Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS): Is cutaneous phenotype a prognostic marker for outcome? A review of clinicopathological features of 27 cases.

Introduction: Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) describes a heterogenous group of severe adverse reactions to medications. The cutaneous phenotype has a number of guises, accompanied by a variety of systemic features including fever, haematological abnormalities and visceral involvement, most commonly the liver. Clinical markers of prognosis have not been identified.<br> Methods: We reviewed the clinical features, dermatopathology and outcomes of 27 consecutive cases of DRESS presenting to a single unit. Results: 4 distinct patterns of cutaneous involvement were identified: an urticated papular exanthem (13/27 patients), a morbilliform erythema (3/27), an exfoliative erythroderma (3/27) and an erythema multiforme-like (EM-like) reaction consisting of atypical targets (8/27). All patients mounted a fever, most developed lymphadenopathy (24/27) and a peripheral eosinophilia (25/27) and the most common organ involved was the liver (26/27). Review of dermatopathic features of patients with DRESS demonstrated a superficial spongiotic dermatitis in the majority of cases (16/27). A smaller number of cases showed basal cell vacuolar degeneration and necrotic keratinocytes (9/27). These patients with these biopsy findings more commonly had an erythema multiforme like cutaneous phenotype, and more severe hepatic involvement. 3 patients died, 2 following failed liver transplants. Conclusions: Our series is the first in which a detailed dermatological assessment has been made of consecutive patients presenting with DRESS, and the largest UK series to date. Our results suggest a possible prognostic role of the cutaneous and dermatopathic findings in DRESS in predicting the severity of visceral involvement in this syndrome.