DRESS syndrome with thrombotic microangiopathy revealing a Noonan syndrome: Case report.

RATIONALE

The life-threatening drug rash with eosinophilia and systemic symptoms (DRESS) syndrome occurs most commonly after exposure to drugs, clinical features mimic those found with other serious systemic disorders. It is rarely associated with thrombotic microangiopathy.

PATIENT CONCERNS

We describe the unique case of a 44-year-old man who simultaneously experienced DRESS syndrome with thrombotic microangiopathy (TMA) after a 5 days treatment with fluindione.

DIAGNOSES

Clinical evaluation leads to the discovery of an underlying lymphangiomatosis, due to a Noonan syndrome.

INTERVETIONS

The anticoagulant was withdrawn, and corticosteroids (1 mg/kg/day) and acenocoumarol were started.

OUTCOMES

Clinical improvement ensued. At follow-up the patient is well.

LESSONS

The association of DRESS with TMA is a rare condition; we believe that the presence of the underlying Noonan syndrome could have been the trigger. Moreover, we speculate about the potential interrelations between these entities.