Version May 2024
| Antigen | Abnormal staining | Usual pattern of staining |
| Keratin 5 | EBS | Absent or markedly reduced |
| Keratin 14 | EBS | Absent or markedly reduced |
| EXPH5 | EBS | Absent |
| KLHL24 | EBS with cardiomyopathy | Increased |
| 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 JEBΔ (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 localized◊ | Reduced | |
| Kindlin-1 | Kindler EB | Absent, reduced, or normal§ |
DEB: dystrophic epidermolysis bullosa; DEJ: dermoepidermal junction; EBS: epidermolysis bullosa simplex; JEB: junctional epidermolysis bullosa; MD: muscular dystrophy; PA: pyloric atresia; RDEB: recessive dystrophic epidermolysis bullosa.
* Terminology has been updated to reflect current classification of epidermolysis bullosa.[1]
¶ In the majority of patients with intermediate JEB.
Δ In a minority of patients with intermediate JEB (previously referred to as "generalized atrophic benign epidermolysis bullosa"); these patients lack concurrent abnormal staining by antibodies to laminin-332.
◊ Reported in 1 patient.
§ Loss-of-function mutations in both KIND1 alleles may be associated with normal immunostaining.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.
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