930859
4
8/19/2019 930859 http://slidepdf.com/reader/full/930859 1/4 Case Report Unilateral Oral Mucous Membrane Pemphigoid: Refractory Atypical Presentation Successfully Treated with Intravenous Immunoglobulins André Laureano and Jorge Cardoso Department of Dermatology and Venereology, Hospital de Curry Cabral, Centro Hospitalar de Lisboa Central, - Lisboa, Portugal Correspondence should be addressed to Andr´ e Laureano; [email protected] Received November ; Accepted February Academic Editor: G´ erald E. Pi´ erard Copyright © A. Laureano and J. Cardoso. Tis is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. A -year-old male presented with a -month history o blisters and painul erosions on the right buccal mucosa. No skin or other mucosal involvement was seen. Te ndings o histopathological and direct immunouorescence examinations were sufficient or the diagnosis o oral mucous membrane pemphigoid in the context o adequate clinical correlation. No response was seen afer topical therapies and oral corticosteroids or dapsone. Intravenous immunoglobulin was started and repeated every three weeks. Complete remission was achieved afer three cycles and no recurrence was seen afer two years o ollow-up. Te authors report a rare unilateral presentation o oral mucous membrane pemphigoid on the right buccal and hard palate mucosa, without additional involvement during a period o ve years. Local trauma or autoimmune actors are possible etiologic actors or this rare disorder, here with unique presentation. 1. Introduction Mucous membrane pemphigoid (MMP) describes a hetero- geneous group o chronic autoimmune subepithelial blister- ing diseases, primarily affecting mucous membranes, with or withoutskininvolvement[].Althoughscarringistheclinical hallmark, it may not be obvious in the oral mucosa, which is the most commonly affected site. Lesions typically consist o desquamative gingivitis, erythematous patches, blisters, and erosions covered by pseudomembranes []. Autoantibodies binding to the epithelial basement membrane zone (BMZ) have been demonstrated in this subset, targeting bullous antigens and , laminin and laminin , type VII collagen, -integrin subunit, and some nonidentied basal membrane zone antigens [, ]. Any oral cavity location can be involved and patients usually have a good prognosis. 2. Case Presentation A -year-old male presented with a -month history o blisters and painul erosions on the right buccal mucosa. His medical history was relevant or hypertension and hypothy- roidism. He had been taking valsartan and levothyroxine or years and denied the use o topical drugs and previous dental procedures. On physical examination, the patient was ound to have ew bullae, erosions, and pseudomembrane-covered erosions on the right buccal mucosa ( Figure ). No skin or other mucosal involvement was seen. He had ragmented teeth with sharp edges adjacent to the lesions. Laboratory evaluation was unremarkable. Histopathological examination o bullous lesion revealed a subepithelial blister with a mostly lymphocytic inltrate in the upper corion (Figure ). Direct immunouorescence o peribullous mucosa showed a linear band o IgG, IgA, and complement compo- nent (C) at the epithelial BMZ (Figure ). ELISA was negative or antibodies against bullous pem- phigoid antigens and and desmogleins and . Correlation between these eatures allowed the diagnosis o MMP. Application o dipropionate betamethasone cream, twice daily, was started. Afer one year the patient had Hindawi Publishing Corporation Case Reports in Dermatological Medicine Volume 2015, Article ID 930859, 3 pages http://dx.doi.org/10.1155/2015/930859
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8192019 930859
httpslidepdfcomreaderfull930859 14
Case Report Unilateral Oral Mucous Membrane PemphigoidRefractory Atypical Presentation Successfully Treated
with Intravenous Immunoglobulins
Andreacute Laureano and Jorge Cardoso
Department of Dermatology and Venereology Hospital de Curry Cabral Centro Hospitalar de Lisboa Central983089983088983094983097-983089983094983094 Lisboa Portugal
Correspondence should be addressed to Andre Laureano andreoliveirasapopt
Received 983090983093 November 983090983088983089983092 Accepted 983090 February 983090983088983089983093
Academic Editor Gerald E Pierard
Copyright copy 983090983088983089983093 A Laureano and J Cardoso Tis is an open access article distributed under the Creative Commons AttributionLicense which permits unrestricted use distribution and reproduction in any medium provided the original work is properly cited
