Abstract

Case Report

Juvenile Xanthogranulomatosis in a Hemophilic Boy: Case Report

K Belcadi*, Z Isfaoun, O EL-Athmani, I El-Abdallaoui, IN Ansari, M Lakhrissi, A Guindo, M El-Kababri, A Kili, L Hessissen, S Sefiani and M El-Khorassani

Published: 15 May, 2024 | Volume 7 - Issue 1 | Pages: 062-065

Juvenile Xanthogranulomatosis (JXG) is a condition, characterized by a proliferation of histiocytes, primarily observed in infants and young children. Cutaneous manifestations appear as yellow-orange-brown papules or nodules, typically localized on the face, neck, and upper chest. While most lesions regress spontaneously, some may require intervention for aesthetic, diagnostic, or hemorrhagic reasons. 
A rare case of disseminated JXG in a child with hemophilia has been reported. In this patient with severe hemophilia A, cutaneous nodules appeared, some associated with bleeding requiring appropriate management. Treatment included the administration of factor VIII to prevent bleeding during surgical procedures and secondary prophylaxis, to control recurrent bleeding. The outcome was favorable with the disappearance of the cutaneous lesions without sequelae, under regular surveillance for both medical conditions. 
This case highlights the rare association between juvenile xanthogranulomatosis (JXG) and hemophilia, a combination that has never been documented in the medical literature. This association only impacts the management of JXG when the cutaneous lesions bleed and their excision becomes necessary.

Read Full Article HTML DOI: 10.29328/journal.japch.1001069 Cite this Article Read Full Article PDF

Keywords:

Xanthogranulomatosis; Hemophilia; Histiocytosis

References

  1. Ribeiro PH, Fritsch LN, Fernandes GJ, Oliveira AM, Ribeiro FB. Upper eyelid juvenile xanthogranuloma: a case report. Rev Bras Oftalmol. 2021; 80(5):e0030.
  2. Collie JS, Harper CD, Fillman EP. Juvenile Xanthogranuloma. 2023 Aug 8. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan–. PMID: 30252359.
  3. Emile JF, Charlotte F, Chassagne-Clement C, Copin MC, Fraitag S, Mokhtari K, Moreau A. Classification histologique et altérations moléculaires des histiocytoses [Histiocytoses: General classification and molecular criteria]. Presse Med. 2017 Jan;46(1):46-54. French. doi: 10.1016/j.lpm.2016.01.016. Epub 2016 Mar 4. PMID: 26952138.
  4. Hernández-San Martín MJ, Vargas-Mora P, Aranibar L. Juvenile Xanthogranuloma: An Entity With a Wide Clinical Spectrum. Actas Dermosifiliogr (Engl Ed). 2020 Nov;111(9):725-733. English, Spanish. doi: 10.1016/j.ad.2020.07.004. Epub 2020 Jul 25. PMID: 32721389.
  5. What Is a Juvenile Xanthogranuloma? Pediatr Dermatol. 2022 Jan;39(1):119-120. doi: 10.1111/pde.14920. PMID: 35106819.
  6. Zou T, Wei A, Ma H, Lian H, Liu Y, Wang D, Zhao Y, Cui L, Li Z, Zhang R, Wang T. Systemic juvenile xanthogranuloma: A systematic review. Pediatr Blood Cancer. 2023 May;70(5):e30232. doi: 10.1002/pbc.30232. Epub 2023 Feb 13. PMID: 36779547.
  7. Höck M, Zelger B, Schweigmann G, Brunner B, Zelger B, Kropshofer G, Kiechl-Kohlendorfer U. The various clinical spectra of juvenile xanthogranuloma: imaging for two case reports and review of the literature. BMC Pediatr. 2019 Apr 24;19(1):128. doi: 10.1186/s12887-019-1490-y. PMID: 31018833; PMCID: PMC6482499.
  8. Puerta P, Candela S, Rovira C, García Fructuoso G. Juvenile xanthogranuloma of skull base. Case report and review of the literature. Acta Neurochir (Wien). 2013 Jun;155(6):1039-40. doi: 10.1007/s00701-013-1718-9. Epub 2013 Apr 21. PMID: 23605373.
  9. Burgdorf WH, Zelger B. JXG, NF1, and JMML: alphabet soup or a clinical issue? Pediatr Dermatol. 2004 Mar-Apr;21(2):174-6. doi: 10.1111/j.0736-8046.2004.21219x. PMID: 15078363.

