Episodic angioedema associated with eosinophilia

Area of Science:

• Pediatric Endocrinology
• Hematology
• Immunology

Background:

• Recurrent angioedema and significant weight gain in a pediatric patient over five years.
• Investigation for underlying causes of persistent symptoms.

Observation:

• Episodes characterized by elevated white blood cell count with marked eosinophilia (89.9%).
• Elevated serum levels of Interleukin-5 (IL-5), Immunoglobulin E (IgE), Immunoglobulin M (IgM), and Lactate Dehydrogenase (LDH).
• Histopathological findings revealed perivascular and diffuse dermal eosinophilic infiltration.

Findings:

• Exclusion of secondary causes of hypereosinophilia and organ infiltration.
• Negative testing for FIP1L1/PDGFRa and ETV6/PDGFRb fusion genes, ruling out specific neoplasms.
• Successful treatment with methylprednisolone and subsequent oral prednisolone taper.

Implications:

• Demonstrates the effectiveness of corticosteroid therapy in managing severe hypereosinophilia presenting as angioedema.
• Highlights the importance of considering idiopathic hypereosinophilic syndrome in pediatric patients with unexplained angioedema and eosinophilia.
• Suggests a potential role for IL-5 in the pathogenesis of eosinophilic infiltration and angioedema in this case.