A healthy 11-day-old male infant is brought to the pediatric clinic for evaluation of rash. The rash started with a 2-mm papule on the left medial epicanthal fold 4 days before the clinic visit. A day before coming to the clinic, the rash had spread to the upper left eyelid and the nasal bridge. What's the diagnosis?
A healthy 11-day-old male infant is brought to the pediatric clinic for evaluation of rash. The rash started with a 2-mm papule on the left medial epicanthal fold 4 days before the clinic visit (Figure 1). The mother had scratched it, trying to pop the papule. The next day, the infant developed multiple erythematous papules and pustules medial to the left eye with sparing of the eye. A day before coming to the clinic, the rash had spread to the upper left eyelid and the nasal bridge (Figure 2).
The patient is asymptomatic and without fever or any sick contacts. The rest of his skin is clear. His mother has no history of genital herpes or oral lesions. No one has kissed the baby. The patient is being fed both breast milk and formula, with vitamin D supplementation. He usually has 6 to 7 wet diapers a day and passes stools around 4 to 5 times a day.
The patient was born via repeat cesarean delivery at 39 weeks’ gestation to a 39-year-old gravida 3 para 2 woman. The mother’s laboratory was unremarkable, including negative tests for human immunodeficiency virus (HIV), hepatitis-B surface antigen, and syphilis screen, group B Streptococcus, gonorrhea, and chlamydia. The mother had no active herpetic lesions during pregnancy.
The rupture of the membranes was at the time of the cesarean delivery. Apgar scores were 9 and 9 at 1 and 5 minutes, respectively. Postnatal resuscitation was not required. Both maternal grandparents have diabetes mellitus type 2. The mother lives with 2 families in the house, the grandfather smokes outside the house, and there are no animals in the house. Her immunizations are up-to-date.
The physical examination revealed normal vital signs and a rash on the patient’s face comprised of multiple 1-mm to 2-mm pustules on erythematous base involving the medial upper and lower eyelids bilaterally (Figure 3). The involvement was more on the left side, bridge of the nose, and contiguous lower mid-forehead. There was no discharge or any oozing from the rash. There were no oral or conjunctival lesions. The rest of the examination was unremarkable.
Some articles have described neonatal cephalic pustulosis as a separate entity from neonatal acne, based on the absence of comedones and the presence of pustules surrounded by an erythematous halo.1 Other differential diagnoses (Table) are as follows:
ERYTHEMA TOXICUM NEONATORUM
Erythema toxicum neonatorum is rarely present at birth and may begin after 24 hours of life. It can appear anytime from birth to 2 weeks and usually lasts for several days to weeks. Rash is polymorphous and usually does not involve the palms and soles.2 Scrapings of pustules will reveal a large number of eosinophils. No treatment is necessary.
TRANSIENT NEONATAL PUSTULAR MELANOSIS
Transient neonatal pustular melanosis is more common in blacks and generally present at birth but rarely appears after birth. It usually resolves within 24 to 48 hours and can involve the palms and soles. Scraping of the pustules can show neutrophils. No treatment is necessary.
Staphylococcal pustulosis is a common bacterial cause of pustular skin lesion that is usually localized to the periumbilical area, neck fold, and diaper area.3 Rarely it may be generalized.4 It presents as cutaneous erythema followed by peeling of skin and can cause significant complications and require prompt treatment. Gram stain of the scrapings can detect the bacteria. Treatment with appropriate antibiotics is recommended to reduce mortality.
HERPES SIMPLEX VIRUS
Herpes simplex virus (HSV) is usually accompanied by a history of HSV-2 in the mother. It generally manifests as grouped vesicles on erythematous base present anywhere on the body but mostly localized to the eye, skin, and mouth. Tzanck preparation can show multinucleated cells but a viral culture is the gold standard. Treatment with systemic acyclovir is recommended to prevent disseminated or central nervous system (CNS) spread.
Eosinophilic pustulosis presents as recurrent white-to-yellow pustules on the scalp or forehead that usually crust within 2 to 3 days but can last for years. There is some association with immunodeficiency. Giemsa stain can be done, which shows eosinophils predominance. It can be treated with topical corticosteroids or erythromycin.
ACROPUSTULOSIS OF INFANCY
Acropustulosis of infancy presents as a chronic recurrent eruption of vesicles and pustules on the hands and feet usually lasting for hours after birth, but it can occur anytime during infancy. The lesions usually disappear in 10 days but can reappear every month for the first 3 years. These erythematous papules can progress to intensely itching pustules in other parts of the body and usually require symptomatic treatment.
