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Degos disease: a new simulator of non-accidental injury
Celia Moss1, Evangeline Wassmer, Geoff Debelle
1Department of Dermatology, Birmingham Children's Hospital, Birmingham, UK. celia.moss@bch.nhs.uk
Insights
Degos disease, a rare vasculopathy, can mimic non-accidental injury in infants presenting with subdural fluid collections and skin lesions. This case highlights the importance of considering rare conditions in differential diagnoses.
Area of Science:
- Pediatrics
- Neurology
- Dermatology
Background:
- Non-accidental injury (NAI) is a significant concern in pediatric cases, often presenting with subdural fluid collections in non-mobile infants.
- Accurate diagnosis is critical due to the severe implications of missed or incorrect NAI diagnoses.
Observation:
- A 6-month-old male presented with bilateral subdural fluid collections and skin ulcers resembling cigarette burns.
- Initial presentation suggested non-accidental injury as the primary diagnosis.
- The infant's neurological condition deteriorated, with progressive cerebral infarctions and non-healing skin lesions.
Findings:
- Histological confirmation revealed Degos disease, an extremely rare and often fatal occlusive vasculopathy.
- Degos disease presented as a simulator of non-accidental injury in this pediatric case.
- The patient received palliative treatment and died 8 weeks post-presentation.
Implications:
- This case underscores the necessity for clinicians to consider Degos disease in the differential diagnosis of infants with unexplained subdural fluid collections and suspicious skin findings.
- Recognizing this rare condition can prevent misdiagnosis of non-accidental injury and guide appropriate patient management.
- Highlights the diagnostic challenges in pediatric neurology and rare diseases.
Abstract:
Recent high-profile cases have made paediatricians very aware of the serious implications of either missing or wrongly diagnosing non-accidental injury. Subdural fluid collections in non-mobile infants usually represent haemorrhage caused by non-accidental injury. We report a 6-month-old male who presented to the Accident and Emergency Department of Birmingham Heartlands Hospital with bilateral subdural fluid collections and skin ulcers resembling cigarette burns. Non-accidental injury was considered to be the most likely diagnosis. However, while under observation in hospital, the child's neurological condition deteriorated with progressive cerebral infarctions, and serial photographs of the skin lesions showed failure to heal. The revised diagnosis, confirmed histologically, was Degos disease, an extremely rare and often fatal occlusive vasculopathy. The child was treated palliatively and died 8 weeks after presentation. This report informs doctors of a new simulator of non-accidental injury to be considered in infants with otherwise unexplained subdural fluid collections.
