Welcome to NP Certification Q&A presented by Fitzgerald Health Education Associates. This podcast is for NP students studying to pass their NP certification exam. Getting to the correct test answers means breaking down the exam questions themselves. Leading NP expert Dr. Margaret Fitzgerald shares her knowledge and experience to help you dissect the anatomy of a test question so you can better understand how to arrive at the correct test answer. So, if you're ready, let's jump right in.  

The nurse practitioner sees a six-week-old with her parents for an urgent care visit. The family is of southeast Asian ancestry. They voiced concern about “dark spots” over her lower back and buttocks that have developed during the past three weeks. The child was born after full-term pregnancy, went home with parents in 24 hours, and has had appropriate weight gain, and is meeting developmental milestones. On physical exam, the infant appears to be in no acute distress and has blue to blue-gray spots over the lower back and the buttocks. The areas are flat with irregular shape and unclear borders, and are noted in the areas consistent with parental report. Areas of discoloration are about 8 to 20 centimeters in diameter. Palpating the areas does not appear to elicit any discomfort in the child, and the color does not blanch with pressure. The skin texture is within normal limits, and the rest of the physical exam is otherwise unremarkable. These findings are most consistent with:  

1. Café au lait spots 
2. Congenital dermal melanocytosis 
3. Port wine stain 
4. Capillary hemangioma 

The correct answer here is B. Congenital dermal melanocytosis, also known as Mongolian spot. 

Where do you start? First figure out what kind of question this is. Given that we're provided with a history and physical exam findings, and then given a choice of four conditions this could be, this is clearly a diagnosis question.  

Some background information here. The current term used to describe what has historically been called Mongolian spots is congenital dermal melanocytosis. This term describes when the lesion occurs— it's congenital, so will be noted at birth or very shortly after birth. Where it occurs—dermal, within the skin. And what's involved? The melanocytes. The abbreviation for this is CDM, and is appearing more and more in the literature. These pigmentation changes and I always hesitate to call them changes; they're just variants of what's norm, are usually seen in children of select ancestry, most commonly African, Asian, or North American Indigenous groups, and are characterized by these flat blue-gray skin discoloration that sometimes can be present at birth. But also, as we have with this little one, then they kind of show up in the first few weeks of life. In practice, you're more likely to see a child like this who did not have these areas of altered pigmentation at birth, but emerged more when the child was a few weeks old.  

Why do I say this? If the CGM was present at birth, and the birth was attended by healthcare providers, and we were told the baby went home with parents after 24 hours. So, it does sound like it was an attended birth. One of the healthcare providers should have picked up on this and then simply reassured the parents as to what this was.  

But sometimes you’ll get the family like this, they go, wait a minute, she didn't have these when she was born, but she's got them now. What is going on? So, you can imagine the parents to be quite taken aback by this and appropriately concerned. So back to what CDM is. This is an altered coloration that occurs from a collection of melanocytes in the deep layers of the skin, and is usually not associated with any particular disease, but is rather related to ancestry. The lesion location? Almost always lower back and buttocks. Sometimes it's high up on the shoulders, less commonly in the extremities. Every now and again, I will see a kid with CGM who has lesions on their arms and legs, and that's considered to be perfectly within the bounds of normal limits. These skin lesions are characterized by being flat. So, in other words, you run your finger over the lesion, the lesions clearly within the skin, not above the skin, irregular in shape with these unclear edges, and can vary widely in size. The skin texture is otherwise perfectly normal.  

Often providers fear that they're going to be missing a child who has been bruised because these can look like bruises, and that they're going to miss a child who has been misused, neglected, or had some kind of a major accident, as CGM can look like an area of ecchymosis. In this case, what you'll be able to do is rely on your comprehensive health assessment skills. And note, in this case, the baby appears to be thriving. The history is absolutely consistent with the evolution of congenital dermal melanocytosis in the first weeks of life. And most importantly, what you're able to do is palpate these lesions, actually press on them fairly hard, and the child doesn't react in pain. If this was bruising and what we were looking at work areas ecchymosis due to the child being misused or having a significant accident, pressing on this area would elicit crying from the little one. Plus, think about the distribution of the lesions. This is really classic CDM. With this condition areas of altered coloration tend to abate by the time the child is in the toddler years. But sometimes persist a little bit beyond that. But generally, by the time the kids get around age five, all of the lesions have been resolved.  

And again, I just have to say, I hesitate to even call them a lesion because this is considered to simply be an ethnicity appropriate condition that we find in the skin. There's no particular treatment for CDM. The next steps would be to discuss the findings with the family member and reassure them that this is normative. But what I often ask the family, Are there other kids in your family who have these? And if so, this gives reassurance. Like somebody will say, Oh yeah, my cousin's son had these when he was a baby or, you know, my niece has these, something along those lines. And that often gives great reassurance. Having this kind of conversation and being reassuring, not dismissive of parental concern will go a very long way to engaging this family and looking to you as a trusted health care provider.  

