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Research Article |

A Congenital Purple Plaque, a Case Report with the Review of Literature

Dermatofibrosarcoma protruberans (DFSP) is an uncommon, locally aggressive tumour arising. it accounts for 0.1% of all malignancies. Although it can present any time from the neonatal period onwards, paediatric cases are rare, and account for 6% of all DFSP. The cause of dermatofibrosarcoma protuberans is not known but, injury to the skin in the affected location could be a risk factor. It can occur within pre-existing scars and tattoos. Clinical presentation is typically as a solitary plaque or nodule most often on the trunk or extremities. The initial lesion is a skin coloured, slow growing plaque which develops a red /brown colour and nodular surface. but tends to recur after excision. It rarely spreads to other sites beyond the skin. The article presents an overview of epidemiology, distinct clinical evolution, histopathological characteristics, gene involvement and differential diagnosis of DFSP. Management should be multi-disciplinary, with a view to maximising tumour clearance while minimising tissue loss. We present A 3-year-old girl was referred to dermatology for a slow-growing painless lesion on her left lower abdomen. The lesion had been present since birth. At ten months-of-age a paediatrician made a clinical diagnosis of infantile haemangioma. Examination of the skin of the left lower abdominal quadrant revealed a 5×3 cm ill-defined pink plaque containing multiple discrete violaceous nodules. Histology revealed a dermal infiltrate of monomorphic spindled cells arranged in a storiform pattern with no cytological atypia, Immunohistochemistry staining was positive for CD34. Following multi-disciplinary review, she was referred to plastic surgery for wide local excision. The indolent behaviour of early DFSP can lead to a delay in diagnosis. Also, they are commonly misdiagnosed as vascular malformations children. It is recommended that patients undergo surveillance for local recurrence for longer than five years following primary excision.

Dermatofibrosarcoma Protruberans, DFSP, Plaque, Fibroblastic, Immunohistochemistry, Moh’s Micrographic Surgery, Spindle Shape Cells, CD34

APA Style

Nasreen, T., Lynch, F., Law, R., Collins, S. (2023). A Congenital Purple Plaque, a Case Report with the Review of Literature. International Journal of Clinical Dermatology, 6(2), 29-32. https://doi.org/10.11648/j.ijcd.20230602.14

ACS Style

Nasreen, T.; Lynch, F.; Law, R.; Collins, S. A Congenital Purple Plaque, a Case Report with the Review of Literature. Int. J. Clin. Dermatol. 2023, 6(2), 29-32. doi: 10.11648/j.ijcd.20230602.14

AMA Style

Nasreen T, Lynch F, Law R, Collins S. A Congenital Purple Plaque, a Case Report with the Review of Literature. Int J Clin Dermatol. 2023;6(2):29-32. doi: 10.11648/j.ijcd.20230602.14

Copyright © 2023 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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