PEMPix is the American Academy of Pediatrics Section on Emergency Medicine’s visual diagnosis competition. It is held annually at the National Conference and Exhibition. This year, all ten finalists will be posted online on PEMBlog.com and at PEMPix.com, one a day with voting opening to AAP Section on Emergency Medicine members thereafter. I hope you enjoy this online-only format, and hope that it will serve to highlight some of the fantastic learning cases that our colleagues submitted this year. It was again very difficult for the panel of judges to select the finalists and I could not have done it without their assistance. As a special treat I’ve included a musical selection form the 1980s as an optional “hint,” and to serve as a soundtrack for your learning.

This PEMPix case was submitted by:

Yu Hsiang Johnny Lo, MD, Pediatric Emergency Medicine Fellow and Alex Rogers, MD from the University of Michigan

Look at that face

10 day-old full-term infant presents with progressive facial rash x2 days. His mother first noticed a red rash on his forehead and eyelids. His pediatrician diagnosed him with with “acne” per the mother, and recommended over the counter emollients. His rash continued to progress and spread across his face. On the day of presentation the patient woke up with yellow crusting and discharge from both eyes, and was unable to open them due to significant swelling. It was time to go tot the Emergency Department.
 
History is notable for a 40 week gestational age home birth via normal spontaneous vaginal delivery with midwife assistance. Mother declined Hepatitis B vaccine, vitamin K, and erythromycin ointment, along with the newborn screen. The mother denied herpes, gonorrhea, or chlamydia infections. She was also Group B Strep positive at 18 weeks gestation, treated with amoxicillin, with negative GBS at 36 weeks. All other prenatal testing was normal.
 
This afebrile infant was well appearing with no skin lesions or abnormal findings other than those shown in the following images.

What is the diagnosis?

A. Erythema toxicum neonatorum

B. Miliaria rubra

C. Neonatal cephalic pustulosis

D. HSV infection

E. Cutaneous candidiasis

C. Neonatal cephalic pustulosis

The treatment team initially considered a full sepsis evaluation and unroofing of a pustule to send for culture and HSV testing. However, Dermatology was consulted and diagnosed patient with neonatal cephalic pustulosis, a benign condition that self-resolves without additional work up or treatments. A surface culture eventually grew Corynebacterium spp. (skin flora) with numerous polymorphonuclear leukocytes. Ophthalmology noted no ocular involvement on exam. The infant was admitted for observation and then subsequently discharged in the following morning. On outpatient follow up few days after discharge, he was recovering well without additional complaints, with resolving lesions and minimal eye swelling.

Neonatal cephalic pustulosis is a benign and self-limited neonatal rash occurring in 10-66% of neonates. Characteristic lesions of inflammatory papules and pustules on the face and scalp usually surface between 12 to 22 days life. Smear of the pustule with potassium hydroxide and culture can sometimes show Malassezia species. In those case you may consider treatment with ketoconazole topically. However, the vast majority of cases will resolve without complications or scarring. The timing, and isolated face and scalp involvement, with a benign clinical course, make benign neonatal cephalic pustulosis the most likely diagnosis.

Erythema toxicum neonatorum (ETN) is seen in more than 20% of full-term infants, usually on second day of life and regresses in 5 to 14 days. These lesions typically evolve from erythematous papules on an erythematous base into pustules within hours, and affect the trunk, extremities, and face. Our patient had isolated facial involvement, with significant localized swelling, not typical of ETN. The cytologic exam of the pustule also did not show presence of eosinophils, a common finding in ETN lesions.

Miliaria rubra occurs in ~4% of neonates, typically after the second week of life. It is caused by occlusion and inflammation of eccrine sweat glands, with hot and humid environments being common triggers. Characteristic lesions of erythematous papules and pustules on an erythematous base occur most commonly on the neck, axilla, and groin. Management usually involve unbundling the newborn, ensuring breathable fabric use, and lowering the environmental temperature. The course is typically benign, but lesions can become superinfected with staph species. This  patient’s isolated facial involvement with significant swelling makes this diagnosis less likely. Miliaria rubra lesions also typically have lymphocytic predominance, which was not present on the smear.

Cutaneous candidiasis can be acquired in utero or perinatally through contact with the vaginal surface. Characteristic lesions are erythematous patches with peripheral papules and pustules over face, chest, trunk, palms and soles, surfacing on the first to sixth day of life. Patients may also have paronychia, oral thrush, or classic diaper dermatitis. The range of symptoms can be from isolated cutaneous eruptions to severe systemic disease indistinguishable from bacterial sepsis. Intrauterine contraceptive devices or other foreign body are possible risk factors for infection. First line treatment is topical antifungals, with systemic medications used for refractory or severe multisystem cases.

Herpes simplex virus infection is seen in ~0.03% of all live births, with the majority of transmission acquired perinatally. 39% of the cases present with skin, eye, and mouth disease. Classic lesions are grouped vesicles and pustules on an erythematous base, often coalesced in groups, with conjunctivitis, excessive tearing, along with ulcerative lesions of the oral mucosa. Newborns often have other symptoms such as irritability, lethargy, poor feeding, fevers, and seizures. Early treatment improves survival and reduces long-term sequelae.

References

Kimberlin DW. Herpes simplex virus infections of the newborn. Semin Perinatol 2007; 31:19.

Flagg EW, Weinstock H. Incidence of neonatal herpes simplex virus infections in the United States, 2006. Pediatrics 2011; 127:e1.

Ghosh S. Neonatal pustular dermatosis: an overview. Indian J Dermatol. 2015;60(2):211. doi:10.4103/0019-5154.152558

Reginatto FP, Villa DD, Cestari TF. Benign skin disease with pustules in the newborn. An Bras Dermatol. 2016;91(2):124‐134. doi:10.1590/abd1806-4841.20164285

Antaya RJ, Robinson DM. Blisters and pustules in the newborn. Pediatr Ann 39:635–645. 2010

Nanda S, Reddy BS, Ramji S, Pandhi D. Analytical study of pustular eruptions in neonates. Pediatr Dermatol 2002; 19:210.

Lyons RE, Levine R, Auld D. Miliaria rubra, a manifestation of staphylococcal disease. Arch Dermatol 1962; 86:282.

Hidano A, Purwoko R, Jitsukawa K. Statistical survey of skin changes in Japanese neonates. Pediatr Dermatol 1986; 3:140.

Pappas PG, Kauffman CA, Andes DR, et al. Clinical Practice Guideline for the Management of Candidiasis: 2016 Update by the Infectious Diseases Society of America. Clin Infect Dis. 2016;62(4):e1‐e50. doi:10.1093/cid/civ933

Darmstadt GL, Dinulos JG, Miller Z. Congenital Cutaneous Candidiasis: Clinical Presentation, Pathogenesis, and Management Guidelines. Pediatrics. Feb 2000, 105 (2) 438-444; DOI: 10.1542/peds.105.2.438