A 983093983095-year-old male presented with a 983094-month history o blisters and painul erosions on the right buccal mucosa No skin or othermucosal involvement was seen Te 1047297ndings o histopathological and direct immuno1047298uorescence examinations were sufficient orthe diagnosis o oral mucous membrane pemphigoid in the context o adequate clinical correlation No response was seen afertopical therapies and oral corticosteroids or dapsone Intravenous immunoglobulin was started and repeated every three weeks
Complete remission was achieved afer three cycles and no recurrence was seen afer two years o ollow-up Te authors report arare unilateral presentation o oral mucous membrane pemphigoid on the right buccal and hard palate mucosa without additionalinvolvement during a period o 1047297ve years Local trauma or autoimmune actors are possible etiologic actors or this rare disorderhere with unique presentation
1 Introduction
Mucous membrane pemphigoid (MMP) describes a hetero-geneous group o chronic autoimmune subepithelial blister-ing diseases primarily affecting mucous membranes with orwithout skin involvement [983089] Although scarring is theclinicalhallmark it may not be obvious in the oral mucosa which isthe most commonly affected site Lesions typically consist o
desquamative gingivitis erythematous patches blisters anderosions covered by pseudomembranes [983090] Autoantibodiesbinding to the epithelial basement membrane zone (BMZ)have been demonstrated in this subset targeting bullousantigens 983089 and 983090 laminin 983091983091983090 and laminin 983091983089983089 type VIIcollagen 983092-integrin subunit and some nonidenti1047297ed basalmembrane zone antigens [983091 983092] Any oral cavity location canbe involved and patients usually have a good prognosis
2 Case Presentation
A 983093983095-year-old male presented with a 983094-month history o blisters and painul erosions on the right buccal mucosa His
medical history was relevant or hypertension and hypothy-roidism He had been taking valsartan and levothyroxine oryears and denied the use o topical drugs and previous dentalprocedures On physical examination the patient was oundto have ew bullae erosions and pseudomembrane-coverederosions on the right buccal mucosa (Figure 983089)
No skin or other mucosal involvement was seen He had
ragmented teeth with sharp edges adjacent to the lesionsLaboratory evaluation was unremarkable Histopathologicalexamination o bullous lesion revealed a subepithelial blisterwith a mostly lymphocytic in1047297ltrate in the upper corion(Figure 983090)
Direct immuno1047298uorescence o peribullous mucosashowed a linear band o IgG IgA and complement compo-nent 983091 (C983091) at the epithelial BMZ (Figure 983091)
ELISA was negative or antibodies against bullous pem-phigoid antigens 983089983096983088 and 983090983091983088 and desmogleins 983089 and 983091Correlation between these eatures allowed the diagnosis o MMP Application o dipropionate betamethasone creamtwice daily was started Afer one year the patient had
Hindawi Publishing CorporationCase Reports in Dermatological MedicineVolume 2015 Article ID 930859 3 pageshttpdxdoiorg1011552015930859
8192019 930859
httpslidepdfcomreaderfull930859 24
983090 Case Reports in Dermatological Medicine
F983145983143983157983154983141 983089 At presentation multiple painul erosions andpseudomembrane-covered erosions on the right buccal mucosawere seen
F983145983143983157983154983141 983090 Histopathological examination o a bullous lesion
revealed a subepithelial blister with a mostly lymphocytic andneutrophilic dense in1047298ammatory in1047297ltrate in the upper corion(hematoxylin and eosin original magni1047297cationtimes983089983088983088)
persistent bullae anderosionson the right buccal mucosa thathealedwithout scarring Oral prednisolone (983088983093 mgkgd) wasstarted or six months and as no response was achievedtreatment with dapsone (983089983088983088 mgd) was administered duringoneyear Further involvement o the right hard palate mucosaoccurred erosions were extremely painul and the patienthad difficulty in eating and depression (Figure 983092)
Intravenous immunoglobulin (IVIg) at a dose o
983090 gkgcycle was started and repeated every three weeksComplete remission was achieved afer three cycles IVIgtherapy was maintained or six additional months Norecurrence was seen afer three years o ollow-up (Figure 983093)
3 Discussion
