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Pediatr Dermatol. 2002 Jul-Aug;19(4):298-301.
A clinical study of childhood alopecia areata in Singapore.

Tan E, Tay YK, Giam YC.

National Skin Center, Singapore.

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.

online pharmacy ref. source: www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12220271&dopt=Abstract

Pediatr Dermatol. 2002 Jul-Aug;19(4):312-6.
Rothmund-thomson syndrome in three siblings and development of cutaneous squamous cell carcinoma.

Piquero-Casals J, Okubo AY, Nico MM.

Department of Dermatology, Hospital Das Clinicas, University of Sao Paulo, Sao Paulo, Brazil. Piquerermatoligico.com.br

Rothmund-Thomson syndrome (RTS) is a rare autosomal recessive genodermatosis. It is characterized by early onset of progressive poikiloderma and several other cutaneous and extracutaneous findings including alopecia, dystrophic teeth and nails, juvenile cataracts, short stature, hypogonadism, and bone defects. There are several reported cases of skin malignancies in RTS patients, indicating a possibly higher incidence of cutaneous and noncutaneous malignancies. We report three siblings with RTS who developed cutaneous squamous cell carcinoma (SCC).

online pharmacy ref. source: www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12220274&dopt=Abstract

Ann Dermatol Venereol. 2002 May;129(5 Pt 2):793-9.
[Genetic dissection of retinoic acid function in epidermis physiology]

[Article in French]

Ghyselinck NB, Chapellier B, Calleja C, Kumar Indra A, Li M, Messaddeq N, Mark M, Metzger D, Chambon P.

Institut de Genetique et de Biologie Moleculaire et Cellulaire, CNRS/INSERM/ULP, College de France, BP 10142, 67404 Illkirch Cedex, CU de Strasbourg, France.

The active metabolite of vitamin A (retinoic acid, RA) acts through the nuclear receptors RARalpha, beta and gamma and RXRalpha, beta and gamma. These receptors form RAR/RXR heterodimers, which bind to genetic regulatory DNA sequences and activate transcription of RA target genes. As RXR form heterodimers with a number of other nuclear receptors, such as the vitamin D3 receptor (VDR) and are involved in several signaling pathways. In the skin, RARgamma and RXRalpha predominate, but RARalpha and RXRbeta are also expressed. To elucidate the role of RA in skin physiology, we produced mutant mouse lines null for RAR or RXR. On the one hand, null mutations for RARa or RXRbeta have no effect on the skin, whereas a RARgamma-null mutation induces alterations in the granular cell layer. On the other, genetic inactivation of RXRa leads to embryonic lethality before epidermal development. Consequently, to determine the role of RXRa in adult mice, studies were performed using conditional somatic mutagenesis (permitting inactivation of a given gene in a specific tissue and in a time-dependent manner). Using this novel genetic approach, mutant mice were obtained in which RXRalpha was not expressed in the skin. These mice developed hair follicle degeneration, then alopecia, similar to that observed in VDR-null mutants, suggesting that hair follicle homeostasis depends on RXRalpha/VDR heterodimers. A similar genetic approach applied to the RARgamma locus demonstrated that topical administration of RA on the skin activates RARgamma/RXR heterodimers in suprabasal cells, and induces expression of a paracrine growth factor (HB-EGF) in these cells which, in turn, stimulates the proliferation of basal cells.

online pharmacy ref. source: www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12223961&dopt=Abstract

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