Journal article
DRESS syndrome with thrombotic microangiopathy revealing a Noonan syndrome: Case report.
- Bobot M Service of Nephrology and Renal Transplantation, La Conception University Hospital, Boulevard Baille, Marseille, France.
- Coen M Department of Internal Medicine, Rehabilitation and Geriatrics, Service of Internal Medicine, Geneva University Hospitals, rue Gabrielle Perret-Gentil, Geneva, Switzerland.
- Simon C Department of Radiology.
- Daniel L Department of Pathology.
- Habib G Department of Cardiology, La Timone University Hospital, rue Saint Pierre Marseille, France.
- Serratrice J Department of Internal Medicine, Rehabilitation and Geriatrics, Service of Internal Medicine, Geneva University Hospitals, rue Gabrielle Perret-Gentil, Geneva, Switzerland.
- 2018-04-12
Published in:
- Medicine. - 2018
Adult
Anticoagulants
Diagnosis, Differential
Drug Hypersensitivity Syndrome
Glucocorticoids
Humans
Lymphatic Abnormalities
Male
Mediastinum
Mutation
Noonan Syndrome
Phenindione
Retroperitoneal Space
Son of Sevenless Proteins
Thrombotic Microangiopathies
Withholding Treatment
English
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The anticoagulant was withdrawn, and corticosteroids (1 mg/kg/day) and acenocoumarol were started.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.
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.
- Language
-
- English
- Open access status
- gold
- Identifiers
-
- DOI 10.1097/MD.0000000000010297
- PMID 29642153
- Persistent URL
- https://sonar.ch/global/documents/232698
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