/mL). Based on the clinical distribution and histologic and immunologic findings, seborrheic pemphigoid was diagnosed. Treatment with prednisone at a low dose of 0.2 mg/kg/d (gradually tapered), together with niacin, 1500 mg/d, and doxycycline, 200 mg/d, produced significant improvement with clearing of lesions and symptoms. After 7 weeks, the patient achieved complete remission and has stayed clear for 2 months with no maintenance medications.Fig 3Subepidermal blister (asterisk) with superficial perivascular lymphohistiocytic (yellow triangle) and eosinophilic infiltrate (blue triangle). (Hematoxylin-eosin stain; original magnification: ×20.)View Large Image Figure ViewerDownload (PPT)Fig 4IgG direct immunofluorescence of perilesional skin shows linear deposits of (A) IgG (+1) and (B) C3 (+2) in the basement membrane zone.View Large Image Figure ViewerDownload (PPT)DiscussionSeborrheic pemphigoid (SP) is a very rare variant of BP with only 4 cases reported in the literature. The first reported case was an elderly woman described by Schynder in 1969. SP is characterized by vesicles, bullae, and erosions involving the seborrheic areas of the body.19Errichetti E. Stinco G. Pegolo E. di Meo N. Trevisan G. Patrone P. Seborrheic pemphigoid.Case Rep Dermatol Med. 2014; 2014: 768217PubMed Google Scholar, 20Cozzani E. Gasparini G. Burlando M. Drago F. Parodi A. Atypical presentations of Bullous Pemphigoid: clinical and immunopathological aspects.Autoimmun Rev. 2015; 14: 438-445Crossref PubMed Scopus (60) Google Scholar Goldberg et al21Goldberg D.J. Sabolinski M. Bystryn J.C. Regional variation in the expression of bullous pemphigoid antigen and location of lesions in bullous pemphigoid.J Invest Dermatol. 1984; 82: 326-328Abstract Full Text PDF PubMed Scopus (46) Google Scholar found that in BP, expression of the BP antigens was variable, with greatest levels on sites of predilection and lowest levels on the scalp, face, and extensor arms. In SP, it is possible that expression of BP antigens is high at the seborrheic areas producing the typical vesicles and bullae. Other findings suggest that the presence of Malassezia and the excess sebum production on seborrheic areas may contribute to the development of SP via koebnerization, acting as a trigger for the disease. In addition, Malassezia is known to activate the classical or alternative complement pathways, possibly augmenting the complement-mediated inflammation seen in typical BP.19Errichetti E. Stinco G. Pegolo E. di Meo N. Trevisan G. Patrone P. Seborrheic pemphigoid.Case Rep Dermatol Med. 2014; 2014: 768217PubMed Google Scholar, 20Cozzani E. Gasparini G. Burlando M. Drago F. Parodi A. Atypical presentations of Bullous Pemphigoid: clinical and immunopathological aspects.Autoimmun Rev. 2015; 14: 438-445Crossref PubMed Scopus (60) Google Scholar Because of the rarity of this disease, there are no specific established treatment regimens. However, according to Errichetti et al,19Errichetti E. Stinco G. Pegolo E. di Meo N. Trevisan G. Patrone P. Seborrheic pemphigoid.Case Rep Dermatol Med. 2014; 2014: 768217PubMed Google Scholar treatment with low-dose systemic corticosteroids is effective in SP. Our patient started oral prednisone at 0.2 mg/kg/d with a subsequent taper. IntroductionBullous pemphigoid (BP) is the most common autoimmune subepidermal vesiculobullous disease usually affecting older patients.1Schmidt E. Della Torre R. Borradori L. Clinical features and practical diagnosis of bullous pemphigoid.Immunol Allergy clin North Am. 2012; 32: 217-232Abstract Full Text Full Text PDF PubMed Scopus (35) Google Scholar In BP, there is no ethnic or racial predilection and it affects both men and women equally.2Gudi V.S. White M.I. Cruickshank N. et al.Annual incidence and mortality of bullous pemphigoid in the Grampian Region of North-east Scotland.Br J Dermatol. 