DRESS syndrome with thrombotic microangiopathy revealing a Noonan syndrome: Case report
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Mickaël Bobot1, Matteo Coen, Clémentine Simon
1Service of Nephrology and Renal Transplantation, La Conception University Hospital, Boulevard Baille, Marseille, France Department of Internal Medicine, Rehabilitation and Geriatrics, Service of Internal Medicine, Geneva University Hospitals, rue Gabrielle Perret-Gentil, Geneva, Switzerland Department of Pathology and Immunology, rue Michel Servet, Geneva, Switzerland Department of Radiology Department of Pathology Department of Cardiology, La Timone University Hospital, rue Saint Pierre Marseille, France.
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The anticoagulant was withdrawn, and corticosteroids (1 mg/kg/day) and acenocoumarol were started.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.
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