Table 1. Common infections in STAT3-HIES... | Rockefeller University Press

Table 1.

Common infections in STAT3-HIES

Infection type	Typical pathogens	Clinical features	Approximate frequency in STAT3-HIES	Recommended 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
Fungal skin infections	Candida spp., dermatophytes	Intertrigo, onychomycosis, tinea infections	Common, however, less systematically reported (22)	• 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

Infection type	Typical pathogens	Clinical features	Approximate frequency in STAT3-HIES	Recommended 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
Fungal skin infections	Candida spp., dermatophytes	Intertrigo, onychomycosis, tinea infections	Common, however, less systematically reported (22)	• 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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