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Cutaneous pustular leukemoid reactions in trisomy 21
Joanna M Burch1, William L Weston, Maureen Rogers
1Department of Pediatrics, University of Colorado Health Sciences Center, Denver, Colorado, USA.
Pediatric Dermatology
|June 6, 2003
Summary
Neonates with Down syndrome can develop pustular skin eruptions due to leukemoid reactions. Early identification and management of transient myeloproliferative disorders are crucial for these infants.
Area of Science:
- Neonatology
- Dermatology
- Hematology
Background:
- Down syndrome (trisomy 21) is associated with an increased risk of hematologic abnormalities.
- Leukemoid reactions, characterized by markedly elevated white blood cell counts, can occur in neonates.
Observation:
- Two neonates with Down syndrome presented with pustular skin eruptions and significantly high white blood cell counts at birth.
- Skin biopsies revealed intraepidermal pustules with atypical mononuclear cells, suggesting an underlying myeloid or T-cell process.
Findings:
- Viral and bacterial cultures were negative, ruling out common infectious causes for the pustular rash.
- One patient's pustules and high white blood cell count resolved with cytarabine (ara-C) therapy, while the other improved with topical corticosteroids as white blood cell counts decreased.
- Immunohistochemistry confirmed myeloid lineage in one case and T-cell lineage in the other.
Implications:
- Transient myeloproliferative disorders (TMPD) should be strongly considered in newborns with Down syndrome presenting with pustular eruptions.
- Recognizing this association can guide appropriate diagnostic workup and management, potentially preventing misdiagnosis and unnecessary treatments.
- Further research into the pathogenesis and optimal management of TMPD-associated skin manifestations in Down syndrome is warranted.