Stevens–Johnson Syndrome (SJS)/Toxic Epidermal Necrolysis (TEN)- Rare, life-threatening mucocutaneous reaction almost always drug-related (rarely due to infection or immunizations) with 25–50% mortality for TEN and much less for SJS
- Presents with poorly demarcated dusky erythematous or purpuric macules, papules, patches, or plaques that subsequently result in flaccid bullae or frank epidermal detachment due to necrosis of the epidermis; often starts on the trunk and spreads to face, proximal upper extremities and neck; erythema with painful mucosal erosions/ulcerations involving genital, buccal, and ocular mucosa seen in most cases; ± Nikolsky sign (dermal-epidermal cleavage with tangential pressure on normal appearing skin), ± Asboe-Hansen sign (bullae extend laterally with pressure); ± palmoplantar involvement; ± respiratory and GI epithelial involvement
- TEN > 30% BSA with skin detachment; SJS/TEN overlap with 10–30% involvement; SJS with < 10% BSA involvement
- Offending drugs: antibiotics (sulfonamides, PCNs), allopurinol, anticonvulsants (carbamazepine, lamotrigine, phenytoin), barbiturates, NSAIDs, antiretrovirals
- Poor outcome associated following (SCORTEN): age > 40, heart rate >120 bpm, BSA > 10% on day 1, ↑ serum urea >27 mg/dl, serum bicarbonate >20 mmol/l, ↑ glucose >250 mg/dl, underlying cancer or hematologic malignancy
- Complications: symblepharon, synechiae, cutaneous scarring, eruptive nevi, phimosis, nail dystrophy, alopecia, blindness
- Histology: full-thickness epidermal necrosis; early lesions with apoptotic keratinocytes
- Treatment: remove offending drug, supportive treatment (electrolyte replacement, wound care, nutritional support, hydration) typically in ICU or burn unit
| | | | | | | Table 7-13 Duration Before Onset of Drug Reaction | | | Reaction | | Typical Onset of Eruption After Drug Ingestion | | | AGEP | | Hours to 2 days | | | Phototoxic eruption | | Hours to 7 days | | | Urticaria | | Hours to 6 days | | | Lichenoid eruption | | 30–100 days | | | Fixed drug eruption | | Up to 2 weeks with first exposure (<24 h in subsequent exposure) | | | DRESS | | 2–6 weeks (mean 3–4 weeks) | | | SJS/TEN | | 7–21 days (first 2 months for anticonvulsant) | | | Morbilliform reaction | | 7–21 days | | | LABD | | 24 h–2 weeks | | | Pemphigus vulgaris | | Several weeks or months | | | | | |
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| Figure 7.4
A: Fixed drug eruption
(Courtesy of Dr. Sophie M. Worobec)
B: Fixed drug eruption (Courtesy of Dr. Paul Getz)
C: Fixed drug eruption (Courtesy of Dr. Paul Getz)
D: Fixed drug eruption (bullous)
E: Acneiform eruption (due to EGFRI)
F: Flagellate pigmentation (bleomycin)
(Reprint from Burgdorf WH, Plewig G, Wolff HH,
Landthaler M, eds. Braun-Falco’s Dermatology. 3rd
ed. Heidelberg: Springer; 2009) |
| | | Figure 7.5
A: Acneiform eruption (EGFRI)
B: Hydroxyurea ulceration
(Courtesy of Dr. Sophie M. Worobec)
C: Nail hyperpigmentation (AZT)
(Courtesy of Dr. Iris K. Aronson)
D: Toxic epidermal necrolysis
(Courtesy of Dr. Paul Getz) |
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