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Neonatal onset multisystem inflammatory disease

A Huttenlocher1, I J Frieden, H Emery

  • 1Department of Pediatrics, University of California, San Francisco, USA.

The Journal of Rheumatology
|June 1, 1995
PubMed
Summary
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Neonatal onset multisystem inflammatory disease (NOMID) presents with varied symptoms. This case highlights neutrophilic eccrine hidradenitis as a potential diagnostic clue for this rare inflammatory disorder.

Area of Science:

  • Pediatric Rheumatology
  • Dermatology
  • Neurology

Background:

  • Neonatal onset multisystem inflammatory disease (NOMID) is a rare autoinflammatory disorder.
  • It typically involves a triad of chronic infantile neurological, cutaneous, and articular syndrome (CINCA) or NOMID, characterized by arthropathy, rash, and central nervous system (CNS) involvement.

Observation:

  • A case of NOMID is presented in a female infant with typical neonatal rash, arthropathy, fever, and failure to thrive.
  • Notably, this patient has not yet developed ocular or CNS manifestations, expanding the known clinical spectrum of NOMID.

Findings:

  • Histopathological examination of the skin revealed neutrophilic eccrine hidradenitis.
  • This finding is considered unique and potentially serves as an important diagnostic clue.

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Implications:

  • This case underscores the variability in clinical presentation of NOMID.
  • Identifying neutrophilic eccrine hidradenitis may aid in earlier diagnosis of NOMID, even in the absence of classic CNS or ocular findings.