Table 3.
Clinical characteristics tables
| Overall (n = 100) |
DRESS (n = 50) |
SJS/TEN (n = 39) |
AGEP (n = 10) |
|
|---|---|---|---|---|
| Time in days from index rash to admission at participating site, median (IQR) | 5.5 (3, 13) (n = 98) | 8.5 (4, 17.5) (n = 48) | 5 (3, 11) | 3.5 (2, 5) |
| Rash components | ||||
| Erythroderma (≥90% BSA involvement | 20 (20%) | 12 (24%) | 8 (21%) | 0 (0%) |
| Maculopapular | 68 (68%) | 46 (92%) | 17 (44%) | 5 (50%) |
| Urticarial (raised oedematous non-scaly plaques) | 32 (32%) | 19 (38%) | 8 (21%) | 5 (50%) |
| Pustules | 15 (15%) | 5 (10%) | 0 (0%) | 10 (100%) |
| Vesicles, bullae or blisters | 32 (32%) | 6 (12%) | 25 (64%) | 0 (0%) |
| Erosion/crusting | 43 (43%) | 6 (12%) | 37 (95%) | 0 (0%) |
| Typical target lesions (3 zones) | 3 (3%) | 3 (6%) | 0 (0%) | 0 (0%) |
| Atypical targets (2 zones) | 24 (24%) | 5 (10%) | 18 (46%) | 1 (10%) |
| Exfoliative dermatitis/desquamation (superficial epidermal peeling) | 37 (37%) | 19 (38%) | 13 (33%) | 5 (50%) |
| Epidermal detachment | 23 (23%) | 0 (0%) | 22 (56%) | 0 (0%) |
| Distribution of Rash | ||||
| Distal extremities (arms/legs) greater than truck/torso, no mucosal surfaces affected | 5 (5%) | 2 (4%) | 1 (3%) | 2 (20%) |
| Distal extremities (arms/legs) greater than truck/torso, 1 mucosal surface affected (conjunctiva, lips/oral, genitalia) | 3 (3%) | 2 (4%) | 1 (3%) | 0 (0%) |
| Distal extremities (arms/legs) greater than trunk/torso, 2 mucosal surfaces affected | 1 (1%) | 0 (0%) | 1 (3%) | 0 (0%) |
| Distal extremities (arms/legs) greater than trunk/torso, 3 mucosal surfaces affected | 1 (1%) | 0 (0%) | 1 (3%) | 0 (0%) |
| Trunk/torso greater than extremities (arms/legs), no mucosal surfaces affected | 13 (13%) | 9 (18%) | 0 (0%) | 3 (30%) |
| Trunk/torso greater than extremities (arms/legs), 1 mucosal surfaces affected | 7 (7%) | 2 (4%) | 5 (13%) | 0 (0%) |
| Trunk/torso greater than extremities (arms/legs), 2 mucosal surfaces affected | 7 (7%) | 0 (0%) | 7 (18%) | 0 (0%) |
| Trunk/torso greater than extremities (arms/legs), 3 mucosal surfaces affected | 4 (4%) | 0 (0%) | 4 (10%) | 0 (0%) |
| Widespread (>50%), 0 mucosal surfaces affected | 33 (33%) | 28 (56%) | 1 (3%) | 4 (40%) |
| Widespread (>50%), 1 mucosal surface | 11 (11%) | 4 (8%) | 6 (15%) | 1 (10%) |
| Widespread (>50%), 2 mucosal surfaces | 8 (8%) | 2 (4%) | 6 (15%) | 0 (0%) |
| Widespread (>50%), 3 mucosal surfaces affected | 6 (6%) | 0 (0%) | 6 (15%) | 0 (0%) |
| Facial involvement | ||||
| No | 15 (15%) | 7 (14%) | 3 (8%) | 4 (40%) |
