DRESS Syndrome With Autoimmune Hepatitis From Strontium Ranelate
Strontium ranelate, which is used for postmenopausal osteoporosis, has been associated with drug rash with eosinophilia and systemic symptoms (DRESS) syndrome, a severe, acute, potentially fatal, multisystem adverse drug reaction characterized by skin rash, fever, hematological abnormalities, and lymphadenopathy with involvement of several internal organs. We report the case of a woman who developed DRESS syndrome with a generalized maculopapular rash, eosinophilia, dyspnea, bilateral cervical lymphadenopathy, and reactivation of Epstein-Barr virus (EBV) with liver damage 3 weeks after administration of strontium ranelate for postmenopausal osteoporosis. Approximately 6 months after total remission of cutaneous symptoms, the patient developed autoimmune hepatitis. This case confirms that strontium ranelate should be considered as a possible factor in the etiopathology of DRESS syndrome as well as in the subsequent development of autoimmune hepatitis. The possibility of developing autoimmune hepatitis as a part of DRESS syndrome related to strontium ranelate use can occur months after the acute episode.
Practice Points
- Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome refers to a severe, acute, potentially fatal, multisystem adverse drug reaction characterized by skin rash, fever, hematological abnormalities, and lymphadenopathy with involvement of several internal organs.
- Strontium ranelate should be considered as a possible factor in the etiopathology of DRESS syndrome and in the development of autoimmune hepatitis as a part of DRESS syndrome.
The total RegiSCAR score of 8 in our case was taken as a definite indication of DRESS syndrome (temperature, 38.5°C [0 points]; enlarged lymph nodes [1 point]; eosinophilia, ≥20% or >1500 μL [2 points]; skin involvement with >50% body surface area involved [1 point] with a maculopapular rash [1 point] and histopathologic findings suggesting DRESS syndrome [1 point]; lung and liver involvement [2 points]). The causative drug was identified by carefully collecting the patient’s medication history and by evaluating clinical outcome characterized by improved skin and systemic symptoms after discontinuation of strontium ranelate.
Because of the high morbidity of DRESS syndrome, it needs to be diagnosed effectively and must be considered in the differential for any patient developing the triad of skin rash, hypereosinophilia, and systemic symptoms, as well as several other side effects when taking strontium ranelate.10
Therapies for DRESS Syndrome
Treatment of DRESS syndrome has not yet been standardized. Prompt withdrawal of the causative drug is the only mandatory activity in the treatment of DRESS syndrome. Systemic corticosteroids may be needed for organ or life-threatening disease, though the efficacy is controversial because it may result in activation of HHV-6, which in turn is probably involved in the pathogenesis of DRESS syndrome.
Conclusion
This case confirms that strontium ranelate should be considered a possible factor in the etiopathology of DRESS syndrome and in the development of autoimmune hepatitis as a part of DRESS syndrome. Case reports underline the importance of recognition of cutaneous adverse reactions in patients undergoing treatment of postmenopausal osteoporosis. The prognosis is good with immediate recognition followed by immediate and permanent withdrawal of the drug, along with hospitalization and systemic corticosteroids when necessary. The possibility of developing autoimmune hepatitis as a part of DRESS syndrome related to strontium ranelate has been reported,11 usually months after the acute episode.
