P6106Epitope spreading in pemphigus patients from S~ao Paulo, Brazil: A 10-yearfollow-up

Livia Delgado, MD, Hospital das Clinicas, Faculty of Medicine, University of S~aoPaulo, S~ao Paulo, Brazil; Celina W. Maruta, MD, Hospital das Clinicas, Faculty ofMedicine, University of S~ao Paulo, S~ao Paulo, Brazil; Claudia G. Santi, MD,Hospital das Clinicas, Faculty of Medicine, University of S~ao Paulo, S~ao Paulo,Brazil; Denise Myamoto, MD, Hospital das Clinicas, Faculty of Medicine,University of S~ao Paulo, S~ao Paulo, Brazil; Valeria Aoki, PhD, Hospital dasClinicas, Faculty of Medicine, University of S~ao Paulo, S~ao Paulo, Brazil

Background: Pemphigus is a rare autoimmune blistering dermatosis. The shift from adisease to another is known as ‘‘epitope spreading’’ (ES). A primary inflammator-y/autoimmune process releases ‘‘hidden’’ epitopes, and evokes a secondary reactionto antigens, distinct from and nonecross-reactive with the target epitope. ES may beintra- or intermolecular.

Objectives: The aim of the present study is to analyze the occurrence of ES within a10-year interval in pemphigus patients followed-up at the Department ofDermatology, University of S~ao Paulo, Brazil.

Methods: The studywas carried out in 351 pemphigus patients from January 2002 toJanuary 2012. A careful search for clinical and laboratory changes suggestive oftransition to a secondary bullous disease was performed, including clinical evalu-ation by at least 2 dermatologists. Laboratory profile (histopathology, direct-DIF andindirect-IIF immunofluorescence, and ELISA) was requested when suspicious skinlesions were detected.

Results: Eight out of 351 pemphigus patients (153 pemphigus foliaceus-PF and 198pemphigus vulgaris-PV) presented clinical transition: 7 from PV to PF (group 1) and1 from PF to epidermolysis bullosa acquisita (EBA) (group 2). In group 1, median ageat PVonset was 54 years andmedian interval of the disease shift was 3 years. Of the 7patients with clinical PF, 4 showed change of pattern from suprabasilar cleavage tosubcorneal acantholysis, 2 had cleavage within the middle epidermal layer, and1 sustained the suprabasilar acantholysis. All patients showed intercellular IgG andC3 deposits during PVand PF diagnosis by DIF. IIF titers varied from 1:160 to 1:5120,and were positive during ES. ELISA index for Dsg1 varied from 1 to 319, and for Dsg3from 0 to 290. Dsg1/Dsg3 indexes corresponded to the clinical PV-PF changes. Ingroup 2, one patient switched from PF to EBA; onset of PF occurred at the age of 7,and ES to EBA 25 years later. Laboratory evaluation showed subepidermal cleavagewith neutrophils, IgG intercellular staining in the epidermis and IgM, IgA, IgG andC3 deposits at BMZ by DIF, IgG deposits by indirect salt-split, recognition of collagenVII by immunoblotting, and positive ELISA for Dsg1.

Conclusions: Intermolecular ES occurred in 2.2% of pemphigus patients. Futuresstudies will be necessary to elucidate the pathogenesis of this event and itssignificance in pemphigus progression.

APRIL 20

cial support: None identified.

Commer

P6126Erythema multiforme associated with the combined use of lamotrigineand cyclobenzaprine: A case report

Lindsay Taylor Morgan Bicknell, University of Texas Medical School at Houston,Houston, TX, United States; Marsal Sanches, MD, PhD, University of TexasMedical School at Houston, Houston, TX, United States

Lamotrigine is widely used in the pharmacologic management of mood disorders. Itis FDA-approved as a maintenance treatment of bipolar disorder, but its common off-label uses include bipolar depression and antidepressant augmentation in patientswith major depressive disorder. The risk of cutaneous reactions, particularlyerythema multiforme, is a well recognized concern during treatment with thismedication. In order to minimize this risk, efforts have been made to identify factorsassociated with a higher rate of lamotrigine-induced rash. We report here a case oferythema multiforme, apparently precipitated by the associated use of lamotrigineand cyclobenzaprine, and discuss the importance of thorough medication review inthe face of puzzling dermatologic diagnoses.

cial support: None identified.

