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`esophageal or gastrolntestinal symptoms.
`impsxance of co siderisng pemphigus in the differential when patients present with
`ounce testing reveals tplal findings of ptssphigus svtgris. This case highlights the
`including mo on:a48mited pemphigus colgaris. Direct and indirect immuooiluores
`clisatno suptftcialb lias a smog niatio»Idp to mmmnuhtllous diseases.
`Although rare. a madder rnhsscopic Nikolsky sign of the rsnphagus or dsophagitis
`restating in odyophagia, dysphagia. or nonspecific amrointestinal symptoms.
`ussko: Moc sal involvement In pemphigus svtgaris may involve the esophagus
`Discussion:
`these findi npps were consistent with a sliagsosls Of nmcOhilainded pmplugs stuiga c
`iscrescd Igc: dkansogkin 3 arttdwdy ksd, anda normal It <iskarsxsgkin 1 atxüsscly ksd.
`Indirect inmuatoll osacsncr revealed psishw IgG cell surface aotdoxly staining. an
`drnotearned prominent 44 coil surface antlxM and c3 artI surface reactivity.
`titto:at. suprfcialé. Mussy of the esophagi. Mr direct kmnwoihxxoxance
`imminent presaute -afinding LAsn:usem with a positiv NikoNq sign so eso(dugkis
`enocisuss along the esop isag s. Epithelial shearing also was notai from minor radoscopy
`The paient was refend fer uppergearointestinal end scvpy; which ,taled bulb and
`15gJal etasswoatkxs mauled two vest oat rnxsiass with to avlattnxn hswdseno:ttt.
`symptoms. including early satiety. bloating. satasea hoarsen., du:0u04a.2nd heardxsm.
`Caw npsrt A Sil)tarokl hunk was evaluated for a castdbthm of gastmdmcsthul
`tinal sympoons in the absence ouf eat:mamas disease.
`mucs»tl pempdsigs svytais in a patient who presented with multiple gaatrointrs-
`rotgrtnxnn-rokcgisis in odder In improve clinical osas-sonsds We report a ease of
`aspkirnt for esophageal invohenaest in tlsrsc patients is required by dermatologists
`peek ulcer d(trase ami /or increase Needing risk. Therefore, a high mskx of
`patients also are commonly mated with oral stemsids, which can further cxacrrhate
`significant morbidity including gastrointestinal herding or painPemphigus
`common. esophageal involvement is reported in only 5% of patients anti may cause
`mokcuks induced hinter ksmatkn. While mama' disease in pemphigusis
`and mucous membranes. The binding of aotoantihodlcs to kreatinoc)tc adhesion
`dnracterized by aanthoaysis and intraepithelial blister Donation involving the skin
`Introduction: Pemphigus vultfaris is a rare, imnnue-mtdlatd blistering disorder
`lake City, UT. United States
`Kristin M. Leifetman, MD, university of Utah Department of Dematniogy, Salt
`Gast entcmdogy. Hepatology and Nutrition. Salt Lake City, tfl: United States:
`Caked States: Kathryn A. Peterson. MD. University of Utah Division of
`Boynton, MD, University of Chah Division of Gastroenterology. Salt fake Chit UT,
`Utah Department of Dermatology. Salt lake City,lff.United States: 'Cathleen K.
`Dermatology Sak Lake City.lfiUnited States: Jennie T Clarke. MD. University of
`ND. United States: Jason fl Ilawka, MD. University of Utah Department of
`Taylor Braunbergce University of North Dakota School of Medicine. Grand Fork,
`vulgaris and multiple gastrofntcstlnal symptoms: A case report
`F_sophagluLs dissecans superficialkt in a patient with mucosal pemphigus
`3511
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`Commercial support.None fafrn))fiovL
`
`tors of much cites is usually prolonged.
`modulatory adjuvants, timximah) show accordingly slow response and hs»piulira
`disease. therapeutic procedures (systemic storokis, immunoapheresu immune.
