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.
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Case:
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?
Answer:
Vitiligo 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.1 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.1
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.
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