Bskirk Barber, MD, FRCPC

“There is no more difficult art to acquire than the art of observation, and for some men it is quite as difficult to record an observation in brief and plain language.”

In this age of evidence-based medicine and randomized clinical trials, it is easy to forget that careful clinical observation and documentation are the foundation of medical practice. In their Photo Essay “

Pityriasis Rosea in Dark-Skinned Children

,” Drs Amer and Fischer illustrate their observations for us in brief and plain language that I am sure Dr Osler would approve of.

Articles

In this age of evidence-based medicine and randomized clinical trials, it is easy to forget that careful clinical observation and documentation are the foundation of medical practice.

Observation and Documentation Still the Foundation of Medical Practice

This 13-year-old girl, who has just entered secondary school, has been devastated by the appearance of psoriatic plaques on her scalp. She states that her peers shun her and make rude comments about the "scabs" on her face. Her mother has told her not to be concerned, noting that she has lived with extensive psoriasis for nearly 30 years and has had to put up with the loss of her hair. Thus, the patient now not only fears her schoolmates' comments but also worries that she is destined to have severe psoriasis and lose her hair like her mother.

Scalp involvement does not predict severe psoriasis or hair loss.

The scalp is commonly affected by psoriasis, and scalp psoriasis is often the initial presentation of the disease.

Diagnosis and prognosis of scalp psoriasis.

 It is often difficult to distinguish between psoriasis and seborrheic dermatitis when the scalp is the only area of involvement; the reason for the difficulty is that both conditions are red and scaly (papulosquamous). Some clinical clues that can help differentiate between the two are:

Psoriasis typically appears as well demarcated plaques, whereas seborrheic dermatitis most commonly involves the scalp diffusely.

A poor response to simple therapy with shampoos alone suggests psoriasis. 

Scalp involvement does not predict the severity of the psoriasis, nor is it associated with hair loss, unless there is a traumatic or infectious complication. However, the psychological burden of scalp psoriasis is often great because the plaques commonly extend a few centimeters onto the forehead and involve the periauricular areas and the inside of the ear. Moreover, scalp psoriasis is frequently difficult to manage.

 The treatment of scalp psoriasis provides unique challenges, not the least of which is poor adherence, which in my experience is the most common reason for treatment failure. The facial component of the disease is best managed with topical corticosteroid creams (low to mid potency) or calcineurin inhibitors (see Dermclinic in the October 2008 issue of CONSULTANT FOR PEDIATRICIANS [pages 414-416]). When it comes to treatment of the scalp proper, patients' hair presents challenges: it limits the delivery vehicle for the active agent to substances that patients will find acceptable, and it often limits the timing of treatment to the overnight hours.

In addition, it is extremely difficult for any topical agent to be effective if the scalp plaques are thick and adherent. Thus, I usually first try to remove the scale. This must be done chemically because vigorous physical removal may result in a Koebner phenomenon and worsen the scalp disease. I typically use 3% salicylic acid compounded in an oil base (I prefer a bath oil because these are more easily removed) and applied to the scalp overnight; this is showered out in the morning. After a few applications of salicylic acid, the plaques will be much thinner, allowing for more effective topical therapy.

The treatment of choice, just as with facial psoriasis, is topical corticosteroids. A number of delivery systems are available. Shampoos and foam delivery systems are often preferred because of their ease of use. Topical alcohol solutions can be used in the daytime because they have little noticeable effect on the hair. Overnight oils and creams are less desirable because of the potential messiness.

Vitamin D analogues are also available as alcohol solutions. These can be prescribed for patients who are concerned about using topical corticosteroids.

This 13-year-old girl, who has just entered secondary school, has been devastated by the appearance of psoriatic plaques on her scalp. She states that her peers shun her and make rude comments about the "scabs" on her face

Scalp Psoriasis in a 13-Year-Old Girl

This 9-year-old girl has extensive psoriasis and is currently receiving narrowband UVB phototherapy for her body plaques. Her mother has insisted on covering her daughter's face during treatment and on having the girl use sunscreen on her face whenever she is outdoors to prevent premature aging.

Which topical therapy would be best for treating this patient's facial psoriasis?

