Table 1.

Common infections in STAT3-HIES

Infection typeTypical pathogensClinical featuresApproximate frequency in STAT3-HIESRecommended treatment options
Skin abscesses/cellulitis S. aureus (often methicillin-susceptible S. aureus; sometimes MRSA) Cold abscesses with minimal erythema/warmth; recurrent furunculosis ∼50–80% (20, 21) • Pathogen identification and antimicrobial susceptibility testing 
• Prompt systemic antibiotics according to local resistance 
• Surgical drainage 
• Initiation of prophylactic antibiotics in patients with recurrent skin abscesses/cellulitis 
Eczema-associated superinfection S. aureus, β-hemolytic streptococci Impetiginization of chronic eczema; crusted lesions Very common (most have secondary bacterial infection of eczema at some point) (22) • Pathogen identification and antimicrobial susceptibility testing 
• Systemic or topical antibiotics plus anti-inflammatory eczema management and antiseptic baths 
• Consider prophylactic antibiotics in patients with recurrent eczema-associated superinfection 
Fungal skin infections Candida spp., dermatophytes Intertrigo, onychomycosis, tinea infections Common, however, less systematically reported (22) • Topical azoles or allylamides for limited infections 
• Oral antifungal agents for extensive, recalcitrant, or nail involvement 
Recurrent pneumonia S. aureus, Streptococcus pneumoniae, Haemophilus influenzae, respiratory viruses Recurrent pneumonias from infancy; often progress to bronchiectasis/pneumatoceles ∼80–90% ever; >60% with ≥2 pneumonias (8, 22, 23) • Pathogen identification and antimicrobial susceptibility testing 
• Early, culture-guided systemic antibiotics 
• Consider antibiotic prophylaxis and/or IGRT 
Chronic lung infection S. aureus, P. aeruginosa, Aspergillus fumigatus Structural lung disease (bronchiectasis, pneumatoceles); chronic productive cough Structural changes are present in ∼80–95% adults; chronic bacterial/fungal colonization is common (22) • Prolonged, culture-guided systemic or inhaled antimicrobials when appropriate 
• Intensive airway-clearance strategies 
• Multidisciplinary care with involvement of pulmonology 
• Consider referral to surgery for complications, e.g., pneumatoceles 
CMC (oral, genital, nails) Candida albicans and other Candida spp. Recurrent oral thrush, angular cheilitis, vulvovaginal candidiasis, onychomycosis ∼50–85% across cohorts (21, 22) • Pathogen identification and antimicrobial susceptibility testing 
• Topical azole or nystatin therapy for limited infections 
• Systemic fluconazole (or alternate azole based on species and resistance) for refractory infection 
• Consider long-term prophylaxis with systemic antifungal agent (e.g., fluconazole) 
Severe disseminated infections (sepsis, deep abscesses) Mainly S. aureus; occasionally gram-negatives Invasive infections often from skin/lung focus Less common; however, major cause of morbidity/mortality (22, 24) • Urgent medical treatment, including broad-spectrum intravenous antibiotics with prompt de-escalation according to cultures and source 
• Aggressive source control (e.g., drainage of abscesses) 
• Review long-term prophylaxis 
• Consider IGRT 
• Consideration of HSCT in selected patients with severe, refractory infectious complications 

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