Clinical, etiologic, and histopathologic features of Stevens-Johnson syndrome during an 8-year period at Mayo Clinic

Abstract

Objective: To examine clinical, etiologic, and histologic features of Stevens-Johnson syndrome and to identify possible correlates of clinical disease severity related to etiologic and histopathologic findings.

Patients and methods: This is a retrospective review of patients seen at Mayo Clinic between January 1, 2000, and December 31, 2007.

Results: Of 27 patients (mean age, 28.1 years), 22 (81%) had involvement of 2 or more mucous membranes, and 19 (70%) had ocular involvement. Medications, most commonly antibiotics and anticonvulsants, were causative in 20 patients. Mycoplasma pneumoniae infection caused 6 of the 27 cases. Corticosteroids were the most common systemic therapy. No patients with mycoplasma-induced Stevens-Johnson syndrome had internal organ involvement or required treatment in the intensive care unit, in contrast to 4 patients each in the drug-induced group. Three patients had chronic ocular sequelae, and 1 died of complications. Biopsy specimens from 13 patients (48%) showed epidermal necrosis (8 patients), basal vacuolar change (10 patients), and subepidermal bullae (10 patients). Biopsy specimens from 11 patients displayed moderate or dense dermal infiltrate. Histologic features in drug-induced cases included individual necrotic keratinocytes, dense dermal infiltrate, red blood cell extravasation, pigment incontinence, parakeratosis, and substantial eosinophils or neutrophils.

Conclusion: Our clinical and etiologic findings corroborate those in previous reports. M pneumoniae-induced Stevens-Johnson syndrome manifested less severely than its drug-induced counterpart. The limited number of biopsies precludes unequivocal demonstration of histopathologic differences between drug-induced and M pneumoniae-induced Stevens-Johnson syndrome.

FIGURE 1.

29-year-old woman (patient 13 in Table 4) with Stevens-Johnson syndrome secondary to Mycoplasma pneumoniae infection. Left, Widespread macules ranging from erythematous to purpuric and flat atypical target lesions on the back. Right, Prominent hemorrhagic crust on the lips, eyes, and nasal mucosa.

FIGURE 2.

Histopathology of Stevens-Johnson syndrome. Left, Focal basal cell vacuolar change with dense superficial dermal lymphocytic inflammation and occasional eosinophils in patient with Stevens-Johnson syndrome secondary to lamotrigine therapy (hematoxylin-eosin, original magnification ×40). Right, Full-thickness necrosis, basal vacuolar change, and subepidermal bullae in patient with Stevens-Johnson syndrome secondary to Mycoplasma pneumoniae infection (hematoxylin-eosin, original magnification ×20).