`and histology lmmunothinresccncc and mokctdar studies are essential to define the
`maphyiococcal scalded skin syndromelodifficult. hascd only tin clinical picture
`abut 6% of PF cases. Differential diagnosis in these generalized cases from
`sokagein). Ptsgression to cryihroskmu is unusual. It has been estimated to occur in
`cases desmogkin1. It has two leas. the sporadic and the endemic one (folio
`fdixrus is an autoimmune butbus disease. with the target antigen bring in most
`health authorities n anticipated, in order to Cotime with dtuximah. Prtnphigus
`the raractory course of PE the treatment plan is not completed yet, as an appn »al of
`week of hospitalization and under systemic prednbonc and asathkoprine. Lime to
`sore we, coxiddnol. Patient was ocicascd with signifiant emprovennost after 7
`multidisciplinary collaboration. Plasmapdseresis and pulses of intravenous prednis-
`tatablished with all diagnostic procedure:. Treatment decisions were nude after a
`weight M the causative antigen. Diagnosis of pemphigts ádlacays (PF) was
`titers. Western bpd analysis in extract of human epidermis confirmed the molecular
`C3. Ant4USG -1 antibodies with ELISA techniques were detected in extremely high
`'Brea and indirect emmtnoßaorescence slowed interceltdar deposition of Igd and
`histology revealed aantholysis just uak:maath the granular layer of the epidermis.
`the time period between the Out and the second hospitalization. At that point.
`loamy with antibiotics and low doses of systemic pradnisone and cyclospor(nr M
`without the achievement of full remission ofcrythmskmu. The patient had a king
`syndrome and treated with systemic intravenous antibiotics She was released
`hospitalized. 5 months ago. with a diagnosis of staphylococcal scalded skin
`trunk. about 9 months ago. which progressively stud to erythnxkmu. Saw was
`ectropion. She had a history of arytlxtnatous papules and mkropunules on the
`A S3)earadd woman prescnted wish crythrodaroa. generatiaed exfoliation and
`of Dermatology, Aristotle Unhrrsity School of Medicine. Thessaloniki. Greece
`Medicine. Thessaloniki. Greece, Dimitrios Sotiriadis, MD. PhD, 2nd Department
`of Molecular Bioktgy, 1st Department of Obstetrics, Aristotle University School of
`Medlin. T a sabuiki, Greece; Alcxanhos Lantbmpuulus, MD. PhD, laboratory
`of Molecular Biology. 1st Department of Obstetrics. Aristotle University School of
`School of Medicine. Thessaloniki, Greece, Partltena Mrltzanidou, JD. Laboratory
`Ballot's Diseases Clinic, 2nd Department of Dermatology, Aristotle University
`University School of Medicine. Thessaloniki. Grace: Miltladh Kokdlos. MD.
`Stella Dasma, MD. PhD, 3rd Department of Internal Medicine. Arisuttk
`Dermatology, Aristotle University School of Midkine. Thessaloniki. Greece
`Atkarerini Patsatsl. MD. PhD. nullous Dimas. Clink. 2nd Department of
`Erythroderntk petnphigta foliaceuss A rare refractory variant
`3404
`
`clinicians to manage this consplex and extensive disorder
`organ spurns. An awareness of the potential clinical manifestations of Eli will help
`the future. It is highly rare to have EBS with such extensive involvement of multiple
`elevated eels of atatinine kinase may suggest posslsk muscular d shtnsction in
`our paten Pus had no clinical sigh of muscular dystrophy by 5 years of age.
`respiratory tact requiring tradsrsraomy- as well as esophageal involvement. While
`urobgk involvement.