2005; 153: 424-427Crossref PubMed Scopus (126) Google Scholar, 3Bernard P. Enginger V. Venot J. Bedane C. Bonnetblanc J.M. Survival prognosis in pemphigoid. A cohort analysis of 78 patients.Ann Dermatol Venereol. 1995; 122: 751-757PubMed Google Scholar, 4Zillikens D. Wever S. Roth A. Weidenthaler-Barth B. Hashimoto T. Bröcker E.B. Incidence of autoimmune sub- epidermal blistering dermatoses in a region of central Germany.Arch Dermatol. 1995; 131: 957Crossref PubMed Scopus (211) Google Scholar, 5Bernard P. Vaillant L. Labeille B. et al.Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group.Arch Dermatol. 1995; 131: 48Crossref PubMed Scopus (19) Google Scholar Based on the reported data in the Philippine Dermatological Society Health Information System, the incidence of BP for 2014 is 0.0009 per 100,000,6Philippine Dermatological Society. Health Information System Task Force Philippine Dermatological Society. Available from [email protected] . Retrieved April 9, 2015Google Scholar which is low compared with the 0.2 to 3 per 100,000 in developed countries such as the United Kingdom.7De Vega I.F. Iranzo-Fernández P. Mascaró-Galy J.M. Bullous pemphigoid: clinical practice guidelines.Actas Dermo-Sifiliográficas (English Edition). 2014; 105: 328-346Crossref Scopus (29) Google Scholar Precipitating factors include intake of drugs such as furosemide, phenacetin, enalapril, and ibuprofen; radiation therapy; ultraviolet A and B exposure; burns; surgery; and organ transplantation.8Schiavo A.L. Ruocco E. Brancaccio G. Caccavale S. Ruocco V. Wolf R. Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies.Clin Dermatol. 2013; 31: 391-399Abstract Full Text Full Text PDF PubMed Scopus (129) Google Scholar BP is typically characterized by mild to intractable pruritic macules, patches, and plaques (urticarial stage) eventually evolving into tense serous or hemorrhagic vesicles and bullae with excoriations and erosions (bullous stage) typically seen on flexural areas, lower abdomen, and lower extremities.1Schmidt E. Della Torre R. Borradori L. Clinical features and practical diagnosis of bullous pemphigoid.Immunol Allergy clin North Am. 2012; 32: 217-232Abstract Full Text Full Text PDF PubMed Scopus (35) Google Scholar Nikolsky and Asboe-Hansen signs are usually negative.9Urbano F.L. Nikolsky's sign in autoimmune skin disorders.Hospital Physician. 2001; 37: 23-24Google Scholar In some cases, BP presents clinically as atypical variants including dyshidrosiform and intertrigolike, prurigo nodularis–like, eczematous, localized, and erythrodermic forms.10Amato L. Gallerani I. Mei S. Pestelli E. Caproni M. Fabbri P. Erythrodermic bullous pemphigoid.Int J Dermatol. 2001; 40: 343Crossref PubMed Scopus (11) Google Scholar, 11Chan L.S. Dorman M.A. Agha A. Suzuki T. Cooper K.D. Hashimoto K. Pemphigoid vegetans represents a bullous pemphigoid variant. Patient's IgG autoantibodies identify the major bullous pemphigoid antigen.J Am Acad Dermatol. 1993; 28: 331Abstract Full Text PDF PubMed Scopus (27) Google Scholar, 12Kim J. Chavel S. Girardi M. McNiff J.M. Pemphigoid vegetans: A case report and review of the literature.J Cutan Pathol. 2008; 35: 1144Crossref PubMed Scopus (16) Google Scholar, 13Kim Y.J. Kim M.Y. Kim H.O. Park Y.M. Dyshidrosiform bullous pemphigoid.Acta Derm Venereol. 2004; 84: 253Crossref PubMed Scopus (12) Google Scholar, 14Korman N.J. Woods S.G. Erythrodermic bullous pemphigoid is a clinical variant of bullous pem- phigoid.Br J Dermatol. 1995; 133: 967Crossref PubMed Scopus (38) Google Scholar, 15Scrivener Y. Cribier B. Le Coz C. et al.Erythrodermic bullous pemphigoid.J Am Acad Dermatol. 1999; 41: 658Abstract Full Text PDF PubMed Scopus (4) Google Scholar, 16Sugimura C. Katsuura J. Moriue T. Matsuoka Y. Kubota Y. Dyshidrosiform pemphigoid: Report of a case.J Dermatol. 