Clinical considerations for epidermal necrolysis

Abstract

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered a spectrum of acute life-threatening mucocutaneous reactions that differ only in severity. Both diseases are characterized by mucous membrane and skin involvement, are often caused by medications, and are collectively known as epidermal necrolysis (EN).

Methods: A severity of illness score has been devised to predict prognosis in patients with EN. The scoring system addresses 7 prognostic factors.

Results: Patients with EN require supportive care. Those with extensive skin involvement should be admitted to an intensive care unit or burn unit if possible. Suspected, as well as unnecessary, medications should be discontinued. Baseline laboratory tests, imaging, cultures, and biopsies should be obtained. Intravenous access should be established and hydration and nutritional support begun. Daily oral care, wound care, pain control, and early physician consultation are also important aspects of treatment.

Conclusion: EN requires early diagnosis, appropriate workup, and appropriate treatment to minimize potential morbidity and mortality. In many clinicians' experience, EN is rare; therefore, education and improved understanding of the potential causes and appropriate treatment regimens are vital when confronted with such a patient.

Keywords: Drug eruptions; Stevens-Johnson syndrome; drug hypersensitivity.

Conflict of interest statement

The authors have no financial or proprietary interest in the subject matter of this article.

Figures

Figure 1.

Histopathology of Stevens-Johnson syndrome. (Left) Focal basal cell vacuolar change with dense superficial dermal lymphocytic inflammation and occasional eosinophils in a patient with Stevens-Johnson syndrome secondary to lamotrigine therapy (hematoxylin and eosin stain, original magnification ×40). (Right) Full-thickness necrosis, basal vacuolar change, and subepidermal bullae in a patient with Stevens-Johnson syndrome secondary to Mycoplasma pneumoniae infection (hematoxylin and eosin stain, original magnification ×20). (Reprinted from Mayo Clinic Proceedings, 85(2), Wetter DA, Camilleri MJ, Clinical, Etiologic, and Histopathologic Features of Stevens-Johnson Syndrome During an 8-Year Period at Mayo Clinic, 131-138, 2010, with permission from Elsevier.)

Figure 2.

Patient with toxic epidermal necrolysis showing bleeding during dressing change. (Reprinted from Burns, 34(5), Ugburo AO, Temiye EO, Ilombu CA, A 12-year retrospective study of non-burn skin loss (burn-like syndromes) at a tertiary burns unit in a developing country, 637-643, 2008, with permission from Elsevier.)