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2014 ; 5
(2
): 120-4
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Xeroderma pigmentosum: clinicopathological review of the multiple oculocutaneous
malignancies and complications
#MMPMID25114464
Halkud R
; Shenoy AM
; Naik SM
; Chavan P
; Sidappa KT
; Biswas S
Indian J Surg Oncol
2014[Jun]; 5
(2
): 120-4
PMID25114464
show ga
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.