2003; 30: 525Crossref PubMed Scopus (19) Google Scholar, 17Tashiro H. Arai H. Hashimoto T. Takezaki S. Kawana S. Pemphigoid nodularis: Two case studies and analysis of autoantibodies before and after the development of generalized blistering.J Nippon Med Sch. 2005; 72: 60Crossref PubMed Scopus (12) Google Scholar, 18Yasuda M. Miyachi Y. Utani A. Two cases of dyshi- drosiform pemphigoid with di erent presenta- tions.Clin Exp Dermatol. 2009; 34: e151Crossref PubMed Scopus (10) Google Scholar In addition to these atypical presentations, BP may be seen on seborrheic areas (scalp, pre- and postauricular, auricular, chest) and is referred to as seborrheic pemphigoid, which has very few reported cases.19Errichetti E. Stinco G. Pegolo E. di Meo N. Trevisan G. Patrone P. Seborrheic pemphigoid.Case Rep Dermatol Med. 2014; 2014: 768217PubMed Google Scholar Here, we report a case of seborrheic pemphigoid in a 67-year-old Filipino man. Bullous pemphigoid (BP) is the most common autoimmune subepidermal vesiculobullous disease usually affecting older patients.1Schmidt E. Della Torre R. Borradori L. Clinical features and practical diagnosis of bullous pemphigoid.Immunol Allergy clin North Am. 2012; 32: 217-232Abstract Full Text Full Text PDF PubMed Scopus (35) Google Scholar In BP, there is no ethnic or racial predilection and it affects both men and women equally.2Gudi V.S. White M.I. Cruickshank N. et al.Annual incidence and mortality of bullous pemphigoid in the Grampian Region of North-east Scotland.Br J Dermatol. 2005; 153: 424-427Crossref PubMed Scopus (126) Google Scholar, 3Bernard P. Enginger V. Venot J. Bedane C. Bonnetblanc J.M. Survival prognosis in pemphigoid. A cohort analysis of 78 patients.Ann Dermatol Venereol. 1995; 122: 751-757PubMed Google Scholar, 4Zillikens D. Wever S. Roth A. Weidenthaler-Barth B. Hashimoto T. Bröcker E.B. Incidence of autoimmune sub- epidermal blistering dermatoses in a region of central Germany.Arch Dermatol. 1995; 131: 957Crossref PubMed Scopus (211) Google Scholar, 5Bernard P. Vaillant L. Labeille B. et al.Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group.Arch Dermatol. 1995; 131: 48Crossref PubMed Scopus (19) Google Scholar Based on the reported data in the Philippine Dermatological Society Health Information System, the incidence of BP for 2014 is 0.0009 per 100,000,6Philippine Dermatological Society. Health Information System Task Force Philippine Dermatological Society. Available from [email protected] . Retrieved April 9, 2015Google Scholar which is low compared with the 0.2 to 3 per 100,000 in developed countries such as the United Kingdom.7De Vega I.F. Iranzo-Fernández P. Mascaró-Galy J.M. Bullous pemphigoid: clinical practice guidelines.Actas Dermo-Sifiliográficas (English Edition). 2014; 105: 328-346Crossref Scopus (29) Google Scholar Precipitating factors include intake of drugs such as furosemide, phenacetin, enalapril, and ibuprofen; radiation therapy; ultraviolet A and B exposure; burns; surgery; and organ transplantation.8Schiavo A.L. Ruocco E. Brancaccio G. Caccavale S. Ruocco V. Wolf R. Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies.Clin Dermatol. 2013; 31: 391-399Abstract Full Text Full Text PDF PubMed Scopus (129) Google Scholar BP is typically characterized by mild to intractable pruritic macules, patches, and plaques (urticarial stage) eventually evolving into tense serous or hemorrhagic vesicles and bullae with excoriations and erosions (bullous stage) typically seen on flexural areas, lower abdomen, and lower extremities.1Schmidt E. Della Torre R. Borradori L. Clinical features and practical diagnosis of bullous pemphigoid.Immunol Allergy clin North Am. 2012; 32: 217-232Abstract Full Text Full Text PDF PubMed Scopus (35) Google Scholar Nikolsky and Asboe-Hansen signs are usually negative.9Urbano F.L. Nikolsky's sign in autoimmune skin disorders.Hospital Physician. 