Khalifa E Sharquie; Raed I Jabbar
Abstract
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%) ...
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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.
Ramin Radmanesh; Mohammad Radmanesh
Abstract
Background: There is no cure for xeroderma pigmentosum (XP) patients who suffer from persistent freckling and recurrent lifethreatening malignancies. We aimed to remove facial lentiginous pigmentations using CO2 laser resurfacing.Methods: 5 patients with clinically proven XP living in their third decade ...
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Background: There is no cure for xeroderma pigmentosum (XP) patients who suffer from persistent freckling and recurrent lifethreatening malignancies. We aimed to remove facial lentiginous pigmentations using CO2 laser resurfacing.Methods: 5 patients with clinically proven XP living in their third decade were scheduled to be treated with CO2 laser resurfacing. After tumescent anesthesia, the whole facial skin was treated with 3 UltraPulse® conventional CO2 ablation passes. The parameters used were 6 mJ, 5 mJ, and 4 mJ for the first to third passes. The mandibular areas were treated with two passes of 4 and 3.2 mJ, while the eye contours were treated with two passes of 3.6 mJ and 3.2 mJ.Results: The face was edematous and almost free of freckling immediately after resurfacing. The edema persisted for a week. The facial skin oozed within the first three days, followed by crust formation. After a week and after complete shedding of the crusts, smooth and erythematous skin appeared. The erythema persisted for more than two months. The patients were free of malignancy and freckling for up to 16 months follow-up.Conclusion: CO2 laser can remove lentiginous pigmentation and prevent or postpone malignancies for a considerable length of time.
