Palpebral syringomas and Down's syndrome.
BACKGROUND
Palpebral syringomas have been reported to be more frequent in patients with Down's syndrome than in the normal population.


OBJECTIVE
The aim of the present study was to evaluate, in a population of institutionalized patients with Down's syndrome, the prevalence of syringomas and their possible cytogenetic relationships.


METHODS
Sixty-one institutionalized patients with Down's syndrome were examined in order to assess the presence of palpebral syringomas. Sixty mentally retarded non-Down's syndrome individuals were used to control group.


RESULTS
Fourteen patients, 13 females and 1 male, were found to be affected. The prevalence of syringomas in both sexes was 23%; 42% of all females, and 55% when only adult females were considered, had syringomas. Thirteen of the 14 affected patients had a karyotype of Down's syndrome with free trisomy 21, 1 had a mosaicism 47,XX, +21/46,XX. Histologic examination confirmed the diagnosis in all 4 biopsied cases. A clear-cell pattern was observed only in 1 patient while, sporadically, few tubules showed a central syringial-type cuticula.


CONCLUSIONS
The higher prevalence found in females, as compared to males, can be partially explained by their older age (mean 23.8 vs. 13.9 years). Palpebral syringomas are a common cutaneous pathology in adult females with Down's syndrome.
Schepis
C
C
Unit of Dermatology, OASI Institute for Research and Mental Retardation and Brain Aging (IRCCS), Troina, Italy.
Siragusa
M
M
Palazzo
R
R
Ragusa
R M
RM
Massi
G
G
Fabrizi
G
G
D000293:Adolescent; D000328:Adult; D002648:Child; D004314:Down Syndrome / Q000150:complications* / Q000235:genetics; D005142:Eyelid Neoplasms / Q000150:complications* / Q000473:pathology; D005260:Female; D006801:Humans; D007621:Karyotyping; D008297:Male; D013544:Sweat Gland Neoplasms / Q000150:complications* / Q000473:pathology; D018252:Syringoma / Q000150:complications* / Q000473:pathology