`ement. Otr case also dennosttxod extensive involvement of the
`(to with two novel pectin mutations resulting in pyloric atresb and significant
`with muscular dystrophy (EILMO). We present a unique case of henidasmoso nap
`associated with subtypes of F.B. including EB with pyloric atresia (En -PA) and Iß
`the gene omitting pteclin, a protein imvhtd in hemi esrmsane functioning, arc
`maxims in proteins of the skin and MUCOUS membranes of the body. Mutations in
`auso epidermal fragility, blistering, and erosions. Eli results from a variety of
`Epidemtotysis bulkxat (EB) it COMprised of a variety of rare. inherited diseases that
`Slates
`United Sales: aloLse Levy: MD, Dell Children s Medical Center. Austin. TX. United
`Motion Woody. MPH. Texas A&M Health Science Center, Round Rock. TX.
`Gregory Walker. MD, MBA, Baylor Scott and White, Temple, TX, united States;
`involvement
`Epiclernolysb bullosa with pyloric atresia and significant urologic
`3536
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`Commercial support None Identified
`
`these rare, amt scare blistering diwsrskrs.
`arc needed to pdwidc additional information about the robs of dietary triggers in
`mentioned. Future pm pecthrstudiesincluding Mortel and or patch alktgen hothtg
`Limitations in our study included popubtion bias and recall Mas as previously
`Conclusion, Our study points to importance of possiok dietary triFgats in PV or BP.
`of smoking and using tobacco.
`peinnriiy because of rocalt bin, timeline associated with smoking as well as nodes
`nations (P - .4513). Smoking information was limited and dfikuh to analyze
`iant difference in willingness to change dietary Iifestk was seen between PV or ItP
`po»tdbgnosis when compared to prediagnss (P = .0336). No sutigially *Wit
`We also saw significantly decreased intake of 00)1 cvmpounets in PV patients
`signifeaml more frequently compared to BP patients prior to diagnosis (P =.0002).
`Rassdts, fused on the salary population. PV patients ate diet containing tannic activa
`significance was considered x P <.05.
`diagnosis with biopsy. Statistical anal sis was carried out using Vassar Sots.
`Nursing (done patients. and patients with dementia or with no confirmatory
`qualified and 20 from which risponskd. Exclusion criteria indudcd other diagnosis.
`pemphigus svlgarn ad/or 'bWkws pemphigt»d were identified of which 61
`k>_uhutkn. 241 patients with the search terms `pxttpdoiguo -prmpldguit'
`of PV and BP patients arc the same. Using the patient registration software at our
`Patients with PO and BP do not charme their dirt after diagnosis. (3) Smoking habits
`h)pothc bed - (I) No dietary difference exists between PV and BP patients. (2)
`difference and smoking patterns prior and post diagnosis of PV or RP. We
`Methods: We performed a rsrmspcthc survey study to anal)oo the dietary
`areas.
`in vitro modes. Nicotine on the other hand has Mon known to hase anticantholytk
`aasociatio of Chet containing alll compounds and tannic acid with acantholysis in
`cvod hr a porttbl eminnmcntal trigger. Previous studies have shown pasibie
`Sacral genetic and environmental etiologic factors hare been postdated. Food
`anoimmunr skin blistering disorders that can invoke the mucous membranes.
`Background, Pemphigus vulgaris (PV) and hulkxu ietnpdsigoid (LIP) are rare
`Yadiu Murky. MD. Saint Louts University. Saint Louis. MO. United States
`Maulik Dhandha. SID, Saint Louis University. Saint Louis. MO, United States.
`bullion pcmphigoid
`Dietary and smoking habits in patients with pemphigus colgarlo and
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`Commercial support: Norte kfetNfkvL
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`inflammatory discuses, and Ili(i deposits were associated to positive :(N(A.
`inflammatory diseases; IgM deposition related to the presence of autoimmune or
`related to renal involvement; IgA sitposils rebted to absence of autoimmuno or
`conditions related to LCV. c3 drpostts, the nest frequent finding of this study,
`Crnplcx at the blood vessel wall by DF is reliant in determine underlying
`the prognosis and wdkdying etiology of LCV. Characterization of tits immune
`histology -proven cutaneous 1(A! INF seems to Ise an important method to establish
`Conclusions: To our knowledge, this is she bigots DIF analysis of patients with
`involvement. Systemic imoher ent was present in 12.5% cases of LCV patients.