There are a number of dermal conditions that are seen at or around birth. Our job as health care providers is to distinguish what's normative finding, particularly in this child of the stated ethnicity, and what is a finding where intervention could be used or is needed. With that in mind, let's take a look at the question and the given options.  

The NP sees a six-week-old with her parents for an urgent care visit. The family is of southeast Asian ancestry. They voiced concern about dark marks over her lower back and buttock that have developed over the past three weeks. The child was born after full-term pregnancy, went home with parents at 24 hours, and has had an appropriate weight gain, and is meeting developmental milestones. On exam, the infant appears in no acute distress and has blue to gray spots over the back and buttocks. These areas are flat with irregular shape and unclear edges and are noted in the areas that the parents report. The areas of discoloration are 8 to 20 centimeters in diameter. Palpating the area does not appear to elicit any discomfort in the child, and the color does not blanch with pressure. Skin texture is within normal limits. The physical exam is otherwise unremarkable. These findings are most consistent with: 

1. Café au lait spots. This is incorrect. Café au lait spots are usually light to dark brown pigmented birthmarks, and just using the term birthmarks means presence at birth. The spots can change in size and increase in number over time. Their distribution is more random than with congenital dermal melanocytosis, and usually does not cover nearly as much body surface area. Café au lait spots do carry similar characteristics with CGM in that they are painless when palpated. The presence of more than six café au lait spots on an infant should create a consideration for further investigation, as sometimes this is associated with other clinical conditions like neurofibromatosis type 1 or other genetically based conditions.  
2. Congenital dermal melanocytosis, a.k.a. Mongolian spots. This is of course, the correct answer. 
3. Port wine stain. This is also incorrect. A port wine stain is a type of a birthmark that usually appears on the face. The lesion typically starts as a flat pink or red patch and is noted at the time of birth. Over time, this lesion can become larger, darker, and the lesion could even develop some thickness to it where you run your finger over it, you can feel the lesion is within the skin, but it's also above the skin. You'll recall that with congenital dermal melanocytes the lesions are flat. Port wine stain is usually first noted in the newborn, and it gets its name because it looks as if somebody has splashed port wine, which has a deep red color onto the child. The origin of this lesion is abnormally formed capillaries, and when these capillaries dilate the lesion darkens. While the face is far and away the most common location for a port wine stain, the neck and the scalp can also be involved. This birthmark grows with the child remaining proportional to the child's size. In other words, if we were to hit the fast forward button now, and a baby with port wine stain is one-third larger overall than they were a few months ago, then the port wine stain will have grown proportionally like that, covering the same area, but of course it's going to be bigger. Port wine stains can be treated when the child's a bit older to minimize appearance. At the same time, the presence of a port wine stain is occasionally associated with specific genetic conditions, and an expert consultation is usually obtained.  
4. Capillary hemangioma. This is, of course again, not the correct answer. Capillary hemangioma are benign vascular neoplastic lesions that are usually noted at the time of birth. But what they do is they grow more rapidly than the child does in the first few weeks of life. In other words, this is a lesion that grows out of proportion to the patient's size. So, one way of thinking of it— barely noticeable at week one of life, very noticeable by week 5 of life. About 80% of these lesions occur on the head or neck region and for a reason that's not well understood, it is much more common in female than in male birth gender assignment infants. The typical history is that the lesion was not noted initially when the baby was first born and then crops up and grows rapidly. The early lesions are often small flat plaques and filled with these small microvessels or telangiectasia, so the lesion could potentially blanch when you run your finger over it. Over time, the lesions then grow rapidly and then, interestingly enough, regress and most children will have little in the way of evidence of the lesion by the time they're around preschool age.  

Treatment is quite variable according to the location and the size of the lesion. For another example, a lesion on the eyelid or the lip will be more aggressively treated than perhaps a small lesion on the thigh. And the reason for that is if you have, like a little one with a lesion on the lip, that's going to grow fast. And what that could do is interrupt with feeding appropriately. Doesn't look like it's painful. But what you could end up with is a little squirt who let’s just say the lesions on the lower lip now, is the structure of the lip is altered due to this rapidly growing hemangioma. And now the baby can't suck properly. Something along those lines. Or on the eyelid and now the eyelid can't be opened properly, so the little squirt will not be seeing properly.  

I can remember a few years ago I had a child who had a small hemangioma on her thigh and the decision was made to do watch-and-wait therapy because it was in a non-cosmetic, non-function area of the body. She did fine. It was total resolution by the time she was old enough, I think, to go into preschool.  

Expert consultation is the rule with capillary hemangiomas to get a better idea of what treatment would be possible for these kids. And interestingly now quite often systemic or topical beta blockers are used. There are a number of other therapies as well. Capillary hemangiomas are generally found in little ones with no other health problem. But again, for larger lesions, lesions in certain locations, this expert consultation will give you an idea of whether genetic testing is needed or not.  

Key takeaway: Parameter in a skin condition and the newborn is key to safe and effective practice. At the same time, knowledge of common conditions where further evaluation is needed is also critical.  

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