Table 1. Patient demographics, clinical features, and HLA genotype.
ALDEN (algorithm for drug causality for epidermal necrolysis) was used to determine culprit drugs for patients with TEN. For patients with MPE, the main putative drugs are also indicated. Disease severity for patients with TEN was evaluated by the SCORTEN (score of TEN) at day 0 (arrival at hospital and diagnosis). The SCORTEN predicts the risk of death. The SCORTEN scale consists of seven independent factors for high mortality and varies from 0 or 1 (low mortality rate) to 5 or more (very high mortality rate). Disease severity was appreciated by calculating percentages of skin detachment (using E-Burn application). The peak of disease was appreciated as the date at which patients with TEN displayed maximal percentage of skin detachment. Disease severity for patients with MPE was estimated on the basis of the extent of skin rash and the presence of systemic and/or visceral symptoms. None of the patients with MPE exhibit symptoms suggestive of drug reaction and eosinophilia systemic symptoms/drug-induced hypersensitivity syndrome, and the Kardaun score was <3 for all the patients. M, male; F, female; ENT, ear nose throat; SLE, systemic lupus erythematosus; HIV+, HIV positive; HCV+, hepatitis C virus positive; na, not applicable; G-CSF, granulocyte colony-stimulating factor. *No culprit drug was identified for patient TEN-9, using ALDEN. The patient received ibuprofen, doxycycline, sulfamethoxazole/trimethoprim, tetracycline, isoniazid, and rifampicin in the days before TEN onset.
| Demographics | Clinical characteristics | HLA genotype | |||||||||||
|
Patient ID |
Sex/ age |
Ethnicity |
Underlying diseases |
Comorbidities | Culprit drug |
Drug exposure before onset (days) |
Date and nature of first symptoms |
SCORTEN (TEN)/ severity (MPE) |
% of skin detachment at day 0 |
% and date of maximal skin detachment |
Treatment | Locus A | Locus B |
| TEN-1 | M/48 | East Asian | Hyperuricemia | None | Allopurinol | 8 | Day 2/fever | 3 | 2% | 100% at day 2 |
A*02; A*33 |
B*38; B*58 |
|
| TEN-2 | M/39 | European | Urine tract infection |
None | Sulfamethoxazole/ trimethoprim |
7 | Day 2/fever | 1 | 6% | 20% at day 5 | Systematic corticosteroid + G-CSF |
A*30; A*30 |
B*13; B*18 |
| TEN-3 | F/40 | European | Hyperuricemia | None | Allopurinol | 15 | Day 3/fever + skin rash |
2 | 20% | 80% at day 2 | A*02; A*03 |
B*27; B*58 |
|
| TEN-4 | M/74 | European | Melanoma | None | Vemurafenib | 22 | Day 4/skin rash |
5 | 30% | 100% at day 1 |
Maintenance of existing corticosteroid therapy + G-CSF |
A*03; A*23 |
B*44; B*51 |
| TEN-5 | M/32 | North African |
Pneumocystis prophylaxis |
HIV+ | Sulfamethoxazole/ trimethoprim |
15 | Day 2/fever | 3 | 10% | 80% at day 2 | Systematic corticosteroid + G-CSF |
A*02; A*24 |
B*44; B*45 |
| TEN-6 | F/83 | European | Urine tract infection |
Cardiac insufficiency |
Norfloxacin | 8 | Day 2/fever/ skin rash |
3 | 20% | 50% at day 5 | G-CSF | A*03; A*- |
B*18; B*73:01 |
| TEN-7 | M/50 | European | Gastritis | Cirrhosis | Pantoprazole | 10 | Day 1/fever + skin rash |
3 | 20% | 100% at day 2 |
G-CSF | A*02; A*11 |
B*15; B*44 |
| TEN-8 | F/33 | European | Bipolar disease | None | Lamotrigine | 12 | Day 3/fever + eye stinging |
2 | 10% | 40% at day 5 | A*02; A*30 |
B*08; B*44 |
|
| TEN-9 | F/34 | African American |
Chronic pain | None | * | 2 | Day 2/fever | 3 | 10% | 50% at day 3 | G-CSF | A*02; A*02 |
B*15; B*53 |
| TEN-10 | F/63 | American | Severe angina | None | Ceftriaxone, ciprofloxacin |
8 | Day 4/skin rash |
2 | 15% | 30% at day 3 | A*01:03; A*68 |
B*08; B*73:01 |
|
| TEN-11 | M/58 | European | Infectious osteoarthritis |
