Drug reaction with eosinophilia and systemic symptoms: is cutaneous phenotype a prognostic marker for outcome? A review of clinicopathological features of 27 cases

TY - JOUR

T1 - Drug reaction with eosinophilia and systemic symptoms: is cutaneous phenotype a prognostic marker for outcome?

T2 - A review of clinicopathological features of 27 cases

AU - Walsh, S.

AU - Diaz-Cano, Salvador

AU - Higgins, Elisabeth

AU - Morris-Jones, Rachael

AU - Bashir, Saqib

AU - Bernal, W.

AU - Creamer, Daniel

PY - 2013/2

Y1 - 2013/2

N2 - Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) describes a heterogeneous 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. Objectives: To assess the cutaneous signs and dermatopathological features of DRESS in order to identify potential prognostic markers. Methods: We reviewed the clinical features, dermatopathology and outcomes of 27 consecutive cases of DRESS presenting to a single unit. Results: Four distinct patterns of cutaneous involvement were identified: an urticated papular exanthem (13/27 patients), a morbilliform erythema (three of 27), an exfoliative erythroderma (three of 27) and an erythema multiforme-like (EM-like) reaction consisting of atypical targets (eight of 27). All patients mounted a fever, most developed lymphadenopathy (24/27) and peripheral eosinophilia (25/27) and the most common organ involved was the liver (27/27). Review of the 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 (nine of 27). The patients with these biopsy findings more commonly had an EM-like cutaneous phenotype, and more severe hepatic involvement. Three patients died, two 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 U. K. 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.

AB - Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) describes a heterogeneous 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. Objectives: To assess the cutaneous signs and dermatopathological features of DRESS in order to identify potential prognostic markers. Methods: We reviewed the clinical features, dermatopathology and outcomes of 27 consecutive cases of DRESS presenting to a single unit. Results: Four distinct patterns of cutaneous involvement were identified: an urticated papular exanthem (13/27 patients), a morbilliform erythema (three of 27), an exfoliative erythroderma (three of 27) and an erythema multiforme-like (EM-like) reaction consisting of atypical targets (eight of 27). All patients mounted a fever, most developed lymphadenopathy (24/27) and peripheral eosinophilia (25/27) and the most common organ involved was the liver (27/27). Review of the 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 (nine of 27). The patients with these biopsy findings more commonly had an EM-like cutaneous phenotype, and more severe hepatic involvement. Three patients died, two 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 U. K. 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.

KW - TOXIC EPIDERMAL NECROLYSIS

KW - HYPERSENSITIVITY-SYNDROME

KW - DRESS SYNDROME

KW - SEVERITY

KW - HISTOPATHOLOGY

KW - FAILURE

KW - RASH

U2 - 10.1111/bjd.12081

DO - 10.1111/bjd.12081

M3 - Article

SN - 0007-0963

VL - 168

SP - 391

EP - 401

JO - British Journal of Dermatology

JF - British Journal of Dermatology

IS - 2

ER -