IADVL
Indexed with PubMed and Science Citation Index (E) 
 
Users online: 6699 
     Home | Feedback | Login 
About Current Issue Archive Ahead of print Search Instructions Online Submission Subscribe What's New Contact  
  Navigate here 
  Search
 
   Next article
   Previous article 
   Table of Contents
  
 Resource links
   Similar in PUBMED
    Search Pubmed for
    Search in Google Scholar for
  Related articles
   [PDF Not available] *
   Citation Manager
   Access Statistics
   Reader Comments
   Email Alert *
   Add to My List *
* Registration required (free)  

 
  In this article
    Abstract
    Introduction
    Case Report
    Discussion
    References

 Article Access Statistics
    Viewed4758    
    Printed94    
    Emailed7    
    PDF Downloaded0    
    Comments [Add]    

Recommend this journal

 
CASE REPORT
Year : 2001  |  Volume : 67  |  Issue : 2  |  Page : 96-97

Multiple cutaneous malignancies in xeroderma pigmentosum


Department of Skin and VD, SB Medical College, Cuttack, Orrisa, India

Correspondence Address:
Department of Skin and VD, SB Medical College, Cuttack, Orrisa, India

   Abstract 

A 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.

How to cite this article:
Mohanty P, Mohanty L, Devi B P. Multiple cutaneous malignancies in xeroderma pigmentosum. Indian J Dermatol Venereol Leprol 2001;67:96-7


How to cite this URL:
Mohanty P, Mohanty L, Devi B P. Multiple cutaneous malignancies in xeroderma pigmentosum. Indian J Dermatol Venereol Leprol [serial online] 2001 [cited 2019 Aug 20];67:96-7. Available from: http://www.ijdvl.com/text.asp?2001/67/2/96/11118



   Introduction Top

Xeroderma pigmentosum (XP) occurs with an estimated frequency of 1 : 25000 in United States and somehow more common in Japan.[1] 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.[2],[3]

   Case Report Top

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 histo­pathological 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.

   Discussion Top

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,[1],[2],[3] 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.[4] 

   References Top

1.Robbins JH, Kraemer KH, Lutzner M A, et al. Xeroderma pigmentosum, an inherited disease with sun sensitivity, multiple cutaneous neoplasia and abnormal DNA repair. Ann inter Med. 1974; 80 : 221- 248.  Back to cited text no. 1    
2.Clark Lambert W. Genetic disease associated with DNA and chromosomal instability . In : Alpor IC ed. Dermatological Clinics. The Genodermatores Vol.5, No.1 Philadelphia : W.B. Sunders, 1987; 85-108.  Back to cited text no. 2    
3.Cleaver JE. DNA damage and repair in light sensitive human skin. 3 Invest Dermatol 1970;54:181-195.  Back to cited text no. 3    
4.Kramer KH. Xeroderma pigmentosum, in: Demis DJ, Dobson RL, Mc Guire J., eds. Clinical Dermatology, Vol.4, Unit-19-7. Hagerstown, Maryland : Harper & Row, 1980 a : 1-33.  Back to cited text no. 4    

 

Top
Print this article  Email this article
Previous article Next article

    

Online since 15th March '04
Published by Wolters Kluwer - Medknow