| Yes | 83 (83%) | 41 (82%) | 36 (92%) | 6 (60%) |
| Unknown | 2 (2%) | 2 (4%) | 0 (0%) | 0 (0%) |
| Facial oedema | 49 (49%) | 30 (60%) | 16 (41%) | 3 (30%) |
| Scalp involvement | ||||
| No | 47 (47%) | 23 (46%) | 17 (44%) | 6 (60%) |
| Yes | 31 (31%) | 19 (38%) | 11 (28%) | 1 (10%) |
| Unknown | 22 (22%) | 8 (16%) | 11 (28%) | 3 (30%) |
| Palms or sole involvement | ||||
| No | 42 (42%) | 22 (44%) | 12 (31%) | 7 (70%) |
| Yes | 45 (45%) | 21 (42%) | 22 (56%) | 2 (20%) |
| Unknown | 13 (13%) | 7 (14%) | 5 (13%) | 1 (10%) |
| Pruritus | ||||
| No | 17 (17%) | 3 (6%) | 12 (31%) | 1 (10%) |
| Yes | 70 (70%) | 44 (88%) | 19 (49%) | 7 (70%) |
| Unknown | 13 (13%) | 3 (6%) | 8 (21%) | 2 (20%) |
| Fever >38.5 °C on admission [within 48 h of rash onset] | 50 (50%) | 34 (68%) | 12 (31%) | 4 (40%) |
| Internal organ involvement (liver, renal, pulmonary) during admission | 67 (67%) | 43 (86%) | 21 (54%) | 3 (30%) |
| Renal Failure | 21 (21%) | 10 (20%) | 11 (28%) | 0 (0%) |
| Liver failure | 36 (36%) | 27 (54%) | 8 (21%) | 1 (10%) |
| Respiratory failure | 12 (12%) | 4 (8%) | 8 (21%) | 0 (0%) |
| Blood abnormality | ||||
| Platelets <150 × 10ˆ9/L | 24 (24%) | 9 (18%) | 14 (36%) | 1 (10%) |
| WBC <4 × 10ˆ9/L | 15 (15%) | 6 (12%) | 9 (23%) | 0 (0%) |
| Hb < 100 g/L | 40 (40%) | 19 (38%) | 20 (51%) | 1 (10%) |
| Atypical lymphocytes (on blood film) | 15 (15%) | 12 (24%) | 3 (8%) | 0 (0%) |
| Eosinophilia (>0.7 × 10ˆ9/L) | 45 (45%) | 37 (74%) | 4 (10%) | 4 (40%) |
| Resolution <15 days | 25 (25%) | 11 (22%) | 8 (21%) | 6 (60%) |
| History of herpes infection (HSV 1/2) in 1 month prior to onset | ||||
| No | 78 (78%) | 41 (82%) | 28 (72%) | 8 (80%) |
| Yes | 5 (5%) | 1 (2%) | 4 (10%) | 0 (0%) |
| Unknown | 17 (17%) | 8 (16%) | 7 (18%) | 2 (20%) |
| History of ANY infection in 1 month prior to onset | ||||
| No | 39 (39%) | 22 (44%) | 15 (38%) | 2 (20%) |
| Yes | 51 (51%) | 27 (54%) | 16 (41%) | 7 (70%) |
| Unknown | 10 (10%) | 1 (2%) | 8 (20%) | 1 (10%) |
| Known concurrent viraemia (HHV6, CMV, EBV - confirmed by positive blood PCR) | ||||
| No | 60 (60%) | 35 (70%) | 21 (54%) | 3 (30%) |
| Yes | 1 (1%) | 1 (2%) | 0 (0%) | 0 (0%) |
| Unknown | 39 (39%) | 14 (28%) | 18 (49%) | 7 (70%) |
| History of prior skin disorder? | 19 (19%) | 11 (22%) | 6 (15%) | 1 (10%) |
| Phenotype specific: | N (%) |
|---|---|
| DRESS (N = 50) | |
| Fever >38.5 °C | 40 (80%) |
| Enlarged lymph nodes at 2 or more sites > 1 cm | 12 (24%) |
| Atypical lymphocytes | 20 (40%) |
| Eosinophilia (×10ˆ9/L) | |
| No | 7 (14%) |
| 0.7–1.49 | 12 (24%) |