This patient's plaque-type facial psoriasis was treated with topical tacrolimus

Facial psoriasis presents a unique challenge because it has a significant impact on a child's self-esteem and because the available treatments may produce local adverse effects. Facial involvement occurs in up to two-thirds of patients with psoriasis and is often seen in early-onset and severe disease. There are 3 distinct clinical patterns of facial involvement in patients with psoriasis:

The hairline pattern is commonly associated with scalp psoriasis and is treated in conjunction with the scalp. The central facial variant is thought to be related to seborrheic dermatitis; a trial of topical ketoconazole in addition to an anti-inflammatory agent often proves effective against this type of facial psoriasis. Topical corticosteroids are the anti-inflammatory treatment of choice for the plaque variant. The clinical challenge of corticosteroid treatment is to balance effectiveness with the known adverse effects of atrophy and telangectasia. I typically initiate treatment with 1% hydrocortisone applied twice daily and increase to a midstrength corticosteroid if I do not see a response in 2 weeks. Topical calcipotriol has been reported to be effective for all types of facial psoriasis, but its tendency to irritate precludes routine use.

In addition, topical calcineurin inhibitors (pimecrolimus and tacrolimus)- which also are anti-inflammatory agents-can be very useful in the management of facial psoriasis. Pimecrolimus and tacrolimus are applied twice daily and do not have the adverse effects of atrophy and telangectasia commonly seen with topical corticosteroids used long-term. However, the treatment of psoriasis is an off-label use of these agents.

Extensive Psoriasis in a 9-Year-Old Girl

This teenager had been taking penicillin for a sore throat about 2 weeks before this rash developed. Her pediatrician thought that the rash was a drug reaction and had her discontinue the medication. Nevertheless, the rash persisted for more than a month after therapy was stopped. 

The patient is devastated by the rash. Her parents have tried to calm her by telling her that it is "just a skin problem" and that it is "really quite minor, all things considered."

Does this look like an allergic reaction to you-or is something else going on?

 Teenagers fear psoriasis more than people of any other age. When psoriasis strikes in adolescents, it is commonly in the 

 Guttate psoriasis has an explosive onset and covers the body in 3- to 5-mm psoriatic papules that do not respect clothing cover. Fortunately, once we gain control of the initial outbreak, many affected youngsters will once again have clear skin-at least until their next bout of "strep throat," which typically precedes the skin outbreak.

Guttate psoriasis is only one of the ways in which the disorder affects children. Psoriasis affects 2% to 3% of the population; in one-third of those affected, the rash develops before their 16th birthday. Patients who have a childhood onset may have different genetics than those with adult-onset psoriasis for several reasons

Girls are more often affected than boys (56% vs 41%). 

First-degree relatives are more likely to be affected (49% vs 37%). 

Spontaneous remission is more likely to occur (35% vs 24%). 

There is greater relative involvement of the face and scalp (57% vs 46%).  

A growing body of research suggests that young adults with early-onset psoriasis may have a significant increase in atherosclerotic heart disease that will manifest early in their lives.

The most common presentation in childhood is 

. However, if one looks only at children younger than 2 years, then the most common type is "

 with a subsequent generalized psoriasis.

Napkin psoriasis may present in 2 ways. The first is a localized variant in which the psoriatic plaques are well-demarcated and bright red; they cover most of the diaper area. The second form starts as the localized variant but rapidly spreads to involve the trunk and extremities symmetrically. These diaper rashes are generally more difficult to control than the irritant dermatitis that is most common. I maintain these basic principles when I treat affected children:

I can usually accomplish these goals with the consistent use of pastes (removed by mineral oil) and judicious use of 1% hydrocortisone. On occasion, I prescribe a topical anti-yeast agent.

Children are particularly disadvantaged by therapy. Most practitioners are reluctant to match their treatment efforts to the severity of the psoriasis because there are few therapeutic guideposts. Topical corticosteroids are the mainstay of therapy for mild psoriasis, but these are often inadequate, even for moderate disease. Therefore, most children are undertreated. This is unfortunate because the effect of psoriasis on their quality of life and that of their family is often dramatic.

It is in the best interest of the child with moderate to severe psoriasis to be referred to a pediatric dermatologist. We are gaining significant experience with the use of "biologics" in the treatment of children with inflammatory diseases, such as psoriasis, arthritis, and Crohn disease, and we are having a tremendous impact on their quality of life with minimal adverse effects.

Raychaudhuri SP, Gross J. A comparative study of pediatric onset psoriasis with adult onset psoriasis. 

Guttate Psoriasis in a Teenage Girl

 This boy has had areas of hypopigmentation around his eyes, mouth, and nose for the past 2 years. He has been applying a topical corticosteroid to the affected area, but new lesions continue to develop. 

