Keratin 5
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normal filamentous network within keratinocytes
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Keratin 14
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EBS, autosomal recessive
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absent or markedly reduced staining of filaments within keratinocytes
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Laminin-332 (laminin-5)
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JEB-H
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absent or markedly reduced staining along the DEJ
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JEB-nH *
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reduced staining along the DEJ
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Type XVII collagen
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JEB-nH *
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reduced or absent staining along the DEJ
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Type VII collagen
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RDEB, severe generalized
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usually absent staining along the DEJ
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RDEB, generalized other
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reduced staining along the DEJ
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RDEB, inversa
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variable staining along the DEJ
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Dystrophic EB-BDN (only during periods of active blistering)
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granular staining within basal and lower suprabasal keratinocytes; absent or markedly reduced staining along the DEJ
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Plectin
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EBS with muscular dystrophy; EBS with pyloric atresia; EBS-Ogna
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absent or reduced staining along the DEJ; absent or reduced staining along the DEJ; reduced staining along the DEJ
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α6β4 integrin
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JEB with pyloric atresia; EBS with pyloric atresia
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absent or reduced staining along the DEJ; absent or reduced staining along the DEJ
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Kindlin-1
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Kindler syndrome
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absent or reduced staining along the DEJ
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