`C3 deposition at who bkxd vend wall :mandated to hcmaturia and mat
`deposition at the blend vessel wall was associated to age and positivesAAA: ßtnll.
`tion. antinuclear antbody and anti 55A/55ß positivity. Immunoglols din G (ltG)
`related to fctnacs and autoinrssunc/Mflamttmory disortku, C3 and C-1 consump-
`flamnutory diseases. Immunogbhuun M O&M) deposition at the blood vessel wall
`deposition at the Neel and wall mimed to age and absence of amsdmmune/in-
`samples. and C3 was the most frequent immsmortactant. Insmuogkttwhn A OM)
`(6'.'3%) wear female imdvkhals DIF analysis shaved p trdsisity in 70.21% of the
`Results: Ages ranged from five to tc7 years Old (yo). median age of 45 and 191/282
`Schad, from January 2007 to Ieccmtx.r 2014.
`DIE diagnosed at the Department of Dermatology. 'Job rosily of Sao Paulo Medical
`3ktiods: Wo performed a retrospective study of 282 biopsy -pmvrn LCV casts with
`tertiary hospital.
`explore de shred hsmunolamsC.ncc (DIE) findings in patients with LCV from a
`pugapillary yenulrs. leading to an inflammatory noway T ar:fore. we aimed to
`anew. its pathophysiobgy relates to deposition of immune compkxr: at the wadi of
`small vcssds, which IS often idiopathic, but may he secondary to an underlying
`Introduction: Lcukocytoc)astk sasculitis (LCV) is an inflammatory sascubpathy M
`Paulo. Brazil
`Hospital tits Chnkas dot Pacstktadc do Medicina dot Unhersidase de Sio Paulo, Sou
`Unhriskladc do Sao Paula. Sao Paulo. ISrazel: Paulo Ricardo COado. MD. PhD,
`Paris Sandier. MI). Hospital das Clinkers da Faculdadc dc Mcdkina da
`tie Medicina /b Univenklade de Sou Paulo, Sao Paulo. Btubi: Paula Cristina do
`Brazil; Nyasa Yuriko Sakai Valente. MD. PhD. Hospital das (:lincas dot Faculdadc
`Clinkers da Facukbde de Medicine da Universidase de Sao, Paulo, San Paulo,
`Unhrrsidadc tie Sant Paulo. Sao Paulo. Bradt Valíira Aoki. MD. PhD. Hospital das
`Neves tie Oliveira. MD, Iospital tics Clinical cis Facuklade do Medicina da
`Medicine da Univ rskbde de Sao Pxtkr. Sao Paulo. Brazil; Caroline Marls Takata
`Antonio Postro Ribciro Ilcr(nger, MID. I lospiul das Clínicas dot facuklak its
`tertiary. hospital
`Clinicopathologk correlation of 282 leukocytoclastk vascuHtis case in a
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`Onnrxcrdall suppxC None Ide nlßed
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`lions during periods of disease activity appxan:d to he useful in 50% of cases.
`that remain uncharacterized alter intmumdtkxttng repeating standard imtstiga
`antilaminin I pemphigoid. BP. 3IMP and EBA. In patients with blistering disorders
`these patients were found to ham subcpkkrmal blistering disorders: particularly
`subjects with negative or inconclusve results on standard investigations. Most of
`trade sWsenfup analysis di(fiadt. Imnsuroldutting was a useful adjunct in 78.4% of
`Dbcussio and conclusions: This study was limited by the small sample size, which
`ouclorKlerlrrdL
`remaining 4 out of 8 patients (50%) had blistering disorders Out remain
`activity and 1/8 (123%) had the dlu wosit confirmed with immunomapping The
`repeating standard investigations (hbology. DIE; lU) during periods of disease
`krconclilsive mYrunobbt rsufS. 3/8 (37.5 %) had the diagnosis established via
`(LAIR)) and I (3.4%) with hollows SI.E (tithe renuinlog 8/37 (21.6%) patients with
`plastic pemnphigtn(PNP). I (3.4%) with PNP, I (3.4 %) with linear IrA hulbus disease
`with cpklemodysis Wilma acquhl a (EIS.5). 2 (6.9%) with exclusion of panne.