Te 1047297ndings o direct immuno1047298uorescence were sufficientor the diagnosis o MMP in the context o adequate clinicalcorrelation [983089] Patients with MMP with oral involvementofen exhibit bilateral lesions We reported a unilateralpresentation on the right buccal and hard palate mucosawithout additional involvement during a period o 1047297ve
F983145983143983157983154983141 983091 Direct immuno1047298uorescence showed a linear band o IgGIgA and C983091 at the epithelial BMZ (original magni1047297cation times983092983088)
F983145983143983157983154983141 983092 No response afer topical and systemic treatment withcorticosteroids and dapsone with urther involvement o the righthard palate mucosa
F983145983143983157983154983141 983093 Complete response afer IVIg therapy and only a delicatewhite pattern o reticulated scarring on the buccal mucosa had beenseen afer 983091 years o ollow-up
years A possible previous chronic in1047298ammatory process o the mucosa associated with local trauma probably exposedhidden antigens o the BMZ and evoked a secondary autoim-mune response explaining this mosaic o disease [983090] Directimmuno1047298uorescence 1047297ndings and the complete responseafer IVIg also suggest an autoimmune etiology here with
8192019 930859
httpslidepdfcomreaderfull930859 34
Case Reports in Dermatological Medicine 983091
unique presentation [983091 983093] Since management o MMP isofen difficult our case also shows a complete response to atherapeutic option not commonly used in the limited or lesssevere disease
Conflict of Interests
Te authors declare that there is no con1047298ict o interestsregarding the publication o this paper
References
[983089] L S Chan A Razzaque Ahmed G J Anhalt et al ldquoTe 1047297rstinternational consensus on mucous membrane pemphigoidde1047297nition diagnostic criteria pathogenic actors medical treat-ment and prognostic indicatorsrdquo Archives of Dermatology vol983089983091983096 no 983091 pp 983091983095983088ndash983091983095983097 983090983088983088983090
[983090] L S Chan ldquoOcular and oral mucous membrane pemphigoid(cicatricial pemphigoid)rdquo Clinics in Dermatology vol 983091983088 no 983089pp 983091983092ndash983091983095 983090983088983089983090
[983091] A S Kourosh and K B Yancey ldquoPathogenesis o mucousmembrane pemphigoidrdquo Dermatologic Clinics vol 983090983097 no 983091 pp983092983095983097ndash983092983096983092 983090983088983089983089
[983092] K A Rashid H M Gurcan and A R Ahmed ldquoAntigen speci-1047297city in subsets o mucous membrane pemphigoidrdquo Journal of Investigative Dermatology vol 983089983090983094 no 983089983090 pp 983090983094983091983089ndash983090983094983091983094 983090983088983088983094
[983093] D A Culton and L A Diaz ldquoreatment o subepidermalimmunobullous diseasesrdquo Clinics in Dermatology vol 983091983088 no983089 pp 983097983093ndash983089983088983090 983090983088983089983090
8192019 930859
httpslidepdfcomreaderfull930859 44
Submit your manuscripts at
httpwwwhindawicom
8192019 930859
httpslidepdfcomreaderfull930859 24
983090 Case Reports in Dermatological Medicine
F983145983143983157983154983141 983089 At presentation multiple painul erosions andpseudomembrane-covered erosions on the right buccal mucosawere seen
F983145983143983157983154983141 983090 Histopathological examination o a bullous lesion
revealed a subepithelial blister with a mostly lymphocytic andneutrophilic dense in1047298ammatory in1047297ltrate in the upper corion(hematoxylin and eosin original magni1047297cationtimes983089983088983088)
persistent bullae anderosionson the right buccal mucosa thathealedwithout scarring Oral prednisolone (983088983093 mgkgd) wasstarted or six months and as no response was achievedtreatment with dapsone (983089983088983088 mgd) was administered duringoneyear Further involvement o the right hard palate mucosaoccurred erosions were extremely painul and the patienthad difficulty in eating and depression (Figure 983092)
Intravenous immunoglobulin (IVIg) at a dose o
983090 gkgcycle was started and repeated every three weeksComplete remission was achieved afer three cycles IVIgtherapy was maintained or six additional months Norecurrence was seen afer three years o ollow-up (Figure 983093)
3 Discussion
Te 1047297ndings o direct immuno1047298uorescence were sufficientor the diagnosis o MMP in the context o adequate clinicalcorrelation [983089] Patients with MMP with oral involvementofen exhibit bilateral lesions We reported a unilateralpresentation on the right buccal and hard palate mucosawithout additional involvement during a period o 1047297ve
