Alopecia areata (AA) is a common cause of nonscarring alopecia. The aim of this epidemiologic study is to review the clinical characteristics and treatment of childhood alopecia areata in a mixed ethnic population. The study population consisted of a total of 392 children seen over a 4-year period with AA diagnosed before the age of 16 years. The female:male ratio was 1:1.4. There were 309 Chinese (78.8%), 51 Malays (13.0%), and 32 Indians (8.2%). The mean age at the time of diagnosis was 11.2 years. The majority of patients (71.7%) had alopecia of less than 6-months duration and 6% had previous episodes of AA. Females appeared to have more severe involvement. A familial history of AA was observed in 33 patients (8.4%). Associated atopy was found in 26.6% of patients and in 32.3% of their first-degree relatives. Other associations such as vitiligo or Down syndrome were rare. For limited AA, topical and/or intralesional corticosteroid was the first-line treatment used and squaric acid dibutyl ester was the choice of treatment for patients with extensive involvement. The profile of the poor respondents to therapy included young age of onset, past history of AA, Down syndrome, and extensive involvement.
Mycosis fungoides (MF) is the most common form of cutaneous T-cell lymphoma. It usually occurs in middle-aged and elderly persons, although several reports have described its occurrence in young children. The aim of this study was to review the profile and outcome of childhood MF in Singapore from 1989 to 1998. A total of nine patients (six males and three females) were diagnosed with MF before the age of 21 years. There were four Chinese, four Malay, and one Indian. The age at the time of histologic diagnosis ranged from 6 to 20 years (mean 14.3 years). Eight of the nine patients presented with hypopigmented patches and plaques. According to TNM staging, three were in stage 1A and six in stage 1B. The treatment modalities included psoralen plus ultraviolet A (PUVA) (n = 5), UVB (n = 2), and potent topical steroids (n = 2). We found that PUVA induced a faster clinical remission, but maintenance PUVA was required to prolong the relapse-free interval. This study also highlighted the need to consider MF in the differential diagnosis of hypopigmented dermatoses in dark-skinned individuals, especially if they occur on the buttocks.
The disparity in familial distribution may point to genetic differences between the two groups. Further studies to evaluate this difference in familial distribution may contribute to the understanding of the pathogenesis of psoriasis.
Generalized lentigines associated with multiple noncutaneous features, as in the LEOPARD syndrome and the Carney complex, have been well reported in the literature. Reports of patients with generalized lentigines without systemic abnormalities (termed "generalized lentiginosis") are increasing as well. Despite the lack of systemic features, patients with generalized lentigines only should be monitored for further development of other noncutaneous features, especially cardiac anomalies. We present two patients with generalized lentiginosis and propose a working algorithm in the approach to a child with this finding.
Harlequin ichthyosis is a rare and severe congenital erythrodermic ichthyosis characterized at birth by hyperkeratotic plates covering the entire body, ectropion, eclabium, poorly developed ears, and contractures of the hands and feet. Two Chinese children, a 2-year-old boy and an 11-year-old girl, presented with these classic features as well as alopecia and loss of eyebrows and eyelashes. The boy was small for his age and was found to have hypothyroidism at the age of 18 months; he is currently on thyroxine replacement therapy. At 6 years of age, the girl developed symmetrical polyarthritis associated with positive rheumatoid factor and radiologic evidence of erosive arthritis, suggestive of juvenile rheumatoid arthritis. She received prednisolone, nonsteroidal anti-inflammatory drugs (NSAIDs), and subsequently methotrexate for her arthritis, with clinical and radiologic improvement. Early therapy with oral retinoids in both children accelerated shedding of the hyperkeratotic plates as well as improved ectropion and eclabium. There was no major adverse reaction to oral retinoids. The development of juvenile rheumatoid arthritis in survivors with harlequin ichthyosis has not been previously described. The use of prednisolone and NSAIDs in the girl did not affect the skin condition, but the addition of methotrexate led to a decrease in erythema. The association with autoimmune disease is probably coincidental. The psychosocial impact of this severe lifelong disease on the two families was enormous. Early retinoid therapy may improve the disorder and help increase survival rates. A multidisciplinary approach, including psychosocial support of the affected families, is vital in the management of this lifelong disease.
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