2001; 37: 23-24Google Scholar In some cases, BP presents clinically as atypical variants including dyshidrosiform and intertrigolike, prurigo nodularis–like, eczematous, localized, and erythrodermic forms.10Amato L. Gallerani I. Mei S. Pestelli E. Caproni M. Fabbri P. Erythrodermic bullous pemphigoid.Int J Dermatol. 2001; 40: 343Crossref PubMed Scopus (11) Google Scholar, 11Chan L.S. Dorman M.A. Agha A. Suzuki T. Cooper K.D. Hashimoto K. Pemphigoid vegetans represents a bullous pemphigoid variant. Patient's IgG autoantibodies identify the major bullous pemphigoid antigen.J Am Acad Dermatol. 1993; 28: 331Abstract Full Text PDF PubMed Scopus (27) Google Scholar, 12Kim J. Chavel S. Girardi M. McNiff J.M. Pemphigoid vegetans: A case report and review of the literature.J Cutan Pathol. 2008; 35: 1144Crossref PubMed Scopus (16) Google Scholar, 13Kim Y.J. Kim M.Y. Kim H.O. Park Y.M. Dyshidrosiform bullous pemphigoid.Acta Derm Venereol. 2004; 84: 253Crossref PubMed Scopus (12) Google Scholar, 14Korman N.J. Woods S.G. Erythrodermic bullous pemphigoid is a clinical variant of bullous pem- phigoid.Br J Dermatol. 1995; 133: 967Crossref PubMed Scopus (38) Google Scholar, 15Scrivener Y. Cribier B. Le Coz C. et al.Erythrodermic bullous pemphigoid.J Am Acad Dermatol. 1999; 41: 658Abstract Full Text PDF PubMed Scopus (4) Google Scholar, 16Sugimura C. Katsuura J. Moriue T. Matsuoka Y. Kubota Y. Dyshidrosiform pemphigoid: Report of a case.J Dermatol. 2003; 30: 525Crossref PubMed Scopus (19) Google Scholar, 17Tashiro H. Arai H. Hashimoto T. Takezaki S. Kawana S. Pemphigoid nodularis: Two case studies and analysis of autoantibodies before and after the development of generalized blistering.J Nippon Med Sch. 2005; 72: 60Crossref PubMed Scopus (12) Google Scholar, 18Yasuda M. Miyachi Y. Utani A. Two cases of dyshi- drosiform pemphigoid with di erent presenta- tions.Clin Exp Dermatol. 2009; 34: e151Crossref PubMed Scopus (10) Google Scholar In addition to these atypical presentations, BP may be seen on seborrheic areas (scalp, pre- and postauricular, auricular, chest) and is referred to as seborrheic pemphigoid, which has very few reported cases.19Errichetti E. Stinco G. Pegolo E. di Meo N. Trevisan G. Patrone P. Seborrheic pemphigoid.Case Rep Dermatol Med. 2014; 2014: 768217PubMed Google Scholar Here, we report a case of seborrheic pemphigoid in a 67-year-old Filipino man. Case reportWe present a case of a 67-year-old Filipino man who is hypertensive, diabetic, and dyslipidemic who is on the following maintenance medications: olmesartan plus hydrochlorothiazide, metformin, aspirin, and simvastatin for more than 5 years. He had no history of seborrheic dermatitis. He presented with a 4-week history of erythematous, severely pruritic, recurrent macules and patches on the cheeks and chest topped with clear fluid-filled vesicles and bullae spreading to the pre- and postauricular areas, mandibular areas, and scalp. A few scattered lesions also appeared on the arms and thighs. Persistence of the lesions prompted a visit to the outpatient department of a tertiary hospital in Makati City, Philippines. Physical examination found a few tense, clear fluid-filled vesicles and bullae on the right preauricular area, chest, and sole of the right foot (Fig 1). Several erythematous erosions were also noted on the scalp, pre- and post-auricular areas, malar areas, mandible, and chest, with a few on the arms and thighs (Fig 2). Nikolsky and Asboe-Hansen signs were negative. No other significant skin or mucosal lesions were appreciated.A 4-mm skin punch biopsy specimen from the edge of the blister on the chest was submitted for histologic examination (hematoxylin-eosin stain), which found a subepidermal blister with a superficial perivascular lymphohistiocytic and eosinophilic infiltrate (Fig 3). Direct immunofluorescence of perilesional skin from the chest showed linear deposits of IgG (+1) and C3 (+2) in the basement membrane zone (Fig 4). Serum enzyme-linked immunosorbent assay to BP180 was positive at 73.45 (cutoff value for positivity, 9 