Eosinophilic Pustular Folliculitis in Newborns
Eosinophilic Pustular Folliculitis
Definition
Eosinophilic pustular folliculitis (EPF) is a rare inflammatory dermatosis characterized by recurrent crops of sterile follicular pustules with significant tissue eosinophilia.
Key Points
- First described by Ofuji et al. in 1970 in Japan
- Three distinct variants are recognized:
- Classic Ofuji's disease
- Immunosuppression-associated EPF
- Infancy-associated EPF
- Peak age of onset: 3-8 months in infantile cases
- Prevalence: Higher in Asian populations
- Gender distribution: Male predominance in adult cases
Clinical Features
Primary Manifestations
- Morphology:
- Sterile follicular pustules
- Erythematous papules
- Annular plaques with peripheral progression
- Crusted erosions
- Distribution:
- Scalp (most common in infants)
- Face and neck
- Upper trunk
- Proximal extremities
Associated Symptoms
- Intense pruritus (hallmark symptom)
- Burning sensation
- Tenderness
- Occasional fever and malaise
Disease Course
- Cyclic nature with flares every 3-4 weeks
- Spontaneous resolution of individual lesions in 7-14 days
- Post-inflammatory hyperpigmentation common
- May persist for months to years without treatment
Pathophysiology
Immunological Mechanisms
- Complex immune dysregulation involving:
- Increased IL-5 production
- Elevated prostaglandin D2 levels
- Enhanced chemotaxis of eosinophils
- Aberrant T-helper 2 response
- Key cellular components:
- Activated eosinophils
- CD4+ T lymphocytes
- Langerhans cells
Histopathological Features
- Early lesions:
- Follicular spongiosis
- Perifollicular inflammation
- Eosinophilic infiltration
- Established lesions:
- Dense eosinophilic infiltrate
- Follicular mucinosis
- Sebaceous gland involvement
Disease Variants
1. Classic Ofuji's Disease
- Demographics:
- Young adults
- Asian predominance
- Male > Female
- Characteristics:
- Follicular pustules in circular arrangement
- Peripheral spreading pattern
- Face, trunk, and proximal extremities
2. Immunosuppression-associated EPF
- Associated conditions:
- HIV/AIDS (most common)
- Hematologic malignancies
- Post-transplant
- Features:
- More extensive involvement
- Treatment-resistant
- May improve with immune reconstitution
3. Infancy-associated EPF
- Characteristics:
- Onset: 3-8 months of age
- Scalp predominance
- Better prognosis
- Self-limiting course
Diagnosis & Workup
Clinical Diagnosis
- History:
- Age of onset
- Pattern of recurrence
- Associated symptoms
- Immune status
- Physical examination:
- Distribution pattern
- Morphology of lesions
- Associated findings
Laboratory Studies
- Essential tests:
- Complete blood count with differential
- Peripheral eosinophil count
- HIV testing when appropriate
- Bacterial and fungal cultures
- Histopathology:
- Skin biopsy (punch or incisional)
- Special stains
- Direct immunofluorescence
Differential Diagnosis
- Infectious:
- Bacterial folliculitis
- Tinea capitis
- Herpes simplex infection
- Inflammatory:
- Atopic dermatitis
- Seborrheic dermatitis
- Follicular mucinosis
- Langerhans cell histiocytosis
Treatment
First-line Therapies
- Topical treatments:
- High-potency corticosteroids
- Tacrolimus 0.1% ointment
- Pimecrolimus 1% cream
- Systemic therapy:
- Indomethacin (most effective)
- Cetirizine or other antihistamines
- Short course oral steroids for severe flares
Second-line Options
- Systemic treatments:
- Dapsone
- Cyclosporine
- Minocycline
- IVIG in resistant cases
- Phototherapy:
- Narrowband UVB
- PUVA for resistant cases
Supportive Care
- Gentle skin care routine
- Prevention of secondary infection
- Management of pruritus
- Treatment of underlying conditions in secondary EPF
Complications & Prognosis
Potential Complications
- Physical:
- Secondary bacterial infection
- Post-inflammatory hyperpigmentation
- Scarring in severe cases
- Psychosocial:
- Quality of life impact
- Social anxiety
- Depression
Prognosis
- Infantile EPF:
- Generally self-limiting
- Resolution by age 3 years
- Excellent response to treatment
- Classic EPF:
- Chronic course
- Regular monitoring needed
- Good response to indomethacin
- Immunosuppression-associated:
- More challenging to treat
- May improve with immune restoration
- Requires long-term management
Disclaimer
The notes provided on Pediatime are generated from online resources and AI sources and have been carefully checked for accuracy. However, these notes are not intended to replace standard textbooks. They are designed to serve as a quick review and revision tool for medical students and professionals, and to aid in theory exam preparation. For comprehensive learning, please refer to recommended textbooks and guidelines.
