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Published on: July 24, 2016
Vesiculopustular eruptions in Down syndrome neonates with myeloproliferative disorders
A Nijhawan1, E Baselga, M A Gonzalez-Ensenat
1Department of Dermatology, Medical College of Wisconsin, 9200 W Wisconsin Ave, Milwaukee, WI 53226, USA.
Insights
Infants with Down syndrome experiencing myeloproliferative disorders may develop a unique skin rash. This self-resolving eruption presents distinct clinical and histopathologic features, potentially aiding diagnosis.
Area of Science:
- Neonatal Dermatology
- Pediatric Hematology
- Clinical Genetics
Background:
- Infants with Down syndrome (DS) have a higher incidence of hematologic abnormalities, including transient myeloproliferative disorder (TMD) and congenital leukemia.
- The differential diagnosis for vesiculopustular eruptions in newborns with DS and hematologic issues is extensive, encompassing both benign and severe conditions.
Observation:
- This study details 3 newborns with DS presenting with vesiculopustular eruptions linked to neonatal myeloproliferative disorders.
- These skin lesions exhibit a distinct distribution and pathergy, containing immature hematopoietic cells resembling circulating blast cells.
Findings:
- The described cutaneous eruption in infants with DS and hematologic abnormalities possesses unique clinical and histopathologic characteristics.
- Resolution of the rash correlates with the subsidence of the underlying hematologic disorder, often occurring without specific treatment.
Implications:
- Recognizing this specific eruption may improve diagnostic accuracy in newborns with Down syndrome and hematologic abnormalities.
- Further research is needed to determine if this cutaneous manifestation serves as a prognostic indicator for the future development of leukemia in these infants.
Background:
Infants with Down syndrome are at increased risk for hematologic abnormalities, including leukemoid reaction, transient myeloproliferative disorder, and congenital leukemia. The differential diagnosis of a vesiculopustular eruption in an infant with Down syndrome and these hematologic abnormalities is broad and includes benign, self-limited disorders as well as life-threatening infections.
Observation:
We describe 3 newborns with Down syndrome and vesiculopustular eruptions associated with myeloproliferative disorders during the neonatal period. These lesions differ from other neonatal vesicular eruptions in that they have a unique distribution, display pathergy, and contain immature hematopoietic cells similar to circulating blast cells. Resolution occurs without treatment as the hematologic disorder subsides.
Conclusions:
Infants with Down syndrome and hematologic abnormalities may have a cutaneous eruption that has characteristic clinical and histopathologic findings. It is possible that this eruption has been unrecognized in the past because of its self-limited course. Whether this eruption is a prognostic factor for the subsequent development of leukemia is uncertain.
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