Multiple cutaneous malignancies in xeroderma pigmentosum
Department of Skin and VD, SB Medical College, Cuttack, Orrisa
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Mohanty P, Mohanty L, Devi B P. Multiple cutaneous malignancies in xeroderma pigmentosum. Indian J Dermatol Venereol Leprol 2001;67:96-97
AbstractA case of xeroderma pigmentosum with multiple cutaneous malignancies is being reported. The case presented with freckles, letigens, and keratosis, a non-tender ulcerated nodular lesion on the nose, a nodular ulcerated lesion on the right outer canthus of the conjunctiva, and a nodular growth which developed on the right cheek which on histopathology was found to be squamous cell cercinoma, basal cell carcinoma and malignant melanoma respectively.
Xeroderma pigmentosum (XP) occurs with an estimated frequency of 1 : 25000 in United States and somehow more common in Japan. Its incidence in the Indian context is not significant. Here is a case report of xeroderma pigmentosum presenting with squamous cell carcinoma, malignant melanoma, and basal cell carcinoma.,
An 18- year- old Muslim male reported with freckles, lentigenes and keratosis on the face, chest back and extremities [Figure - 1] which had gradually developed from the age of two and dimunition of vision and lacrimation of the right eye since six months. Enucleation of the left eye had been done at the age of 16 years. On the nose there was a non- tender indurated lesion on the right outer canthus of conjunctiva. Another nodular pigmented growth occurred over the right cheek within 2 months. Routine haematological and biochemical investigations were normal. On clinical and histo-pathological study the case was diagnosed to be xeroderma pigmentosum with squamous cell carcinoma of the conjunctiva, basal cell carcinoma in the nose and malignant melanoma of the cheek. A sister of the patient was also suffering from xeroderma pigmentosum, but with no cutaneous malignant changes, probably due to her less exposure to U.V. radiation.
Xeroderma pigmentosum under 20 years of age has a greater than 1000 fold increased risk of cutaneous basal cell or squamous cell carcinoma or malignant melanoma. Cases presenting with any two of these malignancies though reported, is also rare, but the presence of all the three types of malignancies is very rare. Though XP patients are more susceptible to UV rays induced neoplasia,,, the patient should adopt to a life style to minimize UV exposure by wearing protective clothing, UV absorbing glasses and long hair styles, in order to reduce the incidence of cutaneous malignant changes.
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