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    Bullous Pemphigoid Vs Epydermolysis Bullosa Acquisita: Diagnosis and How To Differentiate

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    Irene Irene
    Bullous Pemphigoid (BP) and Epidermolysis Bullosa Acquisita (EBA) are subepidermal bullous diseases that can be difficult to differentiate based on clinical features alone. BP typically affects older adults and presents with tense subepidermal blisters on an erythematous or urticarial base that heal without scarring. Pathology shows eosinophilic infiltration and direct immunofluorescence demonstrates linear IgG and C3 deposition along the basement membrane zone. In contrast, EBA usually presents in trauma-prone areas with subepidermal blistering, residual scarring, and milia formation. Direct immunofluorescence of EBA skin biopsies reveals IgG deposits

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    Bullous Pemphigoid Vs Epydermolysis Bullosa Acquisita: Diagnosis and How To Differentiate

    Uploaded by

    Irene Irene
    29 views12 pages
    Bullous Pemphigoid (BP) and Epidermolysis Bullosa Acquisita (EBA) are subepidermal bullous diseases that can be difficult to differentiate based on clinical features alone. BP typically affects older adults and presents with tense subepidermal blisters on an erythematous or urticarial base that heal without scarring. Pathology shows eosinophilic infiltration and direct immunofluorescence demonstrates linear IgG and C3 deposition along the basement membrane zone. In contrast, EBA usually presents in trauma-prone areas with subepidermal blistering, residual scarring, and milia formation. Direct immunofluorescence of EBA skin biopsies reveals IgG deposits

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    Differensial diagnosis bullous pemphigoid vs epidermolisis bullosa acquisita

    Original Title

    Diagnosis BP vs EBA

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    Bullous Pemphigoid (BP) and Epidermolysis Bullosa Acquisita (EBA) are subepidermal bullous diseases that can be difficult to differentiate based on clinical features alone. BP typically affects older adults and presents with tense subepidermal blisters on an erythematous or urticarial base that heal without scarring. Pathology shows eosinophilic infiltration and direct immunofluorescence demonstrates linear IgG and C3 deposition along the basement membrane zone. In contrast, EBA usually presents in trauma-prone areas with subepidermal blistering, residual scarring, and milia formation. Direct immunofluorescence of EBA skin biopsies reveals IgG deposits

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    29 views12 pages

    Bullous Pemphigoid Vs Epydermolysis Bullosa Acquisita: Diagnosis and How To Differentiate

    Uploaded by

    Irene Irene
    Bullous Pemphigoid (BP) and Epidermolysis Bullosa Acquisita (EBA) are subepidermal bullous diseases that can be difficult to differentiate based on clinical features alone. BP typically affects older adults and presents with tense subepidermal blisters on an erythematous or urticarial base that heal without scarring. Pathology shows eosinophilic infiltration and direct immunofluorescence demonstrates linear IgG and C3 deposition along the basement membrane zone. In contrast, EBA usually presents in trauma-prone areas with subepidermal blistering, residual scarring, and milia formation. Direct immunofluorescence of EBA skin biopsies reveals IgG deposits

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    of 12

    Bullous

    Pemphigoid vs
    Epydermolysis
    Bullosa
    Acquisita
    Diagnosis and How to Differentiate
    Bullous Pemphigoid (FP9 944)

    Mostly geriatric >60 y.o. | Faktor resiko : neurologic


    bulla subepidermal isi sel disease (demensia, stroke, Komplikasi : Sepsis, infeksi
    eosinofil | IgG endapan Parkinson), gangguan kulit, ketidakseimbangan
    pada atap celah, DIF linear psikiatri, bedridden, elektrolit, dehidrasi
    IgG dan C3 pada BMZ (atap) polypharmacy

    Th/ kortikosteroid topikal/


    sistemik, imunosupresif lain
    (Aza, MMF, MTX)
    Klinis Bullous Pemphogoid
    PA

    • Bulla tegang, dasar kulit N/


    eritem/urtika  Nikolsky negatif • Celah subepidermal,
    • Sembuh tanpa meninggalkan scar infiltrat eosinofil dan
    (bisa kambuh2an) neutrofil
    • PA pada urtika lesi awal PB
     spongiosis eosinofilik
    Immunofluorescence
    BP (Cont.)
    • Patogenesis
    • Circulating & tissue-
    bound autoantibodies
    thd BP180/ BPAG2
    (antigen yg paling
    berperan) dan
    BP230/BPAG1
    • Ig G yang mentarget
    regio NC16A
    DD/
    Struktur BMZ, Letak Celah dan Autoantibody

    7
    Klasifikasi BMZ separation dengan Dowling Meara  herpetiformis, erosi
    Transmission Electron Microscopy (TEM) luas, kuku, most severe EBS

    EB SIMPLEX Koebner  oral mucosa mild erosion,


    (Hemidesmosome, KRT 5 dan 14) no severe growth retardation

    Weber-Cockayne  most common EB,


    hyperhidrosis palm and soles, area
    trauma juga, tapi tanpa scar dan milia

    INHERITED EB EB JUNCTIONAL
    Herlitz  periorificial granuloma,
    (BPAG2, Anchoring filaments, Lamina mematikan
    lucida)
    EB

    EB ACQUISITA (EBA)
    (Collagen 7 -- gen pengkode defek di Cockayne-Touraine  kuku distrofik,
    lengan pendek kromosom 3/ Anchoring skar atrofi, pada area sering trauma
    fibril di sublamina densa, > orang tua, dengan milia dan scarring
    EB DYSTROPHIC
    area trauma, DIF IgG lantai BAwah)
    (Collagen 7, sublamina densa, defek
    COL7A1 )
    Hallopeau-siemens 
    pseudosyndactyly, mitten hands (sarung
    tangan), SCC
    8
    EBA (FP9 971)

    Autoimmune
    Klinis : Skin fragility, Predileksi : trauma-
    subepidermal bullous
    subepidermal blisters, prone areas (hands,
    disease, IgG
    residual scarring, milia feet, elbows, knees,
    autoantibodi thd
    formation sacrum, nails, mouth)
    kolagen tipe VII

    Th/ sulit | Opsi Th/


    Patologi : bulla DIF : IgG deposit di
    colchicine, cyclosporin
    subepidermal, fibrosis, epidermal–dermal
    A, dapson| refrakter thd
    milia formation junction.
    KS, Aza, MTX
    IF direct &
    Indirect EBA
    Alur
    diagnosis
    dan DD/
    EBA

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