CLINICAL FEATURES IN XERODERMA PIGMENTOSUM PATIENTS AT NATIONAL HOSPITAL OF DERMATOLOGY AND VENEREOLOGY
DOI:
https://doi.org/10.56320/tcdlhvn.39.53Keywords:
xeroderma pigmentosum, poikiloderma, skin cancer.Abstract
A cross-sectional descriptive study was conducted on 35 xeroderma pigmentosum (XP) patients to investigate the clinical features and complication of
skin cancer. The average age of all patients was 23.23 years old, of which 60% were male; 60% of patients had family members who also had XP. The mean age of disease onset was 2.88 ± 3.25 years old. The onset symptoms were all cutaneous lesions (100%) such as sunburn (42.86%), hyperpigmentation (freckles, lentigines) (54.29%). Xerosis was the lesion with highest incidence (82.86%). Other skin manifestations such as sunburn, freckles, seborrheic keratosis, skin atrophy, poikiloderma, telangiectasia were found in over 60% of all patients. T
here were 7 patients (20%) with skin cancer including 6 cases of basal cell carcinoma (BCC) and 1 case of squamous cell carcinoma (SCC). The mean age at first appearance of BCC was 44 ± 18,72 years. BCC patients all received surgical treatment and had good outcome. The only case of SCC was a 16-year-old male patient with a giant tumor on the head, also the only case to have died from skin cancer.