Diabetes, renal insufficiency |
Ceftriaxone | 15 | Day 1/skin rash |
4 | 10% | 60% at day 2 | G-CSF | A*02; A*29 |
B*44; B*45 |
| TEN-12 | F/27 | European | Cirrhosis | Autoimmune hepatitis |
Furosemide | 21 | Day 3/fever + skin rash |
3 | 40% | 40% at day 3 | Maintenance of existing corticosteroid therapy + G-CSF |
A*01; A*- |
B*08; B*51 |
| TEN-13 | F/75 | European | Postsurgery infection |
Bladder adenocarcinoma |
Cefixime | 4 | Day 1/fever + skin rash |
4 | 30% | 30% at day 2 | G-CSF | A*02; A*- |
B*44; B*57 |
| TEN-14 | H/41 | European | Myeloma | None | Revlimid | 15 | Day 1 + fever + skin rash |
2 | 5% | 25% at day 3 | Systematic corticosteroid |
A*02; A*02 |
B*15; B*27 |
| TEN-15 | F/69 | European | Lung infection | Ischemic stroke, SLE |
Levofloxacin, metronidazole |
5 | Day 2 + fever + skin rash |
3 | 10% | 50% at day 3 | A*03; A*30 |
B*18; B*40 |
|
| TEN-16 | F/69 | European | Lung infection | None | Pristinamycain | 1 | Day 0/fever + skin rash |
2 | 10% | 38% at day 2 | A*02; A*03 |
B*35; B*51 |
|
| TEN-17 | M/50 | European | Infection | None | Azithromycin, paracetamol |
5 | Day 2/fever + skin rash |
4 | 20% | 80% at day 5 | G-CSF | A*02; A*03 |
B*07; B*51 |
| TEN-18 | M/58 | European | Liver cancer | HCV+ | Sorafenib | 10 | Day 3/skin rash |
5 | 5% | 48% at day7 | G-CSF | A*03; A*11 |
B*35; B*40 |
| MPE-1 | H/18 | European | ENT infection | None | Amoxicillin | 2 | Day 1/skin rash |
Mild | na | na | Topical corticosteroid |
A*01; A*02 |
B*40; B*51 |
| MPE-2 | H/61 | European | ENT infection | None | Amoxicillin | 3 | Day 1/skin rash |
Mild | na | na | Topical corticosteroid |
A*02; A*- |
B*08; B*40 |
| MPE-3 | F/68 | European | Breast infection | None | Vancomycin | 28 | Day 4/skin rash |
Severe | na | na | Topical corticosteroid |
A*24; A*25 |
B*15; B*18 |
| MPE-4 | F/78 | European | Myeloma | None | Bortezomib | 5 | Day 4/skin rash |
Severe | na | na | Topical corticosteroid |
A*29; A*31 |
B*38; B*44 |
| MPE-5 | F/71 | European | Cardiac insufficiency |
None | Diltiazem | 15 | Day 3/skin rash |
Severe | na | na | Topical corticosteroid |
A*02; A*- |
B*51; B*- |
| MPE-6 | F/62 | European | Infectious osteoarthritis |
None | Vancomycin | 2 | Day 1/skin rash |
Severe | na | na | Topical corticosteroid |
A*01; A*02 |
B*40; B*57 |
| MPE-7 | H/61 | North African |
Pulmonary infection |
None | Vancomycin | 42 | Day 4/skin rash |
Mild | na | na | Topical corticosteroid |
A*02; A*32 |
B*49; B*51 |
| MPE-8 | F/24 | East Asian | Chronic pain | None | Ibuprofen | 9 | Day 3/skin rash |
Severe | na | na | Topical corticosteroid |
A*24; A*- |
B*15; B*38 |
| MPE-9 | F/94 | European | Urine tract infection |
None | Clindamycin | 3 | Day 3/skin rash |
Mild | na | na | Topical corticosteroid |
A*23; A*31 |
B*39; B49 |
| MPE-10 | F/62 | European | Graft-versus-host disease |
Bone marrow transplant |
Piperacillin/ tazobactam, contrast material |
2 | Day 1/skin rash |
Mild | na | na | Topical corticosteroid |
A*02; A*03 |
B*15:16; B*39 |
| MPE-11 | F/39 | North African |
Hypertension | SLE | Macrogol, urapidil, amlodipine |
14 | Day 3/skin rash |
Moderate | na | na | Topical corticosteroid |
A*32; A*34 |
B*39; B*44 |
| MPE-12 | F/62 | European | Hypertension, gout |
None | Allopurinol, fibrate | 28 | Day 2/skin rash |
Mild | na | na | Topical corticosteroid |
A*23; A*68 |
B*44; B*53 |
| MPE-13 | M/52 | North African |
Myeloma | None | Revlimid, bortezomid |
15 | Day 2/skin rash |
Severe | na | na | Systemic corticosteroid |
A*01; A*02 |
B*07; B*51 |
| MPE-14 | F/67 | European | Dermatomyositis | None | Hydroxychloroquine | 15 | Day 4/skin rash |
Mild | na | na | Topical corticosteroid |
A*01; A*29 |
B*08; B*44 |