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Xeroderma Pigmentosa with lid squamous cell carcinoma by Dr Salil K Mandal.MD.Assoc Prof R.I.O M.CH
Xeroderma pigmentosum (XP) is a rare autosomal recessive genetic disorder which starts in early childhood and is characterized by cutaneous photosensitivity, pigmentary changes, photophobia, and propensity for early development of malignancy in sun exposed mucocutaneous and ocular structures. Ocular changes include severe photophobia, conjunctivitis, corneal opacification, conjunctival naevi, epibulbar and lid neoplasms. Xeroderma pigmentosum predominantly affects the UV exposed ocular surface, resulting in eyelid atrophy and malignancy , corneal dryness, exposure keratopathy, and conjunctival tumors.An eight years old Muslim child presented with lower lid mass for last six months .Previously the child was diagnosed as xeroderma pimentosa for the last seven years. According the parents that the initially mass is small for last three months it is growing rapidly, associated with photophobia, lacrimation, conjunctival congestion. Large reddish lid mass measuring 8mm /12mm originated from the conjuctival surface, projected out words involve 2/3rd of the lid Surface is irregular, hard inconsistency, with some heamorraghic spot and blepharo-conjunctivitis. In both eye cornea of the patient had diffuse cellular infiltration.And patient had photophobia and lacrimation and congestion. FNAC from the mass suggest differentiated squamous cell carcinoma.Surgical excision of the followed by lower lid reconstruction by modified check rotational flap under general anesthesia done With one year follow up no such recurrence of the disease.Modified cheek rotational flap was easy and less extensive dissection required to fill up the tissue gape in lower lid
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