RARE CUTANEOUS ADVERSE REACTION TO ROUTINE IMMUNIZATIONS LEADING TO HYPER IGE SYNDROME (HIES) DIAGNOSIS

Abstract

<h3>Introduction</h3> While injection site reactions are common following immunizations, botryomycosis is an uncommon chronic granulomatous bacterial infection that develops in an area of prior injury, typically the skin. Hyper IgE Syndrome (HIES) is a rare primary immunodeficiency characterized by bacterial infections, elevated IgE and cutaneous manifestations; most commonly eczema, mucocutaneous candidiasis and cold abscesses. This case describes the development of a botryomycosis infection following routine two-month vaccinations, leading to HIES diagnosis. <h3>Case Description</h3> At 2 months of age, a male, previously healthy, term baby received routine immunizations to the anterior thigh without immediate adverse effect. Days later he developed small blisters at the prior injection sites, which were pruritic and progressed to large plaques. He was treated unsuccessfully with topical steroid and antifungal creams. At 4 months of age, a skin biopsy was advised which revealed botryomycosis with <i>Staphylococcus aureus</i> as the causative organism. This rare presentation prompted an immunodeficiency work-up, including blood count, immunoglobulins, and dihydrorhodamine testing. Labs revealed neutropenia (620-800 cells/microliter), IgE 1436 IU/mL, and DHR activity was 85% with a subpopulation of reduced activity. Genetic testing revealed a pathogenic mutation of STAT3, diagnostic of an autosomal dominant HIES. Botryomycosis infection resolved on trimethoprim-sulfamethoxazole. <h3>Discussion</h3> Although vaccination can cause benign local skin reactions, botryomycosis is a rare suppurative infection and to our knowledge, there have not been prior reports of botryomycosis diagnosis after vaccination. HIES and botryomycosis are both exceedingly rare and were described together only once. This case underscores that unusual infections with common pathogens are a hallmark of immunodeficiencies.