International Journal of Clinical Dermatology

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

Received: 30 August 2023    Accepted: 20 October 2023    Published: 9 November 2023
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Abstract

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.

DOI 10.11648/j.ijcd.20230602.14
Published in International Journal of Clinical Dermatology (Volume 6, Issue 2, December 2023)
Page(s) 29-32
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

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

References
[1] Foroozan M, Sei JF, Amini M, Beauchet A, Saiag P. Efficacy of Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans: systematic review. Arch Dermatol. 2012; 148 (9): 1055-63.
[2] Brennan B, Zanetti I, De Salvo GL, Orbach D, Gallego S, Francotte N, et al. Dermatofibrosarcoma protuberans in children and adolescents: The European Paediatric Soft Tissue Sarcoma Study Group prospective trial (EpSSG NRSTS 2005). Pediatr Blood Cancer. 2020; 67 (10): e28351.
[3] Loeb DM, Thornton K, Shokek O. Pediatric soft tissue sarcomas. Surg Clin North Am. 2008; 88 (3): 615-27, vii.
[4] Jafarian F, McCuaig C, Kokta V, Laberge L, Ben Nejma B. Dermatofibrosarcoma protuberans in childhood and adolescence: report of eight patients. Pediatr Dermatol. 2008; 25 (3): 317-25.
[5] Sleiwah A, Psomadakis C, Craythorne E, Stefanato CM, Rickaby W, Robson A, et al. Dermatofibrosarcoma protuberans (DFSP) in children: A combined multidisciplinary approach. Pediatr Dermatol. 2021; 38 (1): 233-6.
[6] Somayeh Sgeidaei, Mahsa Salehi, Faremah Abedian Kenari and hamid reza Jafari. Dermatofibrosarcoma protuberans chalenges; a case series and review of literasture. Volume 17, article number 18 (2023).
[7] Darier J. Dermatofibromes progressifs et recidivants ou fibrosarcomes de la peu. Ann Dermatol Syphiligr (Paris) 1924; 5: 542–562.
[8] Aseel Sleiwah, Thomas C wright, Thomas Chapman, Adam Dangoor. Francesca Maggiani, Rachel Clancy. Current treatment options in oncology, volume 23, pages 843-854 (2022).
[9] Gloster HM Jr. Dermatofibrosarcoma protuberans. J Am Acad Dermatol. 1996; 35: 355–374. doi: 10.1016/S0190-9622(96)90597-6. quiz 375–356. Geisinger Health System, Last Update: April 14, 2023. dermatofibroma protrubans.
[10] Fiore M, Miceli R, Mussi C. Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate. J Clin Oncol. 2005; 23: 7669–7675. doi: 10.1200/JCO.2005.02.5122.
[11] Lautier R, Wolff HH, Jones RE An immunohistochemical study of dermatofibrosarcoma protuberans supports its fibroblastic character and contradicts neuroectodermal or histiocytic components. Am J Dermatopathol. 1990; 12: 25-30.
[12] Brabant B, Revol M, Vergote T, Servant JM, Banzet P. Dermatofibrosarcoma protuberans of the chest and the shoulder: wide and deep excisions with immediate reconstruction. Plast Reconstr Surg. 1993; 92: 459–462. doi: 10.1097/00006534-199309000-00012.
[13] Ugurel S. Targeted therapy of dermatofibrosarcoma with imatinib. J Dtsch Dermatol Ges. 2007; 5: 261.
Cite This Article
  • 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

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    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

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    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

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  • @article{10.11648/j.ijcd.20230602.14,
      author = {Tayyiba Nasreen and Fiona Lynch and Ruth Law and Sinead Collins},
      title = {A Congenital Purple Plaque, a Case Report with the Review of Literature},
      journal = {International Journal of Clinical Dermatology},
      volume = {6},
      number = {2},
      pages = {29-32},
      doi = {10.11648/j.ijcd.20230602.14},
      url = {https://doi.org/10.11648/j.ijcd.20230602.14},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijcd.20230602.14},
      abstract = {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.
    },
     year = {2023}
    }
    

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    AB  - 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.
    
    VL  - 6
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Author Information
  • Department of Dermatology, Our Lady of Lourdes Hospital, Drogheda, Ireland

  • Department of Dermatology, Our Lady of Lourdes Hospital, Drogheda, Ireland

  • Department of Histopathology, Our Lady of Lourdes Hospital, Drogheda, Ireland

  • Department of Dermatology, Our Lady of Lourdes Hospital, Drogheda, Ireland

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