U/mL). Based on the clinical distribution and histologic and immunologic findings, seborrheic pemphigoid was diagnosed. Treatment with prednisone at a low dose of 0.2 mg/kg/d (gradually tapered), together with niacin, 1500 mg/d, and doxycycline, 200 mg/d, produced significant improvement with clearing of lesions and symptoms. After 7 weeks, the patient achieved complete remission and has stayed clear for 2 months with no maintenance medications.Fig 3Subepidermal blister (asterisk) with superficial perivascular lymphohistiocytic (yellow triangle) and eosinophilic infiltrate (blue triangle). (Hematoxylin-eosin stain; original magnification: ×20.)View Large Image Figure ViewerDownload (PPT)Fig 4IgG direct immunofluorescence of perilesional skin shows linear deposits of (A) IgG (+1) and (B) C3 (+2) in the basement membrane zone.View Large Image Figure ViewerDownload (PPT) We present a case of a 67-year-old Filipino man who is hypertensive, diabetic, and dyslipidemic who is on the following maintenance medications: olmesartan plus hydrochlorothiazide, metformin, aspirin, and simvastatin for more than 5 years. He had no history of seborrheic dermatitis. He presented with a 4-week history of erythematous, severely pruritic, recurrent macules and patches on the cheeks and chest topped with clear fluid-filled vesicles and bullae spreading to the pre- and postauricular areas, mandibular areas, and scalp. A few scattered lesions also appeared on the arms and thighs. Persistence of the lesions prompted a visit to the outpatient department of a tertiary hospital in Makati City, Philippines. Physical examination found a few tense, clear fluid-filled vesicles and bullae on the right preauricular area, chest, and sole of the right foot (Fig 1). Several erythematous erosions were also noted on the scalp, pre- and post-auricular areas, malar areas, mandible, and chest, with a few on the arms and thighs (Fig 2). Nikolsky and Asboe-Hansen signs were negative. No other significant skin or mucosal lesions were appreciated. A 4-mm skin punch biopsy specimen from the edge of the blister on the chest was submitted for histologic examination (hematoxylin-eosin stain), which found a subepidermal blister with a superficial perivascular lymphohistiocytic and eosinophilic infiltrate (Fig 3). Direct immunofluorescence of perilesional skin from the chest showed linear deposits of IgG (+1) and C3 (+2) in the basement membrane zone (Fig 4). Serum enzyme-linked immunosorbent assay to BP180 was positive at 73.45 (cutoff value for positivity, 9 U/mL). Based on the clinical distribution and histologic and immunologic findings, seborrheic pemphigoid was diagnosed. Treatment with prednisone at a low dose of 0.2 mg/kg/d (gradually tapered), together with niacin, 1500 mg/d, and doxycycline, 200 mg/d, produced significant improvement with clearing of lesions and symptoms. After 7 weeks, the patient achieved complete remission and has stayed clear for 2 months with no maintenance medications. DiscussionSeborrheic pemphigoid (SP) is a very rare variant of BP with only 4 cases reported in the literature. The first reported case was an elderly woman described by Schynder in 1969. SP is characterized by vesicles, bullae, and erosions involving the seborrheic areas of the body.19Errichetti E. Stinco G. Pegolo E. di Meo N. Trevisan G. Patrone P. Seborrheic pemphigoid.Case Rep Dermatol Med. 2014; 2014: 768217PubMed Google Scholar, 20Cozzani E. Gasparini G. Burlando M. Drago F. Parodi A. Atypical presentations of Bullous Pemphigoid: clinical and immunopathological aspects.Autoimmun Rev. 2015; 14: 438-445Crossref PubMed Scopus (60) Google Scholar Goldberg et al21Goldberg D.J. Sabolinski M. Bystryn J.C. Regional variation in the expression of bullous pemphigoid antigen and location of lesions in bullous pemphigoid.J Invest Dermatol. 