Discriminating minor and major forms of drug reaction with eosinophilia and systemic symptoms: Facial edema aligns to the severe phenotype

Abstract

Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a cutaneous and systemic drug allergy disorder. Patients exist on a severity spectrum, with some experiencing a mild form of the disorder that fails to meet the Registry of Severe Cutaneous Adverse Reactions (SCAR) to Drugs diagnostic criteria for DRESS.

Objective: We sought to determine whether there were any cutaneous or dermatopathologic features that discriminate between the mild form of DRESS (DRESS minor) and the severe phenotype (DRESS major).

Methods: Hospitalized patients from a single center with a diagnosis of DRESS were prospectively recruited over a 7-year period. Clinical and dermatopathologic features were analyzed to discriminate between DRESS minor and DRESS major.

Results: Forty-five patients were included, of whom 19 had a Registry of Severe Cutaneous Adverse Reactions (SCAR) to Drugs score of ≤3 (DRESS minor) and 26 had a score of ≥4 (DRESS major). The mean latency period (P = .001), fever >38.5 °C (P = .001), and a reaction lasting >15 days (P = .010) discriminated DRESS major from DRESS minor. Facial edema was the sole discerning cutaneous feature (P = .025). Discriminating histopathologic features included basal squamatization (P = .005), dermal red blood cell extravasation (P = .009), and interface inflammation (P = .005).

Conclusion: We propose a new classification system-DRESS minor-to distinguish the milder illness from the severe form, DRESS major. Facial edema and certain histopathologic features can help discriminate between major and minor versions.

Keywords: DRESS; adverse reactions; drug rash; histopathology; medical dermatology; severe cutaneous.