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