What disorders would you consider in the differential, and what treatment options would you offer?

is a devastating diagnosis to present to parents. The fear, which is palpable in the examination room, is usually based on concerns about an affected relative or friend. Also, vitiligo is viewed as a disfiguring and disabling condition-which is certainly true in some cultures. 

Fortunately, pityriasis alba, or postinflammatory hypopigmentation, is the most common cause of hypopigmentation- not vitiligo. I see relief more commonly than tears when pityriasis is the diagnosis. Pityriasis alba is really quite distinct when you see the ill-defined, scaling patches that are lighter than the surrounding skin but that are not stark white like vitiligo. These patches and the surrounding skin become lighter in the winter and more apparent in the summer as the normal skin becomes pigmented and the scaling patches do not. Simple emollients and 1% hydrocortisone will reduce the inflammatory component and allow for normal pigmentation. 

Vitiligo is the result of the total absence of melanocytes in affected areas of the epidermis. This absence is thought to be the result of an autoimmune event in persons with an underlying genetic predisposition. Recently, Canadian colleagues published an article in which they retrospectively reviewed their records of 101 children seen at Toronto's Hospital for Sick Children from 2000 to 2002 to assess clinical outcomes and assess the safety of topical corticosteroids.

 Half of the children had a family history of autoimmune diseases, and 22% had another family member with vitiligo. The first vitiliginous patch occurred between the ages of 3 years and 10 years in 77% of the children. In two thirds of the children, the head and neck were involved, and in 60%, more than one area was involved. 

The most common treatments for children with vitiligo are topical corticosteroids. It is convention to apply mid- to high-potency corticosteroids to the vitiliginous patches for many months. The Canadian investigators reviewed their data of the safety and effectiveness of this type of management. All of the children had been given topical steroids (83% received high-potency and 17% received mid-potency agents). The first follow-up examination occurred at a mean of 81.7  41 days. Complete repigmentation was recorded in 64% of the patients; there were no statistically significant differences in children given high-potency and mid-potency steroids. In 24% of the children, there was no recorded change, and in 11%, vitiligo had progressed.

In that study, there were a number of children in whom the expected cutaneous changes of long-term topical steroid use developed (striae in 8%, atrophy in 5%, and telangiectasia in 5%). The striking discovery was that adrenal insufficiency developed in some of the children from the topical steroid use. Of the 101 children, serum cortisol levels were tested in 73; of these, 21 (29%) of them had an abnormal cortisol level. This testing was not performed as it would have been had this been a prospective trial: the test was conducted at a median time of 14 days from the start of treatment (range, 1 to 100 days), and only half of the tests were conducted in the early morning. Nevertheless, the information is a good treatment guide for us. Also, 8% of the children had abnormal thyroid function. 

 This retrospective chart review has its limitations. However, the clinical information it has uncovered should guide our treatment plans for these children. First, highpotency steroids are an effective treatment option. Other studies show that they should be the treatment of choice. The potential for adrenal suppression is significant; for that reason, many of my colleagues recommend routine screening of early morning plasma cortisol levels at baseline and again at 4 weeks. It would also seem prudent to include thyroid function testing. An intermittent regimen using 4 to 6 weeks on-off cycles of high-potency topical steroids may reduce the systemic effects. 

Kwinter J, Pelletier J, Khambalia A, Pope E. High-potency steroid use in children    with vitiligo: a retrospective study. J Am Acad Dermatol. 2007;56:236-241.    

Vitiligo on a Boy's Face

DISCUSSION: Option C, Seborrhea, is the correct answer.

 has many of the features of the other papulosquamous conditions listed, but the diagnosis is most commonly clinically evident on examination alone. If you are unsure, a KOH preparation will quickly sort out the dermatophytes. Also, the lack of itch makes atopic dermatitis very unlikely. 

The difficulty arises in excluding psoriasis. Many consultants consider that there is significant overlap between seborrhea and psoriasis because many children with seborrhea also have features of psoriasis. I like to reserve my final thoughts; I am not comfortable excluding psoriasis until I have seen a rapid response to therapy, and until I see that this response continues if the condition recurs.

-yeast commonly found as part of the skin's normal microflora-is the most likely cause of this condition. There are a number of immunologic and biochemical theories to explain individual susceptibility or causation but, for the clinician, the often rapid and complete response to topical azoles is strong evidence that this organism plays a significant role in the etiology.

Infantile seborrhea has many of the features of the other papulosquamous conditions listed, but the diagnosis is most commonly clinically evident on examination alone. If you are unsure, a KOH preparation will quickly sort out the dermatophytes. Also, the lack of itch makes atopic dermatitis very unlikely. 