Cardiac Involvement in DRESS Syndrome

Introduction

Drug Hypersensitivity Syndrome (DHS), also known as DRESS (Drug Rash with Eosinophilia and Systemic Symptoms), is a severe systemic drug reaction, most commonly associated with anticonvulsants, Sulfonamides, Minocycline and Allopurinol.

Diagnosis of DHS may be difficult, as its manifestations can mimic infectious or collagen disorders.

It is defined by fever, skin rash, lymphadenopathy and internal organ involvement: hepatitis, nephritis, myositis and myocarditis, manifested 2-8 weeks after initiation of therapy. Its management includes: withdrawal of the culprit drug, and systemic steroids or immunoglobulin.

Cardiac involvement varies between 2-4%. Manifestations vary from no harm to fibrotic-stage cardiac syndrome. Therapy includes: drug discontinuation, suppression of immune reaction, first by corticosteroids and immunoglobulins, and ventricular assisted devices, in refractory cases.

Materials and Method

An 18 year old healthy young male was admitted to the Cardiology Department, with the diagnosis of suspected viral myocarditis, presented with fever, chest pain, dyspnea, nausea, vomiting, and rash. Physical examination revealed Tachycardia, and Lt sub-maxillary lymphadenopathy.

On ECG: Sinus tachycardia, ST elevation on V4-V6, flattened T wave in lead II, AVF. Laboratory results revealed elevated cardiac enzymes.

An Echocardiogram revealed mild inferior wall Hypokinesis. Detailed history revealed a treatment, in the last month, with Minocycline for Acne-Vulgaris.

The diagnosis of DRESS Syndrome with myocarditis, was reached. The offending drug was stopped, steroid therapy initiated, with good response. Skin biopsy revealed lymphocytic vasculitis. A month later, on follow up, he felt better, with no rash. A cardiac-echo study was normal.

Conclusion

It is recommended that patients with DRESS syndrome, in line with its high morbidity and mortality, should be screened for cardiac involvement. Prompt diagnosis, withdrawal of the offending drug, and treatment with immunosuppressants has been shown to decrease mortality.