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Eosinophilic pustular folliculitis in infancy
Other Resources UpToDate PubMed

Eosinophilic pustular folliculitis in infancy

Contributors: Craig N. Burkhart MD, Dean Morrell MD, Lowell A. Goldsmith MD, MPH
Other Resources UpToDate PubMed

Synopsis

Eosinophilic pustular folliculitis in infancy (EPFI) is a rare self-limited disorder of unknown cause. The pustules seen are sterile. Some question whether it is merely an infantile variant of adult eosinophilic pustular folliculitis (Ofuji disease), but the course and clinical presentation differs, with involvement primarily of the scalp and brow. About 40 cases have been reported, usually appearing within the first days to weeks of life. One human immunodeficiency virus (HIV)-related case was reported at age 3 months. All cases reported began in infants younger than 1 year, but lesions occurred for 3 months to 5 years before resolving.

Related topics: Ofuji disease, Immunosuppression-associated eosinophilic folliculitis

Codes

ICD10CM:
L73.8 – Other specified follicular disorders

SNOMEDCT:
403845000 – Neonatal eosinophilic pustular folliculitis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Erythema toxicum neonatorum starts within the first 2 days of life and resolves within 1 week.
  • Transient neonatal pustular melanosis occurs at birth in infants with dark skin phototypes on the face, neck, and shins.
  • Acropustulosis of infancy occurs in male infants with dark skin phototypes aged 3-6 months, most often on the hands and feet.
  • Langerhans cell histiocytosis lesions are seen in the scalp and flexural areas. A biopsy is diagnostic.
  • Neonatal herpes simplex virus
  • Bullous impetigo
  • Folliculitis
  • Candidiasis
  • Scabies
  • Hyperimmunoglobulinemia E syndrome

Best Tests

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Management Pearls

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Therapy

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References

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Last Updated:05/19/2019
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Eosinophilic pustular folliculitis in infancy
A medical illustration showing key findings of Eosinophilic pustular folliculitis in infancy : Crust, Erythema, Eyebrow, Folliculitis, Pustule, Scalp, Trunk, Cheeks
Clinical image of Eosinophilic pustular folliculitis in infancy - imageId=1836643. Click to open in gallery.  caption: 'Numerous tiny papules and crusts on the forehead.'
Numerous tiny papules and crusts on the forehead.
Copyright © 2024 VisualDx®. All rights reserved.