Commer

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P6694Intravenous immunoglobulin for the treatment of quetiapin-inducedbullous pemphigoid in an HIV-infected patient

Joana Cabete, Dermatology Department, Hospital de Santo Ant�onio dosCapuchos, Centro Hospitalar de Lisboa Central, Lisbon, Portugal; Ana Fidalgo,Dermatology Department, Hospital de Santo Ant�onio dos Capuchos, CentroHospitalar de Lisboa Central, Lisbon, Portugal; Filipa Rocha P�aris, DermatologyDepartment, Hospital de Santo Ant�onio dos Capuchos, Centro Hospitalar deLisboa Central, Lisbon, Portugal; Guida Santos, Dermatology Department,Hospital de Santo Ant�onio dos Capuchos, Centro Hospitalar de Lisboa Central,Lisbon, Portugal; Vasco Serr~ao, Dermatology Department, Hospital de SantoAnt�onio dos Capuchos, Centro Hospitalar de Lisboa Central, Lisbon, Portugal

Background: Drug eruptions are frequent in patients infectedwith HIV. Treatment ofsevere disease is primarily based on immunosuppressive drugs, raising concernsregarding additional immunosuppression.

Case report: A 48-year-old woman with HIV infection (CD4 count 319/�L,undetectable viral load), HIV-associated dementia, and dyslipidemia presentedwith a generalized pruritic bullous eruption, with numerous tense blisters, erosions,and urticarial plaques. The diagnosis of bullous pemphigoid (BP) was confirmed bylight microscopy and direct immunofluorescence. Underlying neoplastic andautoimmune diseases were excluded. The patient was polymedicated, mostlychronic medications, such as antiretroviral therapy, and multiple psychoactivedrugs. Both quetiapine and pitavastatin had been introduced during the past year.The patient was started on oral corticotherapy (1 mg/kg/day prednisone), butazathioprine (100 mg/day) was later added to this regimen given her unresponsive-ness to corticosteroid solely. Slow and partial response, along with the developmentof bicytopenia forced azathioprine suspension, and intravenous immunoglobulin(IVIG; initially 1 g/kg daily for 2 days, later 0.5 g/kg/d for 4 days) was initiated inassociationwith corticotherapy. Despite transient disease control, recurrent bullousflares suggested a persistent triggering factor, namely a drug. Specifically, quetiapinwas discontinued. The patient’s response was immediate, with progressive resolu-tion of lesions and eosinophilia. Inadvertent rechallenge with olanzapine (a relateddrug to quetiapin) led to a new eruption, confirming drug-induced bullouspemphigoid (DIBP). A total of 6 IVIG cycles were completed, and prednisone wasprogressively tapered until suspension.

Discussion: Autoimmune bullous diseases in association with HIV infection are rareand enigmatic, probably as a result of immune dysregulation. Moreover, the specificinduction of BP by quetiapin is rarer, with only 1 previous report in literature.Regarding treatment, IVIG can be an efficient and safe alternative to immunosup-pressive therapy in HIV patients to treat severe autoimmune/inflammatoryconditions.

Conclusions: Quetiapin should be added to the list of neuroleptics previously linkedto DIBP. In addition, IVIG can be a valuable treatment option for the treatment of BPin HIV patients. Further studies regarding safety, efficacy, and dosage are warranted.

cial support: None identified.

Commer

P6464Linear IgA bullous dermatosis herpeticum: Disseminated herpes simplexvirus infection within the lesions of vancomycin-induced linear IgAbullous dermatosis

Shadi Rashtak, MD, Mayo Clinic College of Medicine, Rochester, MN, UnitedStates; Michael Camilleri, MD, Mayo Clinic College of Medicine, Rochester, MN,United States; Rokea el-Azhary, MD, PhD, Mayo Clinic College of Medicine,Rochester, MN, United States

We were consulted on a 59-year-old woman with painful erosions involving theback, posterior lower extremities, lips, and genitalia. She was recently started onmultiple systemic antibiotics including vancomycin for ventilator-associated pneu-monia. Skin and lip swabs were positive for herpes simplex virus-1. Histopathologicexamination of an intact vesicle on the flank showed subepidermal cleft, papillarydermal edema, and mixed inflammation with neutrophils and eosinophils withoutviral cytopathic changes. Direct immunofluorescence studies showed strongcontinuous linear IgA deposition along the dermoepidermal junction. Indirectimmunofluorescence of human salt-split skin demonstrated epidermal IgA staining.The rash resolved with discontinuation of vancomycin and initiation of acyclovir. Inconclusion, disseminated herpes simplex infection can occur within the openlesions of linear IgA bullous dermatosis (LABD) in a manner similar to eczemaherpeticum. To our knowledge this is the first reported case of disseminated herpessimplex infection in the setting of vancomycin-associated LABD.

cial support: None identified.

Commer

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