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      Atypical Fibroxanthoma: An Unusual Skin Neoplasm in Xeroderma Pigmentosum

      case-report

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          Abstract

          Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder related to defective deoxyribonucleic acid (DNA) repair. Various cutaneous manifestations related to ultraviolet (UV) damage characterize the clinical course. Primary malignant cutaneous neoplasms like squamous cell carcinoma, basal cell carcinoma and malignant melanoma have been reported. Atypical fibroxanthoma is a rare dermal neoplasm occurring in UV-damaged skin. We report an unusual case of atypical fibroxanthoma in a 20-year-old male with XP.

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          Most cited references15

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          Defective repair replication of DNA in xeroderma pigmentosum.

          J Cleaver (1968)
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            Xeroderma pigmentosum. Cutaneous, ocular, and neurologic abnormalities in 830 published cases.

            Carrie Scotto, K. Kraemer, Moon-Kyu Lee (1987)
            Quantitative frequencies of clinical abnormalities in xeroderma pigmentosum were estimated by abstracting published descriptions of 830 patients in 297 articles obtained from a survey of the medical literature from 1874 to 1982. The median patient age was 12 years with nearly equal numbers of male and female patients. Cutaneous symptoms (sun sensitivity or freckling) had a median age of onset of between 1 and 2 years. Forty-five percent of the patients described had basal cell carcinoma or squamous cell carcinoma of the skin. The median age of first nonmelanoma skin cancer among patients with xeroderma pigmentosum was 8 years, more than 50 years less than that among patients with skin cancer in the United States. Melanomas were reported in 5% of patients. Ninety-seven percent of the reported basal and squamous cell carcinomas and 65% of the melanomas in patients with xeroderma pigmentosum occurred on the face, head, or neck. Seventy percent probability of survival was attained at age 40 years, a 28-year reduction in comparison with the US general population. Ocular abnormalities were reported in 40% of the patients described and were restricted to tissues exposed to ultraviolet radiation (lid, conjunctiva, and cornea) and included ectropion, corneal opacity leading to blindness, and neoplasms. Neurologic abnormalities were found in 18% of the cases reported, consisting of progressive mental deterioration, hyporeflexia or areflexia, and progressive deafness in some patients in association with dwarfism and immature sexual development. There was scant information concerning the efficacy of any therapeutic regimen.
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              Oculocutaneous manifestations in xeroderma pigmentosa.

              Ganesh K. Agrawal, J Goyal, Sathanur R. Srinivasan … (1994)
              Xeroderma pigmentosum (XP) is a rare genetic disease characterised by defective DNA repair leading to clinical and cellular hypersensitivity to ultraviolet radiation. The oculocutaneous features of 10 patients with XP were studied retrospectively. General features included parental consanguinity (40%), familiarity (60%), onset of symptoms in first 2 years (50%), malignant skin neoplasms (60%), and carcinoma of the tongue (20%). Among the ocular features, 50% of patients presented with photophobia. Lid freckles or atrophic skin lesions were seen in all patients. Lower lid tumours were seen in 30%, chronic conjunctival congestion in 40%, corneal opacification in 40%, squamous cell carcinoma of limbus in 20%, bilateral pterygium in 40%, and visual impairment in 50%. The clinical features (ocular and cutaneous) of the cases are discussed.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Indian J Dermatol
                Journal ID (iso-abbrev): Indian J Dermatol
                Journal ID (publisher-id): IJD
                Title: Indian Journal of Dermatology
                Publisher: Medknow Publications & Media Pvt Ltd (India )
                ISSN (Print): 0019-5154
                ISSN (Electronic): 1998-3611
                Publication date (Print): Sep-Oct 2012
                Volume: 57
                Issue: 5
                Pages: 384-386
                Affiliations
                [1] From the Department of Pathology, Burdwan Medical College, West Bengal, India
                [1 ] Department of Pathology, Medical College, Kolkata, India
                [2 ] Department of Gastroenterology, Institute of Postgraduate Medical Education & Research, Kolkata, India
                Author notes
                Address for correspondence: Dr. Ranjana Bandyopadhyay, 1B/3, Uttarpara Housing Estate, 88 B, G T Road, P.O.- Bhadrakali, Dist- Hooghly – 712 232, West Bengal, India. E-mail: drranjanapatho@ 123456gmail.com
                Article
                Publisher ID: IJD-57-384
                DOI: 10.4103/0019-5154.100493
                PMC ID: 3482803
                PubMed ID: 23112360
                SO-VID: 92be6aae-0b14-441c-8b52-2b4466435d5c
                Copyright © Copyright: © Indian Journal of Dermatology
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : August 2010
                Date accepted : November 2010
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Dermatology
                Keywords: cutaneous neoplasm,xeroderma pigmentosum,atypical fibroxanthoma
                Data availability:
                ScienceOpen disciplines: Dermatology
                Keywords: cutaneous neoplasm, xeroderma pigmentosum, atypical fibroxanthoma

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