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©2013 UpToDate ® Print Email Antigenic alterations in epidermolysis bullosa skin Antigen Abnormal staining in: Usual pattern of staining Keratin 14 EBS-AR Absent or markedly reduced Laminin-332 (laminin-5) JEB-H Absent or markedly reduced JEB-nH, generalized* Reduced Type XVII collagen JEB-nH, generalized • Absent JEB-nH, localized Reduced Type VII collagen RDEB, severe generalized Absent or markedly reduced RDEB, generalized other Reduced RDEB, inversa Variable DEB-BDN (only during period of active blistering) Granular staining within basal and suprabasal keratinocytes; absent or markedly reduced staining along DEJ Plectin EBS-MD Absent or reduced EBS-PA Absent or reduced EBS-Ogna Reduced α6β4 Integrin JEB-PA Absent or reduced EBS-PA Absent or reduced JEB-nH Δ Reduced Kindlin-1 Kindler syndrome Absent, reduced, or normal ◊ BDN: bullous dermolysis of the newborn; DEB: dystrophic EB; DEJ: dermoepidermal junction; DM: Dowling-Meara; EBS: EB simplex; H: Herlitz; JEB: junctional EB; MD: muscular dystrophy; nH: non-Herlitz; PA: pyloric atresia; RDEB: recessive DEB. * In the majority of JEB-nH patients. • In a minority of patients with generalized JEB-nH (previously referred to as generalized atrophic benign EB); these patients lack concurrent abnormal staining by antibodies to laminin-332. Δ Reported in one patient. ◊ Loss-of-function mutations in both KIND1 alleles may be associated with normal immunostaining. From: Fine JD, Eady RAJ, Bauer EA, et al. The classification of inherited epidermolysis bullosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB. J Am Acad Dermatol 2008; 58:931. Table used with the permission of Elsevier Inc. All rights reserved.