Erosive diaper rash in a neonate
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A 2-week-old baby girl presented with a rash in the diaper area. She was born prematurely at 32 weeks’ gestation and was hospitalized in the NICU.
The rash began on her right buttock, lower back and groin shortly after birth as erythematous patches. The rash later progressed to involve the right thigh and became more raised (Figure 1). An ulcer also later developed on the medial buttocks. No other gluteal cleft abnormalities were present. The area was treated with nystatin cream twice daily for 1 week without improvement.
Can you spot the rash?
A. Irritant contact dermatitis
B. Infantile hemangioma
C. Candidiasis
D. Impetigo
E. Herpes simplex virus
Case discussion
The correct answer is B, infantile hemangiomas (IHs), which are common benign vascular tumors that present in early infancy. They classically will grow over time before eventually involuting. IHs occur in approximately 3% to 5% of infants. Risk factors include female gender, white race, prematurity, low birth weight and multiple gestation pregnancies.
IHs are not fully formed at birth and can be subtle in their initial presentation, with telangiectasias, pallor and/or mild erythema. IHs have a characteristic natural history composed of a period of rapid growth (usually peaking by 3 months of age), followed by a period of slower growth and then ultimately a period of gradual involution. During the growth phase, the hemangioma becomes more evident.
The most common appearance as a classic “strawberry” hemangioma refers to the superficial location in the skin and is thereby classified as a superficial hemangioma. IHs can also occur deeper in the skin, presenting as a soft nodule, or they can present as a combination of the two, known as a deep IHs or mixed IHs, respectively. IHs can be further classified as localized or segmental, with the latter referring to a broad area of involvement that may correspond to a developmental subunit. In most cases, IHs start to involute around age 1 year and gradually decrease in size and lighten in color. However, IHs can leave a footprint behind in the form of fibrofatty tissue, atrophy and/or residual telangiectasias.
Although many IHs are uncomplicated, certain subsets of IHs are at higher risk for complications and can require further workup and management. IHs located in the diaper area (especially the perineum) are at high risk for ulceration, which is the most common complication of IHs. Ulceration usually occurs during the rapid growth phase but can recur if there is involvement in the perineum likely due to repeated friction and irritation. Ulceration can lead to pain, bleeding, scarring and secondary infection. Thus, prompt identification and management of a hemangioma in the diaper area is important.
Although erythema and ulceration can also be seen in the setting of severe irritant contact dermatitis or bacterial/herpetic infection, the segmental pattern, presence of telangiectasias and history were all clues to the diagnosis of IHs in our patient. If the diagnosis is in question, bacterial cultures and/or viral PCRs can be obtained for further clarification.
In addition to ulceration, complications of some subsets of IHs in the diaper area can also include underlying internal anomalies. Lumbosacral hemangiomas, especially those that are large/midline, are at high risk for underlying spinal dysraphism and should prompt imaging to evaluate for this condition. Furthermore, segmental lumbosacral and perineal IHs are high risk for additional internal anomalies involving the genitourinary, spinal and skeletal systems collectively known as LUMBAR syndrome (also known as SACRAL or PELVIS syndrome).
LUMBAR stands for lower body hemangioma, urogenital anomalies/ulceration, myelopathy, bony deformities, anorectal malformations/arterial anomalies and renal anomalies. To screen for LUMBAR syndrome, it has been proposed that infants aged younger than 3 months with a segmental lower body hemangioma receive an ultrasound with color doppler of the spine, abdomen, and pelvis in addition to a thorough physical exam. An MRI of the spine is recommended at age 3 to 6 months in these patients, given the high risk for myelopathy.
Management of an ulcerated IH in the diaper area involves wound care and diligent diaper care, which are essential to reduce irritation, prevent further ulceration and promote healing. This involves frequent barrier therapy with either petroleum jelly or zinc oxide paste and occlusive dressings. Topical antibiotics such as mupirocin or metronidazole are often also used. If any signs of infection like malodor or purulence are present, bacterial cultures and oral antibiotics should be considered. Topical timolol may be considered to treat the hemangioma. Systemic therapy may be needed; oral propranolol is often used to treat ulcerated IHs but should be used with caution in the setting of LUMBAR syndrome, given the risk of arterial anomalies. Any associated internal anomalies should be managed by the appropriate specialists.
References:
Eichenfield LF, et al. Neonatal and Infant Dermatology. 3rd ed. London: Elsevier; 2015.
Iacobas I, et al. J Pediatr. 2010;doi:10.1016/j.jpeds.2010.05.027.
Johnson EF, Smidt AC. J Pediatr. 2014;doi:10.1016/j.jpeds.2013.08.045.
For more information:
Khurana is an attending physician at The Children’s Hospital of Philadelphia. She can be reached at khuranam@email.chop.edu.