1984; 82: 326-328Abstract Full Text PDF PubMed Scopus (46) Google Scholar found that in BP, expression of the BP antigens was variable, with greatest levels on sites of predilection and lowest levels on the scalp, face, and extensor arms. In SP, it is possible that expression of BP antigens is high at the seborrheic areas producing the typical vesicles and bullae. Other findings suggest that the presence of Malassezia and the excess sebum production on seborrheic areas may contribute to the development of SP via koebnerization, acting as a trigger for the disease. In addition, Malassezia is known to activate the classical or alternative complement pathways, possibly augmenting the complement-mediated inflammation seen in typical BP.19Errichetti E. Stinco G. Pegolo E. di Meo N. Trevisan G. Patrone P. Seborrheic pemphigoid.Case Rep Dermatol Med. 2014; 2014: 768217PubMed Google Scholar, 20Cozzani E. Gasparini G. Burlando M. Drago F. Parodi A. Atypical presentations of Bullous Pemphigoid: clinical and immunopathological aspects.Autoimmun Rev. 2015; 14: 438-445Crossref PubMed Scopus (60) Google Scholar Because of the rarity of this disease, there are no specific established treatment regimens. However, according to Errichetti et al,19Errichetti E. Stinco G. Pegolo E. di Meo N. Trevisan G. Patrone P. Seborrheic pemphigoid.Case Rep Dermatol Med. 2014; 2014: 768217PubMed Google Scholar treatment with low-dose systemic corticosteroids is effective in SP. Our patient started oral prednisone at 0.2 mg/kg/d with a subsequent taper. Seborrheic pemphigoid (SP) is a very rare variant of BP with only 4 cases reported in the literature. The first reported case was an elderly woman described by Schynder in 1969. SP is characterized by vesicles, bullae, and erosions involving the seborrheic areas of the body.19Errichetti E. Stinco G. Pegolo E. di Meo N. Trevisan G. Patrone P. Seborrheic pemphigoid.Case Rep Dermatol Med. 2014; 2014: 768217PubMed Google Scholar, 20Cozzani E. Gasparini G. Burlando M. Drago F. Parodi A. Atypical presentations of Bullous Pemphigoid: clinical and immunopathological aspects.Autoimmun Rev. 2015; 14: 438-445Crossref PubMed Scopus (60) Google Scholar Goldberg et al21Goldberg D.J. Sabolinski M. Bystryn J.C. Regional variation in the expression of bullous pemphigoid antigen and location of lesions in bullous pemphigoid.J Invest Dermatol. 1984; 82: 326-328Abstract Full Text PDF PubMed Scopus (46) Google Scholar found that in BP, expression of the BP antigens was variable, with greatest levels on sites of predilection and lowest levels on the scalp, face, and extensor arms. In SP, it is possible that expression of BP antigens is high at the seborrheic areas producing the typical vesicles and bullae. Other findings suggest that the presence of Malassezia and the excess sebum production on seborrheic areas may contribute to the development of SP via koebnerization, acting as a trigger for the disease. In addition, Malassezia is known to activate the classical or alternative complement pathways, possibly augmenting the complement-mediated inflammation seen in typical BP.19Errichetti E. Stinco G. Pegolo E. di Meo N. Trevisan G. Patrone P. Seborrheic pemphigoid.Case Rep Dermatol Med. 2014; 2014: 768217PubMed Google Scholar, 20Cozzani E. Gasparini G. Burlando M. Drago F. Parodi A. Atypical presentations of Bullous Pemphigoid: clinical and immunopathological aspects.Autoimmun Rev. 2015; 14: 438-445Crossref PubMed Scopus (60) Google Scholar Because of the rarity of this disease, there are no specific established treatment regimens. However, according to Errichetti et al,19Errichetti E. Stinco G. Pegolo E. di Meo N. Trevisan G. Patrone P. Seborrheic pemphigoid.Case Rep Dermatol Med. 2014; 2014: 768217PubMed Google Scholar treatment with low-dose systemic corticosteroids is effective in SP. Our patient started oral prednisone at 0.2 mg/kg/d with a subsequent taper." @default.
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