`membrane pcmphigdd (MMP), 3 (111.3%) with bulbuls pcmphigtitl (BP). 3 (10.3`)
`namely 14 (48.3 %) patients with anti bminin 1 penip liigoid. 4 (13.8%) with mucous
`Immtnmddsxting was useful in mabhshing the diagnosis in 29 patients (78.4%):
`had sohepid mmal and 4 (10.8%) had Mraepi krmal Mistring disasses.
`(48.6%) were women. Mean age of presentation was 57.6 years. Thirty -three (89.2%)
`Results: Thin)sr rn patients were recruited. of which 19 (51.4 %) were men and 18
`wale performed at the Department of Dermatology, University of Lubeck. Germany.
`inconsigrnt clinical, pathological or srndogical findings. Immunoldating atxhes
`with negative findings on standard imrstigtrkms (hist04og1)1E 11F, ELISA) or
`May 2015. Patients were included if they had undifferentiated blistering disorders
`Methods: Subjects were (remittal at the National Skin Centre from January 2008 to
`,nine investigaikxn were negative. inconclusive, or disrnnbnn.
`immuntdddting in the diagnosis of suspected immunohunom dbcaxs where
`diseres might he inadequate. This study aimed to determine the usefulness of
`immumxtothent antibody assays (EUSA) used in the diagnosis of immunobulbus
`mtdltcs (DIF), indirect immunolurecence studies (111.) and enzyme-linked
`Background: Routine investigations such as IiLgMoogy. direct immuntal onscvmce
`Yen Lo Um, MBBS, N2U01u1 Skin Csinrc, Singapore
`Skin Centre, Singapore; Stut Ikon Tan. MBI ti. National Skin Centre. Singapore.
`MBBS. National Skin Centre. Singapore: Mark Bann Yang Tang. MBBS. National
`1Lxin Evelyn Tay, MISS, National Skin Centre, Singapore; Emily Piping Gan.
`patients with imnwtnobullous diseases
`Characterization of circulating nutoantibodies by immunoblotting In
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`YMJD10806_10809_proof
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`ABS 5.4.0 DTI)
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`3:11 am
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`ABS 5.4.0 DTD
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`J AN ACRD DouctorDLAB145
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`Moo 2016
`
`Moo 2016
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`Commercial support: .None identified
`
`Commercial support: Norse MettflfievL
`
`Commercial wallos None kientified
`
`disease. the true incktence of concurrent autoimmune disease is unclear.
`tpaka in a 1 .panokl female with biopsy pnnen PE Iiowwarr, since PF is such a rare
`autoimmune nadbtad diseases diagnosed in children with PE such as neurontyeStis
`disease skvdopet after the diagnosis of PF was made. There have been other
`discasc and PP its cisMßsxxt Ica teens reported in the literature. in this case, Grass.'
`in children. with fewsv than 50 eases reported. Only one otlw case of (:raves'
`present with scaly, crusted erosions in a sebortheic distributkn. Sporadic PP is rare
`I. It b more common in adults in the 4t1s6th decades of life. Patients typically
`autoimmune blistering dis o(kr which targets the desmo>rsnsmal protein dksmogktin
`nearly rear on 10 mg QOI) of pnoktiwde. Pemphigus foliaceus (PF) is an
`tapered down n am prcdnisone. At 6 months status pout -thyroid ablation, she was
`subsequently started on prrhsiso rte and mycophandatc msofrtil. She was sk,wly
`tutor IgG and C3 in the epidkrm's consistent with pemphigus fsdiaccus. She was
`tutor
`with Cosinophilic spongio>.sis and positive direct immunofiuoresccncr for intend -
`wassail( for direct insmunohdrescence. tlisto>pathobgy showed a subcornal split
`noted. Iiinp sy Ma veskle was performed for ravine histology and periksional skin
`and superficial en»kos with crust on her buttocks, back. chest, face anal scalp were
`her follow- up dermatology visit. flaccid vesicles on the inner upper arms and axd0.
