Stevens–Johnson syndrome induced by mizoribine in a patient with systemic lupus erythematosus

Abstract

A 32-year-old Japanese woman, who had a treatment history of systemic lupus erythematosus (SLE) with lupus nephritis World Health Organization class IV for 11 months, visited our hospital due to fever, facial erythema, and erosion of the oral cavity on November 10, 2003. Her mucosal erosion and facial skin erythema progressed over the following week, and Stevens-Johnson syndrome was diagnosed due to pathological findings of the skin. Among the administrated drugs, only mizoribine, started 6 months earlier, produced a positive reaction in the drug lymphocyte stimulation test. Increased prednisolone and high dose intravenous gamma-globulin were given successfully. Cyclosporine at 50 mg was administered to control the SLE, followed by an increase to 100 mg on January 7, 2004. She suffered from abdominal pain, blindness, and convulsion on January 9. The magnetic resonance image of her brain prompted a diagnosis of reversible posterior leukoencephalopathy syndrome. After withdrawal of cyclosporine and control of hypertension, symptoms disappeared rapidly. Cyclophosphamide pulse therapy was successfully administrated to control lupus nephritis. This is the first report describing the relationship between Stevens-Johnson syndrome and mizoribine. Although the use of mizoribine is thought to be safe, careful observation is necessary.

Stevens-Johnson syndrome and toxic epidermal necrolysis in patients with lupus erythematosus: a descriptive study of 17 cases from a national registry and review of the literature

“…A total of 14 publications with SJS or TEN in the context of (S)LE have been published between 1961 and 2009 26–39 . Of these, 11 publications, including 12 patients and 14 episodes contained sufficient data for evaluation (Table 4).…”

“…Of these, 11 publications, including 12 patients and 14 episodes contained sufficient data for evaluation (Table 4). 26,28–36,39 All concerned female subjects, median age 29 years (range 9–76). The authors interpreted nine episodes as SJS and five as TEN.…”

“…However, the recurrent cases are far more suggestive for EMM than for SJS 26 . (iii) In two reports the presentation was based on clinical and histopathological findings more likely to be related to (drug‐induced) LE 35,36 …”

Stevens–Johnson syndrome in a juvenile systemic lupus erythematosus patient

“…3,[5][6][7] The efficacy of corticosteroids remains controversial. However, these drugs were also previously used in lupus patients concomitantly with IVIG.…”

“…3,4 This is a vesiculobullous disease of skin and mucosa with conjunctivitis and diffuse hemorrhagic blisters, generally with the presence of Nikolski´s sign. Of note, a few case reports have demonstrated SJS associated with adult SLE 5,6 and particularly in JSLE patients. 6,7 However, as far as we know, the prevalence of this manifestation in a pediatric lupus population has not been studied.…”