F983145983143983157983154983141 983091 Direct immuno1047298uorescence showed a linear band o IgGIgA and C983091 at the epithelial BMZ (original magni1047297cation times983092983088)
F983145983143983157983154983141 983092 No response afer topical and systemic treatment withcorticosteroids and dapsone with urther involvement o the righthard palate mucosa
F983145983143983157983154983141 983093 Complete response afer IVIg therapy and only a delicatewhite pattern o reticulated scarring on the buccal mucosa had beenseen afer 983091 years o ollow-up
years A possible previous chronic in1047298ammatory process o the mucosa associated with local trauma probably exposedhidden antigens o the BMZ and evoked a secondary autoim-mune response explaining this mosaic o disease [983090] Directimmuno1047298uorescence 1047297ndings and the complete responseafer IVIg also suggest an autoimmune etiology here with
8192019 930859
httpslidepdfcomreaderfull930859 34
Case Reports in Dermatological Medicine 983091
unique presentation [983091 983093] Since management o MMP isofen difficult our case also shows a complete response to atherapeutic option not commonly used in the limited or lesssevere disease
Conflict of Interests
Te authors declare that there is no con1047298ict o interestsregarding the publication o this paper
References
[983089] L S Chan A Razzaque Ahmed G J Anhalt et al ldquoTe 1047297rstinternational consensus on mucous membrane pemphigoidde1047297nition diagnostic criteria pathogenic actors medical treat-ment and prognostic indicatorsrdquo Archives of Dermatology vol983089983091983096 no 983091 pp 983091983095983088ndash983091983095983097 983090983088983088983090
[983090] L S Chan ldquoOcular and oral mucous membrane pemphigoid(cicatricial pemphigoid)rdquo Clinics in Dermatology vol 983091983088 no 983089pp 983091983092ndash983091983095 983090983088983089983090
[983091] A S Kourosh and K B Yancey ldquoPathogenesis o mucousmembrane pemphigoidrdquo Dermatologic Clinics vol 983090983097 no 983091 pp983092983095983097ndash983092983096983092 983090983088983089983089
[983092] K A Rashid H M Gurcan and A R Ahmed ldquoAntigen speci-1047297city in subsets o mucous membrane pemphigoidrdquo Journal of Investigative Dermatology vol 983089983090983094 no 983089983090 pp 983090983094983091983089ndash983090983094983091983094 983090983088983088983094
[983093] D A Culton and L A Diaz ldquoreatment o subepidermalimmunobullous diseasesrdquo Clinics in Dermatology vol 983091983088 no983089 pp 983097983093ndash983089983088983090 983090983088983089983090
8192019 930859
httpslidepdfcomreaderfull930859 44
Submit your manuscripts at
httpwwwhindawicom
8192019 930859
httpslidepdfcomreaderfull930859 34
Case Reports in Dermatological Medicine 983091
unique presentation [983091 983093] Since management o MMP isofen difficult our case also shows a complete response to atherapeutic option not commonly used in the limited or lesssevere disease
Conflict of Interests
Te authors declare that there is no con1047298ict o interestsregarding the publication o this paper
References
[983089] L S Chan A Razzaque Ahmed G J Anhalt et al ldquoTe 1047297rstinternational consensus on mucous membrane pemphigoidde1047297nition diagnostic criteria pathogenic actors medical treat-ment and prognostic indicatorsrdquo Archives of Dermatology vol983089983091983096 no 983091 pp 983091983095983088ndash983091983095983097 983090983088983088983090
[983090] L S Chan ldquoOcular and oral mucous membrane pemphigoid(cicatricial pemphigoid)rdquo Clinics in Dermatology vol 983091983088 no 983089pp 983091983092ndash983091983095 983090983088983089983090
[983091] A S Kourosh and K B Yancey ldquoPathogenesis o mucousmembrane pemphigoidrdquo Dermatologic Clinics vol 983090983097 no 983091 pp983092983095983097ndash983092983096983092 983090983088983089983089
[983092] K A Rashid H M Gurcan and A R Ahmed ldquoAntigen speci-1047297city in subsets o mucous membrane pemphigoidrdquo Journal of Investigative Dermatology vol 983089983090983094 no 983089983090 pp 983090983094983091983089ndash983090983094983091983094 983090983088983088983094
[983093] D A Culton and L A Diaz ldquoreatment o subepidermalimmunobullous diseasesrdquo Clinics in Dermatology vol 983091983088 no983089 pp 983097983093ndash983089983088983090 983090983088983089983090
8192019 930859
httpslidepdfcomreaderfull930859 44
Submit your manuscripts at
httpwwwhindawicom
8192019 930859
httpslidepdfcomreaderfull930859 44
Submit your manuscripts at
httpwwwhindawicom
