childhood and adult-onset xeroderma pigmentosum: clinical evaluation with striking new findings

Background: xeroderma pigmentosum variant (xp-v) is a genetic disorder that starts in early childhood with a mild disease course. the aim of study was to record all cases of xp-v that were seen and examined over a specific period.methods: this descriptive study included 48 patients; there were 4 (8.33%) patients with xeroderma pigmentosum (xp) and 44 (91.66%) patients with xp-v. patients with xp-v were divided into childhood and adult-onset types.results: childhood-onset type was detected in 34 patients, including 20 (58.82%) males and 14 (41.17%) females. their ages ranged from 3-25 years, with a mean of 15 years. freckling and solar keratosis were observed in 100% and 23.68% of patients, respectively, while non-melanoma skin cancer (nmsc) was detected in 8 (21.05%) patients, including one case with squamous cell carcinoma (scc) and 7 with keratoacanthoma. in the adult-onset type, ten cases were seen, half of each gender. their ages ranged from 23-60 years, with a mean of 32 years. patients gave a history of early adult onset of their disease. skin hyper-photosensitivity was the first problem, followed gradually by other features of solar damage to the face, including freckles and solar keratosis. scc and keratoacanthoma were each observed in two patients.conclusions: the clinical picture of xp-v was similar to ordinary xp but with late age onset and a slower course. the clinical picture of adult-onset xp-v was similar to the childhood type.