Miliaria pustulosis can be divided into 2 types—miliaria rubra and miliaria crystalline—within the first week of life. It presents as a polymorphous rash on the face, trunk, and intertriginous areas. It is precipitated in warm weather as excess sweating can cause obstruction of the eccrine duct. No treatment is necessary but cool baths and breathable fabrics are helpful.
Congenital dandidiasis can present at birth or may appear after a few days as macules, papules, and pustules over the body but sparing the diaper area. It usually involves the palms and soles and can cause yellowish discoloration of fingernails with paronychia. Treatment is topical antifungal.
The infant was seen in the clinic and subsequently admitted to the hospital due to suspected herpes simplex infection (Figure 4). He was started on intravenous acyclovir 60 mg/kg/day divided Q8 hourly. Bacitracin ointment was applied topically on the rash twice a day. The viral culture was done from the wound specimen. Ophthalmology consultation was obtained, and they confirmed that there was no involvement of the eye. The following tests were sent: herpes viral PCR, Tzanck smear, and direct fluorescent antibody (DFA) test.
On the second day of admission, the pustules were larger and the red base more confluent (Figure 5). Because of concern for HSV, the Infectious Disease service was consulted and upon their suggestion fungal stain for potassium hydroxide (KOH) and cultures were sent. Intravenous acyclovir was continued and bacitracin ointment was replaced with miconazole. Final results showed negative fungal and viral cultures, the KOH stain was negative, and HSV PCR was not detected. Acyclovir was discontinued. The rash started to improve (Figure 6) and the patient was discharged home on miconazole ointment.
Diagnosis of neonatal cephalic pustulosis
Neonatal cephalic pustulosis usually develops during the first month of life and is characterized by uniform small papules and pustules on a red base involving the face and neck. It is related to the stimulation of sebaceous glands by maternal androgens.5,6 Histologic examination demonstrates an inflammatory infiltrate of primarily neutrophils and yeasts of the genus Malassezia (M).6 Some have suggested that neonatal cephalic pustulosis results from an inflammatory response to this yeast. Higher colonization is associated with increased severity of pustulosis.1 Most cases resolve spontaneously in several weeks without treatment but treatment with anti-yeast topical therapy may hasten resolution.
Neonatal cephalic pustulosis can be differentiated from neonatal acne by the presence of monomorphic lesions, the absence of comedones or nodular lesions, and the absence of a follicular distribution. The following criterion was suggested to define this entity: (a) pustules on face and neck; (b) age at onset, younger than 1 month; (c) isolation of M furfur by direct microscopy in pustular material; (d) elimination of other causes of pustular eruption; (e) response to topical ketoconazole therapy.5
Treatment usually is not required as neonatal cephalic pustulosis is self-limiting. In severe cases, ketoconazole cream 2% or other topical antifungal agents and hydrocortisone cream 1% can be used.7
The patient was reevaluated in clinic a week after hospital discharge, and the rash was almost completely resolved with a fine ring of desquamation (Figure 7). Routine visits after that showed no residual rash.
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. 2002;138(2):215-218.
2. Zuniga R, Nguyen T. Skin conditions: common skin rashes in infants. FP Essent. 2013:407:31-41.
3. Melish ME. Staphylococcal infections. In: Feigin RD, Cherry JD, eds. Textbook of Pediatric Infectious Diseases; Philadelphia, PA: WB Saunders;1987:1260-1291.
4. Mogre DA. Generalised staphylococcal pustulosis on a neonate: a case report. Australas Med J. 2013;6(10):532-535.
5. Rapelanoro R, Mortureux P, Couprie B, Maleville J, Taieb A. Neonatal Malassezia furfur pustulosis. Arch Dermatol. 1996;132(2):190-193.
6. Niamba P, Weill FX, Sarlangue J, Labrèze C, Ckouproe B, Taieh A. Is common neonatal cephalic pustulosis (neonatal acne) triggered by Malassezia sympodialis? Arch Dermatol. 1998;134(8):995-998.
7. Howard RM, Frieden IJ. Vesicles, pustules, bullae, erosions, and ulcerations. In: Eichenfield LF, Frieden IJ, Mathas EF, Zaenglein AL, eds. Neonatal and Infant Dermatology. 3rd ed. London: Saunders, 2015;111-139.