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Submitted: 04 November 2015 Modified: 18 March 2019
HERDIN Record #: PCHRD1511040758217

Multiple basal cell carcinomas in an 11-year-old Filipino-American girl with basal cell nevus syndrome.

Jacqueline Michelle G. de las Alas,
Clarita C. Maaño

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Basal cell nevus syndrome (BCNS) is a rare autosomal dominant multisystem disorder due to a mutation in the PTCH1 gene. Dermatologic manifestations include basal cell carcinoma (BCC) and palmoplantar pits. This case of childhood-onset BCC arising in a child of Filipino descent with BCNS illustrates the importance of early detection and diagnosis, which will then facilitate early counseling and management of this lifelong condition. An 11-year-old Filipino-American girl presented with multiple variegated pearly papules on the face, clavicular area and upper back. She also had multiple palmoplantar pits. Shave biopsy of a papule showed BCC. Trans-abdominal ultrasound revealed bilateral ovarian calcifications. Jaw and chest radiography were unremarkable. Skin lesions were initially treated with ablative erbium:YAG laser and later recurred after 3 months. Imiquimod cream was then started which resulted in partial resolution of lesions after 12 weeks. The dermatologist's role in the diagnosis of BCNS is integral, especially in this case where the patient is a seemingly well child with predominantly dermatologic manifestations. Clinical findings of BCNS, including BCC, are recurrent. Lifetime monitoring, counseling and adequate patient education and appropriate treatment of lesions are central to the management of this condition.

Publication Type
Journal
Publication Sub Type
Journal Article, Reprint
Title
Journal of the Philippine Dermatological Society
Frequency
Semi-Annual
Publication Date
November 2013
Volume
22
Issue
2
Page(s)
69-73
LocationLocation CodeAvailable FormatAvailability
Philippine Council for Health Research and Development Library Box No. 98-A Fulltext Print Format (Request Document)
Philippine Council for Health Research and Development Fulltext pdf (Request Document)