Neonatal pustular facial rash
A previously healthy 2-week-old girl developed multiple, asymptomatic pustules on her scalp, forehead, eyelids, and upper cheeks. She has been growing and developing well with appropriate weight gain. What's the diagnosis?
Case
A previously healthy 2-week-old girl developed multiple, asymptomatic pustules on her scalp, forehead, eyelids, and upper cheeks. She has been growing and developing well with appropriate weight gain. What’s the Diagnosis?
Neonatal pustular facial rash | Image credit: Author provided
Diagnosis: Benign cephalic pustulosis
Etiology and clinical findings
Benign cephalic pustulosis, often referred to as neonatal acne, is a common condition in neonates that consists of papules and pustules typically appearing on the cheeks, chin, and forehead.1 It often presents in the first few weeks of life, particularly around the third week.2 On physical exam, children have otherwise normal findings. The pregnancies are usually uncomplicated.3
Some studies have suggested an association with Malassezia colonization.3,4 Malassezia can be found on neonates’ skin at one to three days after birth, and colonization increases after the first week of life.5 A proposed mechanism involves neonatal sebum production facilitating growth of lipophilic yeasts such as Malassezia.4 Other data, however, have found no significant correlation.5 Furthermore, while one study found an association between colonization and disease severity, it also found neonates with benign cephalic pustulosis who had negative Malassezia cultures.1 This conflicting evidence suggests that the development of this condition is likely multifactorial, relying on other contributors in addition to microbiological influences. Further research is necessary to delineate the pathophysiology of benign cephalic pustulosis.
On microscopic evaluation, benign cephalic pustulosis can show inflammatory cells, predominantly polymorphonuclear neutrophils; rarely, there may also be eosinophils, basophils, and lymphocytes.4 Histology may reveal hyperplastic sebaceous glands with keratin plugs.2
Differential diagnosis
Diagnosis of benign cephalic pustulosis is clinical. The differential includes other benign pustular dermatoses, papulopustular rashes, and more serious infectious etiologies. Benign pustular dermatoses include erythema toxicum neonatorum, transient neonatal pustular melanosis, eosinophilic pustular folliculitis, and acropustulosis of infancy. Erythema toxicum neonatorum is another common benign newborn skin condition which typically appears in the first few days of life but can occur up to a few weeks of age. It is characterized by erythematous papules and pustules with erythematous bases and is self-limiting. Smears with Wright stain show eosinophils. Transient neonatal pustular melanosis usually presents at birth or within the first few days of life. It consists of pustules with a non-erythematous base which crust and can result in hyperpigmented macules with a collarette of scale. Eosinophilic pustular folliculitis is a rare inflammatory condition consisting of papules and pustules localized to the scalp. It presents on average around six months of age and is more common in males.6 Biopsies reveal eosinophilic infiltrate, folliculitis, and interfollicular pustules.7 Acropustulosis of infancy is chronic and more commonly involves the palms and soles. Papulopustular rashes include miliaria, which results from obstruction of the eccrine glands and leads to vesicles, papules, and pustules; milia, which are papules occurring from epidermal inclusion cysts; and sebaceous gland hyperplasia, which are yellow macules and papules on the nose and cheeks of newborns.
Infectious causes on the differential include bacterial, viral, and fungal infections. Cultures may be obtained for patients with high clinical suspicion for possible infectious origins. Bacterial infections engendering pustular eruptions include Staphylococcus aureus, Streptococcus, Listeria, and syphilis. Viral causes include herpes simplex virus and varicella zoster virus, which have multinucleated giant cells on microscopy with Wright stain. Fungal infections include congenital candidiasis. Lastly, nodular acne is another entity which can present at several months of life and manifests as deep inflammatory nodules.8
Treatment
Management includes supportive care, such as regular cleansing with unscented soap and water. Children with more involvement may benefit from antifungal creams which can provide relief within a week of use.3,4 More severe cases can be treated with topical acne therapies. Otherwise, the rash is typically self-limiting, often resolving within a few months. After resolution, there is no scarring or increased incidence of acne later in childhood. Patients with severe or persisting symptoms may benefit from further evaluation such as endocrinological studies. Patients may also be referred to specialists including pediatric dermatologists and endocrinologists.
Clinical course
The patient was started on clotrimazole cream applied daily. She tolerated this treatment well without complications and the rash cleared in three weeks. On follow up, she continued to do well with no residual scarring.
References:
1.Bernier V, Weill FX, Hirigoyen V, et al. Skin colonization by Malassezia species in neonates: a prospective study and relationship with neonatal cephalic pustulosis. Arch Dermatol. Feb 2002;138(2):215-218. doi:10.1001/archderm.138.2.215
2.Katsambas AD, Katoulis AC, Stavropoulos P. Acne neonatorum: a study of 22 cases. Int J Dermatol. Feb 1999;38(2):128-130. doi:10.1046/j.1365-4362.1999.00638.x
3.Rapelanoro R, Mortureux P, Couprie B, Maleville J, Taïeb A. Neonatal Malassezia furfur pustulosis. Arch Dermatol. Feb 1996;132(2):190-193.
4.Niamba P, Weill FX, Sarlangue J, Labrèze C, Couprie B, Taïeh A. Is common neonatal cephalic pustulosis (neonatal acne) triggered by Malassezia sympodialis? Arch Dermatol. Aug 1998;134(8):995-998. doi:10.1001/archderm.134.8.995
5.Ayhan M, Sancak B, Karaduman A, Arikan S, Sahin S. Colonization of neonate skin by Malassezia species: relationship with neonatal cephalic pustulosis. J Am Acad Dermatol. Dec 2007;57(6):1012-1018. doi:10.1016/j.jaad.2007.02.030
6.Hernández-Martín Á, Nuño-González A, Colmenero I, Torrelo A. Eosinophilic pustular folliculitis of infancy: a series of 15 cases and review of the literature. J Am Acad Dermatol. Jan 2013;68(1):150-155. doi:10.1016/j.jaad.2012.05.025
7.Fertitta L, Bodemer C, Molina T, Frassati-Biaggi A, Fraitag S, Leclerc-Mercier S. Eosinophilic Pustular Folliculitis of Infancy: A Histologic Assessment of 43 Cases. Am J Dermatopathol. Jun 1 2022;44(6):395-403. doi:10.1097/dad.0000000000002006
8.Cunliffe WJ, Baron SE, Coulson IH. A clinical and therapeutic study of 29 patients with infantile acne. Br J Dermatol. Sep 2001;145(3):463-466. doi:10.1046/j.1365-2133.2001.04397.x
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