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Chronic omphalitis in a 4-month-old girl
1Groupe de Pathologie Pediatrique, Hôpital des Enfants Malades, Paris, France.
Pathology, Research and Practice
|March 1, 1991
Summary
A rare case of chronic omphalitis in an infant revealed a key diagnostic clue: scanty inflammatory cells despite high peripheral leucocyte counts. This finding suggests Deficiency in Leukocyte Adhesion Molecules (DLAM), an uncommon inherited disorder.
Area of Science:
- Immunology
- Pediatrics
- Genetics
Background:
- Chronic omphalitis can present from birth, posing diagnostic challenges.
- Distinguishing inflammatory conditions from congenital anomalies is crucial in neonates.
Observation:
- A 4-month-old girl presented with chronic omphalitis since birth.
- Biopsy showed edematous tissue with surprisingly few inflammatory cells.
- Peripheral blood leucocytes were significantly elevated (>30,000/microliter).
Findings:
- The discrepancy between local scant inflammatory infiltrate and high peripheral leucocyte count suggested an underlying immune defect.
- Deficiency in Leukocyte Adhesion Molecules (DLAM) was suspected.
- Immunocytochemical analysis using anti-CD11a, CD11b, and CD18 antibodies is required for confirmation.
Implications:
- This case highlights DLAM as a rare cause of omphalitis.
- Early diagnosis of DLAM is vital for managing this autosomal recessive inherited disorder.
- Prompt immunocytochemical testing can confirm DLAM, enabling appropriate patient care.
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