Pediatricians and family practitioners are likely to be the first to see young patients with troubling skin conditions and can often respond quickly with a number of effective therapies. However, as pediatric dermatologist Tor Shwayder makes clear in this interview with Skin & Aging, sometimes even general dermatologists need to call in a pediatric specialist to manage what he calls “extreme skin conditions.”

There comes a point at which general practitioners such as pediatricians and family practice physicians should refer patients to a dermatologist, or even a pediatric dermatologist. That point, says pediatric dermatologist Tor Shwayder, is when any condition either presents as or becomes extreme — that is, when there is a risk of scarring from acne, when large parts of the body are affected, and in some cases, when the condition appears in potentially life-threatening locations.

“There are conditions that just don’t go away with the first-line treatment indicated for primary care,” maintains Tor Shwayder, MD, FAAP, FAAD, LRAM, who is Director, Pediatric Dermatology, Henry Ford Hospital, Detroit, MI. He offers a rundown of conditions that call for the expertise of a dermatologist, and perhaps a pediatric sub-specialist, either at initial presentation or when they become extreme.

ACNE

Common and variable as it is, Dr. Shwayder is clear about when acne can be considered extreme — when there’s any sign of scarring. (See Figure 1.) “We want most of all to prevent scarring, so if it’s not getting better with the usual approaches, refer,” he urges.

First-Line Therapy

He describes a step-by-step march toward the offices of dermatologists. Steps 1 through 3 — first-line therapy — would include over-the-counter treatments, which should be tried first before progressing to the prescription pad of the general practitioner. “Pediatricians would likely start with prescription creams and lotions such as vitamin A derivatives, benzoyl peroxide and antibiotics.” However, when the third step — prescribing oral antibiotics — does not control the condition, he insists, the patient should be referred to a pediatric dermatologist.

Dermatologists Only

“When first line therapies have failed and/or there is a risk of scarring, this is the juncture at which we come in,” says Dr. Shwayder. For adolescent girls, more aggressive options might include birth control pills, but generally the top of the medications list at this point would be isotretinoin (Accutane). “While there’s nothing magical about it, there’s a lot to learn and a fair number of side effects and forms to fill out/comply with…plus there are a lot of lawyers waiting to sue you over it, so therefore you have to know what you’re doing,” he explains. Other approaches intended to prevent acne scarring cited by Dr. Shwayder include blue light therapy and injections into the cysts. The ultimate goal, he says, is to make scar revision unnecessary “I’ve seen acne in patients as young as 6 or 7 and horrendous scarring in 10 year olds.”

BIRTHMARKS

Dr. Shwayder emphasizes the importance of having certain types of birthmarks seen “sooner than later,” including those that are pigmented, hypopigmented, large and/or covering affecting large areas of the body.

Hypo- and Hyperpigmented Birthmarks

Dr. Shwayder considers two risks when he sees a child with large dark congenital nevi, which are usually brown to black but may start out red in fair-skinned people before turning brown). (See Figure 2.) “There is a 2% to 4% risk of melanoma. The other risk, leptomeningeal melanocytosis, is harder to quantify but has deadly potential as it can cut off cerebral spinal fluid circulation. For that reason, we usually try to get MRIs in the first 4 months of life with this finding,” he says.

Epidermal nevi, which proliferate on the top layer of the skin, he notes, carry no malignancy risk, but they may be associated with epidermal nevus syndrome. (See Figure 3.) “This is a phenomenon in kids who have seizures and mental retardation in association with large birthmarks, which can be unsightly — velvety and furry — as well as smelly due to their trapping anaerobic bacteria and fungi.”

Café au lait macules — These are usually single and smaller, but if there are many, they can be a sign of neurofibromatosis type 1. While Dr. Shwayder does not consider this an emergency, he says, “A child with multiple café au lait macules should be seen by someone who knows how to talk about them.”

Swirls, Whorls or Splotches (Nevus Depigmentosa)

Hypomelanosis of Ito syndrome is characterized by the presence of whorled hypochromic skin lesions and is often associated with systemic manifestations. In addition, Dr. Shwayder points out, “A really large nevus depigmentosa involving a large area of the body can be associated with the epidermal nevus syndrome mentioned earlier.”

Large Yellow Patches (Nevus Sebaceous)

A large nevus sebaceous of the head and neck can signal Schimmelpenning syndrome, which is associated with mental retardation, seizures, etc.

Large Capillary Malformations

This category would include large red birthmarks — either flat, like port wine stains, or raised, like hemangiomas. (See Figure 4.) Dr. Shwayder considers the most serious among these to be flat capillary malformations involving the head and neck because they also can involve the brain, as in Sturge-Weber syndrome, and large hemangiomas of the head and neck, which can be associated with PHACE Syndrome, a condition he describes as “fairly devastating because it involves malnormalities and malformations of the brain and or vessels that feed into it — together with abnormalities of the eyes and the large vessels that come off the heart that feed the brain.” In addition, he says, “red birthmarks that involve an entire limb can cause that limb to be larger or smaller than the other limb.”

RED SCALY SKIN

Dr. Shwayder states that a child with total body erythema and scale should be seen and promptly treated by someone who can differentiate between lifelong genetic diseases like ichthyoses, including X-linked ichthyosis and Netherton syndrome — all of which present with dry, thickened, scaly or flaky skin — and those that are reasonably benign like eczema, psoriasis and seborrheic dermatitis.

“I’ve seen kids who are red from head to toe, with widely varied diagnoses. Widespread cutaneous infections may signal immune deficiency, as in widespread candida”

EXTREME BLISTERS

Major concerns associated with extreme blisters include epidermolysis bullosa, which causes the fragile, easily injured skin to blister, leaving it vulnerable to infection. (See Figures 5 and 6.) Other serious conditions associated with blistering include Staphylococcal scalded skin syndrome, streptococcus pyogenes infection, and candida.

CONCLUSION

The take-away message, from Dr. Shwayder’s point of view, is that extreme skin conditions require extreme vigilance and care. He believes primary care physicians need clear guidelines to know when a great level of expertise is needed for accurate diagnosis and effective/timely treatment for conditions that can be life-threatening; nearly as important is intervention that can spare a child the trauma of a scarring, disfiguring, or an otherwise emotionally devastating outcome that could have been prevented.