Dr. Daniel Krowchuk, a pediatric dermatology specialist at Wake Forest Baptist Health Brenner Children's Hospital, presents case studies of children with birthmarks, melanoma and rashes, and describes how these occurrences may be symptoms of more serious conditions.
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DANIEL KROWCHUK: Two resistant diaper rashes. So our first patient has had this eruption that involves not only the diaper area, but the face as well. And it has been unresponsive to conventional therapies. Things that I would point out when you look at this is just how well defined the affected areas are. Very abrupt borders, and it almost looks-- particularly when you see the face-- as if the lead agents are just stuck on the skin. And lastly, that there's a periorificial distribution, so you've got evidence of rational around the anus and around the mouth. And taken together, these features, once you've seen it once or twice, really sets the diagnosis of acrodermatitis enteropasthica, which is an autosomal recessive disorder in which a mutation creates problems with transporting zinc into the body through the intestine. So patients can absorb a bit, but not a sufficient amount. And in formula fed infants, this generally presents very early in life, within the first days or week or so of birth. And those who are breastfed, it often occurs after weaning, because breast milk has more sufficient amounts of zinc that are better absorbed. So there are a variety of symptoms that can occur in acrodermatitis enteropasthica. And I won't belabor these. The skin lesions are as we've already described. You can also have some hair loss. Interestingly children who have this often have behavioral disturbances. They're a little bit fussier and more iritable, a little bit more depressed than usual. There can be gastrointestinal symptoms, including growth retardation, failure to thrive, diarrhea, and some ophthalamologic consequences as well. So as it turns out, while acrodermatitis enteropasthica is the most common example of this sort of eruption, a variety of other entities can produce a rash that is almost identical. And those include the deficiencies that I've listed here, including Kwashiorkor and CF and essential fatty acid deficiency and some ways in which you might distinguish the two entities. The patient that you see on the right is one we saw in our dermatology clinic some years ago. The top photograph is her first visit. And after instituting zinc supplementation, it's striking how rapidly the improvement occurs. The bottom photograph is 10 days after she started supplementation. And you'll also notice this is a little girl whose parents had remarked on the fact she was just crabbier than could be imagined. But you see, on the bottom photograph, the hint of a smile. She was even happier 10 days into therapy. And so you'll want to monitor the zinc levels periodically. This may resolve over time, and it may not. It may require lifelong supplementation with zinc. OK. Our second resistant diaper rash. So again, a similar story. Not responsive to anti-fungals, not responsive to topical corticosteroids. And you can see that there's something deep erythema in the inguinal folds, something we might see in candidiasis or in seborrheic dermatitis. But I draw your attention to the lesion-- sort of to the diaper line superiorly where it almost looks hemorrhagic or petechial. And that should be a real red flag. Those sorts of lesions just don't happen in candidiasis or seb derm. And this is something that many of you may have encountered, Langerhans cell histiocytosis, a form of histiocytosis, in which Langerhans cells proliferate in potentially a variety of organ systems. But skin and bone most often are involved. It can occur at any age, but typically is seen in young children between the ages of one and four. So just looking at some of these skin lesions, because these are often the first signs of this disease. So again, you can have erythema in the creases. So that it could be inguinal folds, axilla, behind the ear. Scaling to brown papules, petechiae and hemorrhage, and even pustules, particularly on the palms and soles. And again, this photograph of a prior patient of ours. Again, the erythema in the folds, but with some areas of small petechiae. You can see erosions in the axilla. And you can see the papular nature of the eruption on the scalp of this infant and even on the trunk. Now, if you go on and you say, well, could it be seb derm? The answer is sort of, except that, again, that hemorrhagic nature of things is unusual in seb derm. And seb derm usually response very nicely to an appropriate topical corticosteroid, so that failure to respond would be perhaps another warning sign. So if you suspect that this is present, the thing that's going to be needed is a biopsy. So if you have a pediatric dermatology or general dermatology colleague who feels most comfortable with this, a biopsy special stain procedures performed. If the diagnosis is confirmed, then referral to a hem/onc is needed for evaluation of systemic involvement and appropriate therapy.
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