July 1997
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This infant has characteristic "flaky paint"
dermatitis associated with kwashiorkor.
SAN FRANCISCO — A year or more of pre-diagnosis observation in the case of a newborn with generalized redness and scaling is not unusual. According to dermatologist Elaine C. Siegfried, MD, often a definitive diagnosis is possible only after a period of observation — "sometimes a year, sometimes more than a year," she said.
"It is usually a very difficult time for these parents who are worried about what is wrong with their child, but I always counsel the parents that they will be very lucky if we are able to give their baby's abnormality a name by the time the child is 1 year old," said Siegfried. "Our goal is always to prevent some of the associated complications." Siegfried is assistant professor of pediatrics and dermatology at the Saint Louis University Health Sciences Center in St. Louis.
Generalized redness and scaling is often clinically and histologically non-specific. In searching for clues of infection in these babies, find primary lesions and they will offer clues to help direct your workup, said Siegfried. Screening diagnostic studies include potassium hydroxide (KOH), Tzanck smear, surveillance cultures for staphylococcal scalded skin syndrome (SSSS), a complete blood count, and depending on the epidemiology of the area, syphilis serology and HIV studies. "You really are pushed to begin empiric therapy in these babies because the risk-benefit ratio favors this," she said.
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Candida and SSSS are frequently responsible and usually offer good clinical clues to direct your workup and enable you to feel confident in your treatment recommendations, Siegfried said. "Syphilis, 'the great mimicker,' and AIDS are two diseases that you have to keep in the back of your mind because they have a variety of presentations," she said.
It is wise to have a very high index of suspicion regarding Candida septicemia because this can kill a premature infant. "A really good clue is that their urine cultures are often positive," said Siegfried. "Positive Candida cultures from the urine are often overlooked as incidental findings or contamination, but you have to remember that with a baby who has generalized redness and scaling to pay attention to the urine Candida cultures."
SSSS is a common cause of generalized redness and scaling. These babies, Siegfried said, very typically have periorbital erythema with a sad look about them and erythroderma accentuated in skin folds as well. A good clue to this diagnosis is that the oral mucosa is not involved, she said.
In addition to infection, there are a variety of other disorders either primary cutaneous or syndrome associated that could result in generalized redness and scaling. When Siegfried consults on a case such as this, she said her first question is always whether "the baby is otherwise healthy and thriving."
If the baby is healthy, the probability is high that the cause of redness is atopic dermatitis, seborrheic dermatitis or psoriasis. It may be difficult to differentiate among the three in the first three months of life.
"There are some clues," said Siegfried, "but fortunately initial empiric therapy is the same for these three diseases." After a period of evolution, she pointed out, the differences become more apparent. She recommends frequent tap-water bathing, mild topical steroids and frequent use of bland ointment emollients as initial therapy.
When cases diagnosed as atopic dermatitis or seborrheic dermatitis do not respond to topical steroids, Siegfried says, consider psoriasis. "But just because an infant is diagnosed with psoriasis early on does not mean the prognosis is poor. A 13-year prospective study of infantile psoriasis documented only mild disease at follow-up," said Siegfried. "I tell parents this does not mean their children will not have psoriasis when they are adults, but they are not committed to a lifelong history of terrible psoriasis either."
The bottom line, said Siegfried is whether or not the baby is otherwise healthy. "If they are not, you have to have a very high index of suspicion, and you have to do your workup in a careful way, first ruling out all of the things that you can treat and then doing the more esoteric studies as acute conditions are ruled out."
The rarely described disorders that could present as a red, scaly baby are not rare in Siegfried's practice. "I see infants with a syndrome-associated eyrthroderma several times a year," she said. "As the molecular genetics and molecular biology of this category of diseases is recognized, their nosology will change, but right now I think about them in the broad categories of ichthyosis and ectodermal dysplasias — and of course there is tremendous overlap in these categories."
What about the collodion baby? These babies are frequently premature and have an easily recognizable phenotype of skin that looks thick and waxy, but in reality almost completely lacks skin barrier function. "I think about the collodion baby as a subset of an erythrodermic baby," said Siegfried. "Their status is comparable to that of a 24- or 25-week premature infant. They have the same or worse problems with temperature stability and worse problems with hypernatremic dehydration and increased susceptibility to infection."
Be aware of the potential for complications and understand that you can't get too aggressive with treatment, Siegfried warned. "Avoid manual débridement and be careful to avoid percutaneous toxicity," she said.
Siegfried recommended watching these babies very carefully for signs that will help you make a diagnosis. "Skin biopsy may help early on," she said. "Although directed laboratory tests can establish the diagnosis, I am very judicious about my use of laboratory tests based on the baby's other findings and the family history," Siegfried reported here at the Annual Meeting of the American Academy of Dermatology.
For more information:
- Siegfried EC. Dermatology in the newborn nursery. Presented at the American Academy of Dermatology meeting. Mar. 21-26. San Francisco.
- Farber EM, Mullen RH, Jacobs AH, Nall L. Infantile psoriasis: a follow-up study. Pediatric Dermatology1986;3:237.
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