`thyroid antibodies and was stand on mcthimazol and atetutkd. Pour week Later at
`who diagnosed her with Graves' disease shoe to a tow 1311 and markedly elevated
`WA antibiotic and topical al steroids. She was also urgently referral to endocrinology
`pariasiksrm dermatitis with impctiginizatiots was made, and she was started on an
`her chest grew pan.srnshisr Siopht4orsxttcr unreal. A provisional diagnosis of
`pruritus and continued diarrhea, fatigue. and cold intolerance. Bacterial culture of
`yellow cram on the chest. upper amts and bilateral breaxs. She complained of
`forehead with thick yellow malodorous crust. and pink papules and plaques with
`Exam in the dermatology clinic rcvcakd diffuse pink plaques do the scalp and
`presumptive diagnosis Malaya capitis and was empirically treated with grim:Malvin
`evaluation by the ED revealed a low TSli. normal CDC and she was given a
`breasts for 7 works. and diarrhea. fatigue and welkin loss for one amnia Initial
`A 15- yearold Hispanic female presented with a scaly eruption on per scalp and
`Kansas City, MO, llnitd Sates
`United States; Kimberly AHnr(i. MD. ChiMrens Mkry Ik»pitatKansas City.
`Rachel Iaarnsan, MD, Childrens Mercy Hospital-Kansas City, Kansas City. MO.
`Concurrent ent pemphigus foliaceus and Graves' disease
`3045
`
`AB144J AN A(:ws DEaM.cl(x.
`
`Costssserekll support: None Idertt ifiedf.
`
`a particular therapy.
`of psoriasis subtypes loonier to klrntil which patients are mon gkcly to respond to
`nil). We atakipotc that glycan hionarkers may te used in the future for stratification
`having the potential to find new and unique homarkers for diseases of amoimmo
`healthy COMMIS sw.rc identified. llese findings supp on the tick' of glyttscknee as
`total of 73 gtycoc ngugaes that air differentially expressed in psoriasis comma' to
`giyccoc lation profile 432 32 patients with psoriasis compared to 10 healthy comrols. A
`roc and PS.C. This method was then utilized ro characterize the sitespccific
`expressed between MSC and contras. 27 between Mani' ctntrois and 20 between
`104) as comparison. This drndmwtated 30 unique glcdeoniagiles difkomtiall
`and primary sclerosing ctodaogitis (PSC. n = 36), by mosey( healthy controls (n Q
`gastrointestinal autoimmune conditions, primary diary cirrhosis (PBC, n as 74)
`ations associated with autoimmune diseases. This was initially done in two
`mukipk P.-action monitoring (MR,M) to succexdsl y cluraclerize gleam alter-
`glcosybtions an he used as hi onarkris for autoimmunit. This study ttt izCt
`as hiomarkrrs have yet to he insestigct 1. We fought to determine if site -specific
`including rheumatoid arthritis, vasetdkis, and myasthenia grads, bu their utilization
`Altmtkou In gt cos lation have been kienutied in multiple autoimmune diseases,
`California. Dosis. Sacramento, C.A. United States
`California, Davis, Davis, CA, linked States; F,uanuat Maotrakis, Mto. University of
`Department of Chemistry, Davis. California: Kyoungni Kies, PhD. University of
`Angeles. CA. United Slate% CarIMU Lebrilla, PItD, University of C]Rfurtb, 1)avb
`(A. United States, April Armstrong, MD. University of Southern California, Los
`States; Wilson Lau. MD. University of California. Sat Francisco. San Francisco,
`Reason Wilkcn. MD, University of California. Davis. Sacramento. CA. (Ivied
`Forum Patel. MI). University of California, Davis. Sacramento. CA, United Stares;
`Characterization of glycosylation profiles in psoriasis
`3046
`
`

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