Stevens-Johnson Syndrome Associated with Daclizumab Use- Report of Two Cases

Background: Rash has been reported in 11% of patients using Daclizumab (DAC). Autoimmune disorders including liver failure associated with autoimmune hepatitis have been reported.  There are no reports of Stevens-Johnson Syndrome (SJS), however, "autoimmune allergic" dermatologic conditions have occurred with DAC. SJS together with toxic epidermal necrolysis (TEN) form a spectrum of dermatologic disease. We now report two cases of SJS, one of which more appropriately simulated TEN, in multiple sclerosis (MS) patients taking DAC.

Objectives: To report a potentially lethal systemic dermatologic complication associated with DAC use.

Methods: Two cases of SJS associated with the use of DAC were evaluated.

Results: Case1. A 52 year old black woman with MS complained of flu-like symptoms and had low grade pyrexia after 5 injections of DAC. She was taking no other medications. REMS blood work was normal up until the event. Her myelopathy worsened and EDSS score deteriorated from 2.5 to 4.5. She developed a confluent macular rash over her face, torso, back, and limbs. The rash was pruritic. After several days the patient developed pronounced alopecia and blister-like peeling lesions on her skin that formed raw areas with intense pain. Cystic lesions were also present in the oropharynx.  Fever persisted with temperature spikes to 103 degrees, and WBC count rose to 18.6 with shift and a sedimentation rate of 84. She declined hospitalization, but was treated with high dose steroids and antihistamines. Over several weeks, blood labs normalized with resolution of the leukocytosis. The patient's skin exfoliated and alopecia gradually resolved over weeks. No super infections occurred. Several skin biopsies were performed and revealed spongiotic and interface dermatitis with histologic features consistent with drug reaction. Case 2. After 6 months of DAC, a 68 year old Caucasian man developed the insidious onset of a diffuse macular rash associated with pruritis, which appeared initially to be in a photo sensitive skin area but eventually generalized. REMS blood work was normal up until the event. He was initially treated with antihistamines. Over several days the rash appeared to be generalizing and eventually involved at least 30% of his body.  Repeated temperature spikes of up to 103 degrees were recorded. Over several days the macular rash evolved into intense painful blisters with induration. Headache, malaise, and arthralgia were noted.  WBC count was initially 23.5 with shift and sedimentation rate was 77. He was hospitalized and treated aggressively with IV steroids and antihistamines. His skin eventually exfoliated. Skin biopsy had revealed epidermal necrosis with minimal inflammatory change. After a period of several weeks, the patient improved.  

Conclusions: This is the first report of SJS associated with DAC use. The cases suggest that vigilance is mandatory when using DAC since more serious and potentially lethal dermatologic complications can occur.