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. 2014 Jun;5(2):120-4.
doi: 10.1007/s13193-014-0307-6. Epub 2014 Apr 9.

Xeroderma pigmentosum: clinicopathological review of the multiple oculocutaneous malignancies and complications

Rajshekar Halkud  1 , Ashok M Shenoy  1 , Sudhir M Naik  1 , Purshottam Chavan  1 , K T Sidappa  1 , Siddharth Biswas  2
Affiliations

Xeroderma pigmentosum: clinicopathological review of the multiple oculocutaneous malignancies and complications

Rajshekar Halkud et al. Indian J Surg Oncol. 2014 Jun.

Abstract

Multiple oculo-cutaneous malignancies are a common manifestation on sun-exposed facial areas in patients with Xeroderma pigmentosum (XP). Commonly seen are the basal cell carcinoma and the squamous cell carcinomas which manifest in the early first decade in contrast to fifth and sixth decade in the general population. XP manifests as photosensitivity, hyperpigmentation, premature skin aging and malignant changes like squamous cell carcinoma, basal cell carcinoma, fibrosarcoma and rarely malignant melanoma as well as internal malignancies. We report 11 cases of Xeroderma pigmentosa managed in our institute which included sex males and five females. All had photosensitivity, hyperpigmentation and consanguinity with facial malignant lesions like SCC and BCC. Ocular signs of photophobia and excessive lacrimation was seen in all the cases while blurring of vision due to corneal clouding, corneal injection, pterygium and limbal SCC were seen in 5 cases. SCC of the lids were seen in 7 cases while BCC seen in 8 cases and limbal and conjunctival SCC seen in one case. All were managed with excision while one case of melanoma with neck secondaries needed radical neck dissection while the other orbital exenteration. Oculo-cutaneous malignancies occur in the sun exposed areas so patients are advised regular follow up with speciality care. Awareness about the rare condition and importance of early detection and prevention of UV rays induced skin damage should be propagated. The disease is ultimately fatal, life can be prolonged by simple preventive measures to minimize sun exposure and early detection of the skin lesions and management.

Keywords: Basal cell carcinoma; Photosensitivity; Squamous cell carcinoma; UV radiation.

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Figures

Fig. 1
Fig. 1
Oculocutaneous lesions seen in 11 year old boy
Fig. 2
Fig. 2
SCC of the nose with corneal clouding seen in 8 year old girl
Fig. 3
Fig. 3
Limbal SCC in milder variant of XP
Fig. 4
Fig. 4
Orbital exenteration done
See this image and copyright information in PMC

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