Enlarging Hypopigmented Lesions in a 6-Year-Old Girl
A 6-year-old girl presented for a well-child examination. She had no complaints, but her mother had noted spots on her neck 2 to 3 years ago and similar spots on her knees 2 to 3 months ago. Prior treatment with triamcinolone cream had no effect. The family history was negative for similar lesions. Her physical examination results demonstrated 6 smooth, 1 to 2 cm, hypopigmented patches on her back and lower legs (Figure).
What is the cause of these hypopigmented lesions?
(Answer and explanation is on the next page).
Answer: Vitiligo
When treating a patient with hypopigmented lesions, common causes such as postinflammatory hypopigmentation, pityriasis alba, tinea versicolor, and vitiligo along with less common diseases must be considered (Table).1,2 Common entities can often be diagnosed by their clinical characteristics or simple tests; a Wood’s lamp examination or potassium hydroxide skin scraping preparation can help confirm tinea versicolor. Our patient’s lesions displayed the characteristic milky-white, sharply defined, scaleless patches of vitiligo (Figure).3 The distribution was consistent with symmetrical and bilateral nonsegmental forms of vitiligo; the unilateral and segmental form has a different prognosis and therapeutic options.4 While rarely necessary, histopathologic evidence of scant or absent melanocytes can confirm the diagnosis.1,4
Vitiligo affects about 1% of the general population and presents before 20 years of age in almost 50% of patients.1 There is no difference in prevalence between sex, race, or skin type.1,4 A family history is present in up to 35% of pediatric patients, in contrast with 8% in adults.5-7 Although a definitive etiology is unknown, the prevailing theory suggests autoimmunity underlies the destruction of melanocytes.8
Spontaneous repigmentation occurs in 1% to 25% of patients; however, wait and watch approaches are not recommended as affected areas tend to expand, and early therapy appears more efficacious.9,10 Topical corticosteroids are first-line therapy and achieve some repigmentation in up to 90% of patients.11 Unfortunately, prolonged use can cause skin atrophy and development of telangiectasias. Topical calcineurin inhibitors, tacrolimus and pimecrolimus, have comparable efficacy to topical corticosteroids but lack their side effects.8 However, the US Food and Drug Administration has warned of a possible link to lymphoma and skin cancer. Other treatments include narrowband UV phototherapy and monochromatic excimer light. Recalcitrant cases may benefit from grafting and/or melanocyte transplantation.8
Vitiligo is associated with auditory, ocular, and autoimmune abnormalities. Sensorineural hearing loss and ocular disease may be found in 20% and 40% of patients, respectively, while autoimmune hypothyrodism or hyperthyroidism affects up to 24% of pediatric patients with vitiligo.1,8 Other associated disorders include pernicious anemia, type 1 diabetes mellitus, Addison disease, alopecia areata, and several polyendocrinopathy syndromes.1 Accordingly, it is recommended that patients receive 25 (OH) vitamin D and antinuclear antibody tests at disease diagnosis, undergo hearing evaluations, be advised when taking ototoxic medications, receive regular ophthalmic examinations, and receive annual thyroid screening, blood chemistries, and complete blood counts.12-14
When hypopigmented skin lesions are noted in children, vitiligo should be in the differential diagnosis. Although it is often considered a cosmetic disorder, it has many well-documented disease associations. Alongside treating patients’ depigmentation, health care providers need to be vigilant in screening for changes to hearing and vision, and for endocrine disease. They should also recommend regular application of sunscreen to prevent sunburn.
Beau DiCicco, MD, graduated from the the John P. and Kathrine G. McGovern Medical School at University of Texas Health Science Center in Houston.
Helena Jenkinson, BS, is a student at the John P. and Kathrine G. McGovern Medical School at University of Texas Health Science Center in Houston.
Elizabeth Eisenmenger, BS, is a student at the John P. and Kathrine G. McGovern Medical School at University of Texas Health Science Center in Houston.
Lynnette Mazur, MD, MPH, is a professor of pediatrics at the John P. and Kathrine G. McGovern Medical School at University of Texas Health Science Center in Houston.
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