History
A 28-year-old female was admitted to hospital with viral encephalitis. During her illness she developed a saggital sinus thrombosis and resultant epilepsy. She was commenced on anticoagulants and phenytoin. Six weeks after her hospital admission she develops an itchy and painful rash on her face, trunk and limbs. At the time of referral to the dermatology team she has an ongoing fever, headache and general malaise. She has no previous medical history of note.
Examination
The patient’s face and ears are erythematous with marked oedema; elsewhere she has a widespread maculopapular eruption (Fig. 24.1). There is lym phadenopathy in the cervical, axillary and inguinal regions. Mucous membranes are normal and no blis-ters are seen. Her temperature is 39.1 °C.
Questions
• What is the dermatological diagnosis?
• What management would you initiate?
Medicine
This patient was diagnosed with a drug hypersensitivity syndrome called DRESS (drugrash with eosinophilia and systemic symptoms). The symptoms and signs of DRESS may be misdiagnosed as an ongoing infection as patients having a swinging tempera-ture, malaise, lymphadenopathy, a skin rash and eosinophilia. The systemic part of the syndrome can manifest with internal organ involvement most commonly liver, lung or kidneys. The mortality rate is around 10 per cent.
The cutaneous manifestations of DRESS can vary quite widely. However, most patients have quite erythematous and oedematous skin especially in the head and neck region.
Patients may present with marked oedema around their eyes and ears. The morphology of the widespread skin eruption may be macular, papular, coalescing plaques, occasionally vesicular or pustular lesions, and there may be areas of desquamation. Mucous membranes are spared. The rash may be mildly itchy, the skin may feel tight and uncomfortable. Marked reactive lymphadenopathy is usually present in the cervical and axillary lymph node basins. The patient’s fever is usually marked and swinging. Patients look and feel unwell. Eosinophilia and raised liver function tests are commonly seen but may lag behind the onset of the skin eruption. The most common drugs implicated in DRESS include antibiotics and anticonvulsants. DRESS usually occurs within 8–12 weeks after commencing the culprit drug. The pheny-toin was the drug implicated in this case and was therefore stopped immediately. The patient was treated with pulsed intravenous methyl prednisolone (1 g daily for 3 days) and then a tapering course of oral prednisolone starting at 40 mg daily and reducing down slowly over 6 weeks.
There is rapid normalization of the patient’s temperature and eosinophil count with com-mencement of systemic steroids. The hepatitis may not settle for several weeks, and may even deteriorate further before settling. In severe cases of reactive hepatitis in DRESS patients may eventually require liver transplantation due to the acute liver damage.
KEY POINTS
• DRESS can often present with clinical signs and symptoms that may be misdiagnosed as an infection.
• A lag of 8–12 weeks occurs between starting the culprit medication and the onset of DRESS.
• DRESS has a 10 per cent mortality rate; the culprit drug should be stopped immediately and systemic steroids given.
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