| ≥1.5 | 31 (62%) |
| Rash | |
| ≥ 50% body | 47 (94%) |
| Rash at least 2 of oedema, infiltration, purpura, scalling | 34 (68%) |
| Biopsy suggestive of DRESS | 39 (78%) |
| Pustules | 6 (12%) |
| Blisters | 6 (12%) |
| Number of internal organs involved | |
| 0 | 4 (8%) |
| 1 | 32 (64%) |
| 2 | 13 (26%) |
| 3 | 1 (2%) |
| Internal organ involved | |
| Renal | 19 (38%) |
| Liver | 39 (78%) |
| Pulmonary | 1 (2%) |
| CNS | 1 (2%) |
| Cardiac | 3 (6%) |
| GI track | 1 (2%) |
| Resolution of rash< 15 days | 9 (18%) |
| Biological investigations - More than 3 performed to exclude alternative diagnosis | 47 (94%) |
| RegiSCAR Score, median (IQR) | 6 (5, 6) (n = 49) |
| SJS/TEN (N = 39) | |
| Total estimated BSA of rash | |
| < 10% BSA | 6 (15%) |
| 10–30% BSA | 10 (26%) |
| 30–50% BSA | 2 (5%) |
| 50–90% BSA | 11 (28%) |
| 90–100% BSA (erythroderma) | 8 (21%) |
| Maximum estimated BSA of epidermal detachment (denuded/sloughed) during the course of illness | |
| BSA <10% | 18 (46%) |
| BSA 10–30% | 8 (21%) |
| BSA >30% | 10 (26%) |
| Unknown | 3 (8%) |
| Blistering pattern in SJS/TEN (select best match) (choice = erythema with sloughing | |
| Unchecked | 14 (36%) |
| Checked | 25 (64%) |
| Blisters on dusky macules | 15 (38%) |
| Vesicles (<5 mm)) | 5 (13%) |
| Nikolsky's sign | 21 (54%) |
| Blistering or bullous rash | 31 (79%) |
| Did the patient present with a fever >38 °C | 17 (44%) |
| Heart rate on admission >120 bpm | 10 (26%) |
| Urea on admission >10 mmol/L | 13 (33%) |
| SCORTEN score | |
| 0-1 | 16 (41%) |
| 2 | 6 (15%) |
| 3 | 7 (18%) |
| 4 | 8 (21%) |
| 5+ | 1 (3%) |
| Ophthalmology review | 33 (85%) |
| Gynaecology review | 12 (31%) |
| Urology review | 8 (21%) |
| Has Linear IgA disease, pemphigoid, SSSS, TEN-like lupus, other autoimmune bullous disease been excluded on biopsy | 35 (90%) |
| AGEP (N = 10) | |
| Type of pustules | |
| Follicular | 1 (10%) |
| Non-follicular | 7 (70%) |
| Both | 1 (10%) |
| Unknown | 1 (10%) |
| AGEP extent of erythema/pustules | |
| 10–30% BSA | 2 (20%) |
| 30–50% BSA | 4 (40%) |
| 50–90% BSA | 4 (40%) |
| Distribution | |
| Folds predominate | 3 (30%) |
| Torso | 8 (80%) |
| Limbs | 6 (60%) |
| Widespread | 2 (20%) |
| Head and neck | 2 (20%) |
| AGEP mucosal involvement | 1 (10%) |
| AGEP score, median (IQR) | 7 (6, 8) (n = 6) |
Abbreviations: AGEP, acute generalized oedematous pustulosis; SJS, Stevens Johnson Syndrome; TEN, toxic epidermal necrolysis; DRESS, drug reaction with eosinophilia and systemic symptoms; IQR, interquartile range; BSA, Body Surface Area.