Yoke-Chin Giam

A clinical study of childhood alopecia areata in Singapore.

Abstract: 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.

Harlequin Ichthyosis in Association with Hypothyroidism and Juvenile Rheumatoid Arthritis

Abstract: 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.

Generalized lentiginosis in two children lacking systemic associations: case report and review of the literature.

Abstract:  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.

Pseudopili annulati in a dark-haired individual: a light and electron microscopic study

Abstract: We report pseudopili annulati for the first time in a dark‐haired Chinese girl. The hair was elliptical in shape and twisted along its long axis. The banded or ringed appearance was due to incident light being reflected by the flattened segments. Banding was also noted under polarized light but was not present under transmitted light. Under transmitted light the whole length of the hair appeared dark and the thickness varied at regular intervals, giving rise to fusiform segments due to the twisted nature of the hair shaft. Scanning and transmission electron microscopic examinations revealed no abnormalities in the cuticle and cortex.

Marie Unna hypotrichosis in a Chinese family.

Abstract: Marie Unna hypotrichosis is a rare, autosomal dominant hypotrichosis characterized by sparse or absent hair at birth with regrowth of coarse, wiry hair from childhood, followed by progressive loss on approaching puberty. We report a Chinese mother and child seen at our skin center with features of Marie Unna hypotrichosis. The family pedigree showed two affected sisters and one affected brother, consistent with an autosomal dominant mode of inheritance. The clinical, genetic, histologic, and ultrastructural features were consistent with the diagnosis of Marie Unna hypotrichosis. This is the first Chinese family reported.

Whorled scarring alopecia: a rare phenomenon in incontinentia pigmenti?

Incontinentia pigmenti is a rare X-linked dominant disease that affects the ectodermal tissues and is usually lethal in males. Two girls, 1 Malay and 1 Chinese, with incontinentia pigmenti of the Bloch-Sulzberger type had a whorled pattern of scarring alopecia. This phenomenon, hitherto unreported in association with this disorder, corresponded to the lines of Blaschko. The mother and maternal grandmother of the Malay girl also had whorled scarring alopecia. This phenomenon is permanent and can be used as a marker to ascertain affected adult women who may no longer have cutaneous manifestations. X chromosome inactivation in females during early embryogenesis results in a mosaic population of cells, which explains the linear and patchy cutaneous manifestations of incontinentia pigmenti.

Eruptive syringoma in a Chinese boy.

An 8‐year‐old Chinese boy was referred to the National Skin Centre (NSC) for evaluation of multiple white asymptomatic papules of 2 weeks duration. These lesions appeared suddenly on the face, neck, trunk, and extremities. There was no family history of a similar papular eruption. He was otherwise in good health.

Lepromatous leprosy in erythema nodosum leprosum reaction mimicking Sweet's syndrome

A 52-year-old Malay man presented with an acute oneweek history of multiple erythematous papules andnodules on the limbs, trunk, and face. On physical exami-nation, he had multiple erythematous nontender, non-pruritic nodules and plaques on the trunk, limbs, andforehead (Fig. 1). A few nodules showed pustulation andulceration (Fig. 2). He was febrile at 38 C. Systemicreview and general examination were normal. There wereno thickened nerves, neurological deficits, or deformitiesof the hands or feet. Eyes and oral mucosa were normal.The initial clinical impression was that of Sweet’s syn-drome in view of the acute onset of typical lesions andfever. Complete blood count revealed marked leukocytosis(24.1 · 10

Nevoid hypomelanosis associated with unilateral palmoplantar keratoderma: a result of genetic mosaicism

A healthy, 9-month-old Chinese girl of a non-consanguineous marriage presented with hypopigmented whorls and streaks, which were noticed at three months of age (Fig. 1). The lesions followed Blaschko’s lines and were widespread and bilateral, with a left-sided predominance. There was no preceding inflammatory stage, and her parents and two siblings were unaffected. Ocular, dental, neurological, and developmental assessments were unremarkable. A diagnosis of nevoid hypomelanosis was made. At 14 months, the hypopigmented patches showed signs of repigmentation. However, the patient developed a new unilateral, hyperkeratosis affecting the left palm and sole associated with hypohidrosis, which was consistent with diffuse, non-transgradient PPK (Fig. 2).