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Palpebral syringomas and Down's syndrome
C Schepis1, M Siragusa, R Palazzo
1Unit of Dermatology, OASI Institute for Research and Mental Retardation and Brain Aging (IRCCS), Troina, Italy.
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.
Conclusion:
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.