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Newborn presenting with a severe rash
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A newborn boy was admitted to the neonatal intensive care unit for evaluation of a severe, total-body rash. He was born full-term after a normal pregnancy and vaginal delivery was complicated only by being group B strep-positive. The mother received two doses of penicillin prior to delivery. She had no history of herpes, but did have a documented history of varicella when she was 12 years old. Her VDRL, hepatitis B and HIV screens were also negative. At delivery, the boy’s Apgar scores were 8 and 9 (1 and 5 minutes).
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The patient had an extensive rash consisting
of dozens of discrete, apparently blistering, excoriated-appearing lesions with
no erythemia. Source: Photos courtesy of James H. Brien |
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The family history is unremarkable, and no one else in sick at home.
Examination upon arrival to the NICU revealed normal vital signs and an alert baby in no distress, but with an extensive rash consisting of dozens of discrete, apparently blistering, excoriated-appearing lesions with no erythema as shown in figures 1 – 2. A larger lesion was noted adjacent to the scrotum on the right side (figure 3).
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A larger lesion was noted adjacent to the
patient’s scrotum on the right side. |
Initial lab tests included a CBC, metabolic profile, blood culture, skin lesion bacterial and HSV cultures and lesion biopsy. The baby was empirically treated with ampicillin + gentamicin + acyclovir, pending lab results.
What’s Your Diagnosis?
- Langerhan Cell Histiocytosis
- Neonatal Herpes simplex virus infection
- Neonatal Varicella
- Incontinentia pigmenti
Case Discussion
The skin biopsy showed this baby had Langerhan cell histiocytosis (A). This is an uncommon condition, formerly referred to as Histiocytosis X, which includes three types; (1) Letterer-Siwe disease, (2) Hand- Schüller-Christian (HSC) disease and (3) Eosinophilic granuloma. This classification has essentially faded into history as frequent overlap of features led to the thought that these represent a spectrum of the same disease. This baby turned out to have multiorgan involvement, including severe developmental delay, bone marrow suppression (before chemotherapy), liver and spleen enlargement and intestinal infiltration.
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Other manifestations may include oral lesions,
including gingival hypertrophy with ulcerations. |
If we were using the old classification, the baby’s illness would be consistent with Letterer-Siwe Disease. Other manifestations may include oral lesions, including gingival hypertrophy with ulcerations (Figure 4), which are often confused with thrush in infants, as well as bone lesions and pulmonary infiltrates (both seen in Figure 5 with severe pulmonary infiltrative disease and mandibular destruction). Both figures 4 & 5 are of a different patient. HSC disease consists of the triad exophthalmos, diabetes insipidus and lytic skull lesions. Eosinophilic granuloma consists only of lytic bone lesion(s), as shown in figures 6 & 7.
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Severe pulmonary infiltrative disease and
mandibular destruction. |
The treatment of this condition is usually best managed by an oncologist, as the treatment is similar to that of some cancers. The prognosis for the most severe type of LCH is poor.
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Eosinophilic granuloma consists only of lytic
bone lesion(s), as shown in the picture and scan above. |
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Neonatal HSV should always be suspected in cases of a newborn with vesicular skin lesions and empirically treated until it can be ruled out. It would be unusual for herpes to appear at delivery, but congenital HSV can occur. When seen, it is likely to be in a baby that is obviously ill, but it is always best to error on the side of treatment with acyclovir at a dose of 60 mg/kg/day ÷ q8 hours until HSV can be ruled out. This baby’s HSV cultures were all negative. Nowadays, we would probably use PCR.
Neonatal varicella can certainly occur, and might appear similar to this baby’s rash. However, the mother had documented varicella as a child, making it highly unlikely that this baby would be at risk of that infection.
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The initial stage (maculopapular/vesicular
stage) of incontinentia pigmenti (IP) may have lesion similar to the
patient’s, however, they only occur along the lines of Blaschko (caused by
the migration of embryonic cells). |
The initial stage (maculopapular/vesicular stage) of incontinentia pigmenti (IP) may have lesions similar to this baby’s, however, they only occur along the lines of Blaschko (caused by the migration of embryonic cells), as shown in Figure 8. Since this is an X-linked dominant disorder, most males die in utero, which also helps rule out this baby having IP. Most babies with IP will also have systemic involvement as well, including bone, CNS and numerous eye problems. The treatment depends on the various systems affected. Usually these babies are followed by neurologists and ophthalmologists early on in anticipation of problems. Some less severely affected babies may be unrecognized unless they develop seizures, a detached retina or some other systemic problem resulting from the disorder.
Vice Chair for Education at The Children’s Hospital at Scott
and White and Associate Professor of Pediatrics at Texas A&M University,
College of Medicine, Temple, Texas. E-mail: jhbrien@aol.com.
e-mail:jhbrien@aol.com
While not one of the choices listed in the quiz, any baby born with true blistering lesions at delivery should be evaluated for epidermolysis bullosa, an inherited mechanobullous disorder. There are three types; EB simplex, junctional EB and dystrophic EB (most severe). The only treatment is protecting the skin from even minor trauma, especially with the dystrophic type as the chronic scarring may ultimately lead to severe deformity of the hands and feet. These patients should probably be seen by an orthopedic surgeon with an interest in this disorder early on as part of a team approach to care.
I would like to thank Amanda Farris, DO, one of our Pediatric Hospitalists, for recognizing, referring and helping with gathering the data for this case.
Columnist comments
If you’re looking for a good excuse to go to Hawaii, AND get a few hours of high-quality CME, I would suggest going next month during the week of September 20th. The new Chief (Chair) of the Department of Pediatrics at Tripler Army Medical Center in Honolulu, Colonel Marty Weisse (Figure 9), has arranged for a 2-day symposium on the 22nd and 23rd, for the commemoration of the Department Conference Room to the memory of Jim Bass (Figure 10).
If you can be there, you will get to hear Larry Pickering from the CDC, Russell Steele from The Ochsner Clinic in New Orleans and myself (two out of three ain’t bad). I know it is short notice, but sometimes the best deals can be made at the last minute.
Dr. Bass died in 2001 from complications of cancer, but during his long and distinguished Army Medical Corps career (more than 40 years of active duty), he trained hundreds of pediatric residents and dozens of infectious disease fellows, including COL Weisse and myself. There will be several good lectures and a good time for all. If you would seriously like to consider showing up, just e-mail me (jhbrien@aol.com) and I’ll provide more details.
In the meantime, stay cool.