Spitzoid melanoma in a child with xeroderma pigmentosum
Xeroderma pigmentosum (XP) is an autosomal recessive condition associated with a marked increase in the development of malignancies. We report a 10 year girl with freckles, lentigens and keratosis on the face and neck which had gradually developed from the age of two. History of marked photophobia w...
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Medknow Publications
2019-01-01
|
| Series: | Indian Journal of Paediatric Dermatology |
| Subjects: | |
| Online Access: | http://www.ijpd.in/article.asp?issn=2319-7250;year=2019;volume=20;issue=2;spage=148;epage=150;aulast=Devi |
| _version_ | 1818480020978401280 |
|---|---|
| author | Bijayanti Devi Anup Goswami Y Imlongchaba Angila Dorjee |
| author_facet | Bijayanti Devi Anup Goswami Y Imlongchaba Angila Dorjee |
| author_sort | Bijayanti Devi |
| collection | DOAJ |
| description | Xeroderma pigmentosum (XP) is an autosomal recessive condition associated with a marked increase in the development of malignancies. We report a 10 year girl with freckles, lentigens and keratosis on the face and neck which had gradually developed from the age of two. History of marked photophobia was present. Few months prior she developed single firm, pigmented, non-tender, freely- mobile, sessile nodule over left ala of nose. Multiple frecklings with atrophic hypopigmented patches were distributed all over face and sparsely on neck. Histopathology examination (HPE) from growth over nasal ala revealed malignant tumour composed of spindloid to epithelioid cells arranged in sheets and nests in dermis suggestive of spitzoid melanoma. Fine needle aspiration cytology (FNAC) from left post-auricular lymph nodes revealed high cellularity comprising of many pleomorphic cells in clusters having enlarged irregular hyperchromatic nuclei. Wide local excision was done with nasolabial transposition of flap cover. |
| first_indexed | 2024-12-10T11:17:34Z |
| format | Article |
| id | doaj.art-a70cafadb8004e35b2f246e6aac0fffa |
| institution | Directory Open Access Journal |
| issn | 2319-7250 |
| language | English |
| last_indexed | 2024-12-10T11:17:34Z |
| publishDate | 2019-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Paediatric Dermatology |
| spelling | doaj.art-a70cafadb8004e35b2f246e6aac0fffa2022-12-22T01:51:06ZengWolters Kluwer Medknow PublicationsIndian Journal of Paediatric Dermatology2319-72502019-01-0120214815010.4103/ijpd.IJPD_38_18Spitzoid melanoma in a child with xeroderma pigmentosumBijayanti DeviAnup GoswamiY ImlongchabaAngila DorjeeXeroderma pigmentosum (XP) is an autosomal recessive condition associated with a marked increase in the development of malignancies. We report a 10 year girl with freckles, lentigens and keratosis on the face and neck which had gradually developed from the age of two. History of marked photophobia was present. Few months prior she developed single firm, pigmented, non-tender, freely- mobile, sessile nodule over left ala of nose. Multiple frecklings with atrophic hypopigmented patches were distributed all over face and sparsely on neck. Histopathology examination (HPE) from growth over nasal ala revealed malignant tumour composed of spindloid to epithelioid cells arranged in sheets and nests in dermis suggestive of spitzoid melanoma. Fine needle aspiration cytology (FNAC) from left post-auricular lymph nodes revealed high cellularity comprising of many pleomorphic cells in clusters having enlarged irregular hyperchromatic nuclei. Wide local excision was done with nasolabial transposition of flap cover.http://www.ijpd.in/article.asp?issn=2319-7250;year=2019;volume=20;issue=2;spage=148;epage=150;aulast=DeviSolar lentigensspitzoid melanomaxeroderma pigmentosum |
| spellingShingle | Bijayanti Devi Anup Goswami Y Imlongchaba Angila Dorjee Spitzoid melanoma in a child with xeroderma pigmentosum Indian Journal of Paediatric Dermatology Solar lentigens spitzoid melanoma xeroderma pigmentosum |
| title | Spitzoid melanoma in a child with xeroderma pigmentosum |
| title_full | Spitzoid melanoma in a child with xeroderma pigmentosum |
| title_fullStr | Spitzoid melanoma in a child with xeroderma pigmentosum |
| title_full_unstemmed | Spitzoid melanoma in a child with xeroderma pigmentosum |
| title_short | Spitzoid melanoma in a child with xeroderma pigmentosum |
| title_sort | spitzoid melanoma in a child with xeroderma pigmentosum |
| topic | Solar lentigens spitzoid melanoma xeroderma pigmentosum |
| url | http://www.ijpd.in/article.asp?issn=2319-7250;year=2019;volume=20;issue=2;spage=148;epage=150;aulast=Devi |
| work_keys_str_mv | AT bijayantidevi spitzoidmelanomainachildwithxerodermapigmentosum AT anupgoswami spitzoidmelanomainachildwithxerodermapigmentosum AT yimlongchaba spitzoidmelanomainachildwithxerodermapigmentosum AT angiladorjee spitzoidmelanomainachildwithxerodermapigmentosum |