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Antigenic alterations in epidermolysis bullosa skin

Antigenic alterations in epidermolysis bullosa skin
Antigen Abnormal staining Usual pattern of staining
Keratin 14 EBS Absent or markedly reduced
Laminin-332 (laminin-5) Severe JEB (previously JEB generalized severe, Herlitz JEB) Absent or markedly reduced
Intermediate JEB* (previously JEB generalized intermediate, non-Herlitz JEB) Reduced
Type XVII collagen Intermediate JEB (previously JEB generalized intermediate, non-Herlitz JEB) Absent
Localized JEB Reduced
Type VII collagen Severe RDEB Absent or markedly reduced
Intermediate RDEB Reduced
RDEB inversa Variable
Self-improving DEB (also known as bullous dermolysis of the newborn, 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, intermediate with PLEC pathogenic variants (formerly type Ogna) Reduced
Alpha-6 beta-4 integrin JEB-PA Absent or reduced
EBS-PA Absent or reduced
JEB localizedΔ Reduced
Kindlin-1 Kindler EB Absent, reduced, or normal◊
EBS: epidermolysis bullosa simplex; JEB: junctional epidermolysis bullosa; RDEB: recessive dystrophic epidermolysis bullosa; DEB: dystrophic epidermolysis bullosa; DEJ: dermoepidermal junction; MD: muscular dystrophy; PA: pyloric atresia.
* In the majority of intermediate JEB patients.
¶ In a minority of intermediate JEB patients (previously referred to as "generalized atrophic benign epidermolysis bullosa"); these patients lack concurrent abnormal staining by antibodies to laminin-332.
Δ Reported in 1 patient.
◊ Loss-of-function mutations in both KIND1 alleles may be associated with normal immunostaining.
Terminology has been updated to reflect current classification of epidermolysis bullosa. Has C, Bauer JW, Bodemer C, et al. Consensus reclassification of inherited epidermolysis bullosa and other disorders with skin fragility. Br J Dermatol 2020; 183:614.
Original figure modified for this publication. 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.
Graphic 62232 Version 4.0