Table 1.
Age, gender and clinical presentation of EBA patients
| Age (years)b | |||
| - Median | 50 | ||
| - Range | 1–94 | ||
| - Mean | 46.7 | ||
| - Std Deva | 22.1 | ||
| Genderc | |||
| - Female | 54% | ||
| - Male | 46% | ||
| Clinical phenotyped | |||
| - Non-MB | 55% | ||
| - MB | 38% | ||
| - Both | 7% | ||
| Mucosal involvement (any)e | Mucosal involvement (specific)f | ||
| - Yes | 23% | - Ocular | 14.1% |
| - No or not indicated | 77% | - Oral | 90.8% |
| - Esophagus | 11.5% | ||
| - Trachea/larynx | 8.8% | ||
| - Anal | 3.0% | ||
| - Genital | 14.1% | ||
| Associated diseasesg | Associated inflammatory diseasesg | ||
| - Any | 9.6% | - CD | 0.9% |
| - Inflammatory | 4.4% | - RA | 0.5% |
| - Metabolic | 1.3% | - Thyroiditis | 0.4% |
| - Infection | 0.9% | - UC | 0.6% |
| - Cancer | 1.9% | - Psoriasis | 0.4% |
| - Cardiovascular | 0.6% | - DTH | 0.1% |
| - Neurology | 0.5% | - Acquired hemophilia | 0.1% |
| - Other | 0.2% | - AIBD | 0.6% |
| - SLE | 0.4% | ||
| - Nephritis | 0.1% | ||
| - ITP | 0.1% | ||
aAbbreviations: Std Dev standard deviation, non-MB non-mechanobullous EBA variant, MB mechanobullous EBA variant, CD Crohn’s disease, RA rheumatoid arthritis, UC ulcerative colitis, DTH delayed type hypersensitivity, AIBD autoimmune bullous dermatoses, SLE systemic lupus erythematosus, ITP idiopathic thrombocytopenic purpura
bSpecified in 384 cases
cSpecified in 561 cases
dSpecified in 366 cases
eReported in 261 cases
fAs multiple sites were affected in some patients, the sum of specific mucosal involvement is greater than the reported 23% of patients experiencing any mucosal involvement
gReported in 97 cases. The sum of individual associated diseases may be higher than the indicated total due to multiple associated diseases