Figures:

Similar Articles

  • A girl with a stiff neck
    G Carlone*, A Prisco, F Vittoria, E Barbi and M Carbone G Carlone*,A Prisco,F Vittoria,E Barbi,M Carbone. A girl with a stiff neck. . 2020 doi: 10.29328/journal.japch.1001019; 3: 058-060
  • Juvenile Xanthogranulomatosis in a Hemophilic Boy: Case Report
    K Belcadi*, Z Isfaoun, O EL-Athmani, I El-Abdallaoui, IN Ansari, M Lakhrissi, A Guindo, M El-Kababri, A Kili, L Hessissen, S Sefiani and M El-Khorassani K Belcadi*, Z Isfaoun, O EL-Athmani, I El-Abdallaoui, IN Ansari, M Lakhrissi, A Guindo, M El-Kababri, A Kili, L Hessissen, S Sefiani, M El-Khorassani. Juvenile Xanthogranulomatosis in a Hemophilic Boy: Case Report. . 2024 doi: 10.29328/journal.japch.1001069; 7: 062-065

Recently Viewed

  • Environmental Factors Affecting the Concentration of DNA in Blood and Saliva Stains: A Review
    Divya Khorwal*, GK Mathur, Umema Ahmed and SS Daga Divya Khorwal*, GK Mathur, Umema Ahmed, SS Daga. Environmental Factors Affecting the Concentration of DNA in Blood and Saliva Stains: A Review. J Forensic Sci Res. 2024: doi: 10.29328/journal.jfsr.1001057; 8: 009-015
  • Why Down-managing Backlog Forensic DNA Case Entries Matters
    JH Smith* and JS Horne JH Smith*, JS Horne. Why Down-managing Backlog Forensic DNA Case Entries Matters. J Forensic Sci Res. 2024: doi: 10.29328/journal.jfsr.1001056; 8: 001-008
  • Scintigraphic non-invasive diagnosis of amyloid cardiomyopathy
    Laroussi Mohamed-Salem*, Tomás E Rodríguez-Locarno, Tatiana Moreno-Monsalve, Isabel Castellón-Sánchez, José F Contreras-Gutiérrez and Antonia Claver-Valderas Laroussi Mohamed-Salem*,Tomás E Rodríguez-Locarno,Tatiana Moreno-Monsalve,Isabel Castellón-Sánchez,José F Contreras-Gutiérrez ,Antonia Claver-Valderas. Scintigraphic non-invasive diagnosis of amyloid cardiomyopathy. J Cardiol Cardiovasc Med. 2019: doi: 10.29328/journal.jccm.1001058; 4: 156-158
  • Anomalies of coronary artery origin: About two cases
    Dioum M*, Sarr EM, Manga S, Mingou JS, Diack A, Diop AD, Bindia D, Diagne PA, Sarr AN and Diop IB Dioum M*,Sarr EM,Manga S,Mingou JS,Diack A,Diop AD,Bindia D,Diagne PA,Sarr AN,Diop IB. Anomalies of coronary artery origin: About two cases. J Cardiol Cardiovasc Med. 2019: doi: 10.29328/journal.jccm.1001051; 4: 117-119
  • Late discover of a traumatic cardiac injury: Case report
    Benlafqih C, Bouhdadi H*, Bakkali A, Rhissassi J, Sayah R and Laaroussi M Benlafqih C,Bouhdadi H*,Bakkali A,Rhissassi J,Sayah R,Laaroussi M. Late discover of a traumatic cardiac injury: Case report. J Cardiol Cardiovasc Med. 2019: doi: 10.29328/journal.jccm.1001048; 4: 100-102

Read More

Most Viewed

Read More

Help ?