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Verruciform Xanthoma in a Patient with Recessive Dystrophic Epidermolysis Bullosa

Verruciform Xanthoma in a Patient with Recessive Dystrophic Epidermolysis Bullosa. Kathie P Huang, MD Anna Bruckner, MD. HPI. 10 year-old boy. Recessive Dystrophic Epidermolysis Bullosa (RDEB). Malnutrition, s/p G-tube Anemia No prior h/o skin cancer.

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Verruciform Xanthoma in a Patient with Recessive Dystrophic Epidermolysis Bullosa

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  1. Verruciform Xanthoma in a Patient with Recessive Dystrophic Epidermolysis Bullosa Kathie P Huang, MD Anna Bruckner, MD

  2. HPI 10 year-old boy Recessive Dystrophic Epidermolysis Bullosa (RDEB) Malnutrition, s/p G-tube Anemia No prior h/o skin cancer Several-year history of an asymptomatic enlarging plaque on the left inguinal area

  3. September 2005

  4. November 2007

  5. November 2007

  6. Epidermal acanthosis Papillomatosis Hyperkeratosis

  7. Neutrophilic infiltrate Papillary dermis with foamy xanthoma cells

  8. Verruciform xanthoma (VX)

  9. Verruciform Xanthoma Presentation • Uncommon benign entity • Primarily found in oral mucosa • Also reported in anogenital and periorificial sites

  10. Verruciform Xanthoma Reported in the following settings • Lymphedema • Pemphigus vulgaris • Graft-versus-host disease • Congenital hemidysplasia with ichthyosiform erythroderma and limb defects syndrome • Recessive Dystrophic Epidermolysis Bullosa

  11. Verruciform Xanthoma Etiology • Poorly understood • Trauma, irritation, and infection are contributing factors • Currently not thought to be related to HPV Khaskhely NM et al Am J Dermatopathol. 2000 Oct;22(5):447-52 Moshin SK et al Am J surg Pathol 1998 Apr;22(4):479-87

  12. Verruciform Xanthoma Origin of Foam Cells • Occurs in normolipemic patients • Increased epidermal lipids in reactive conditions • Degenerating keratinocytes may be the source of lipid droplets in foam cells Ide F et al Oral Diseases 2008 (14), 150-157 Moshin SK et al Am J of Surg Path 1998 (22) 479-487

  13. Parakeratosis Neutrophils Proposed Mechanism Keratinocyte Damage Dendritic Cells Traumatic Event/ Inciting Agent Foam Cells Moshin SK et al, Am J Surg Pathol. 1998 Apr;22(4):479-87

  14. Prior Studies • EB is characterized by skin fragility, recurrent trauma, and impaired wound healing • VX presented as solitary lesions on sacral region, dorsal foot, and posterior neck • Rare case reports of atypia and carcinoma arising in VX • Recommend close follow-up Orpin SD et al, 2008 Clin Derm, in press Murat-Susic S et al, 2007 Int J of Derm, 46, 955-959

  15. Management Patient Course • Treated with imiquimod, topical antibiotics, and topical steroid without improvement • Currently managed with close observation Excision would lead to a non-healing wound

  16. Verruciform Xanthoma Summary • Uncommon association with RDEB • Clinically can mimic SCC • Benign entity, but requires close follow-up

  17. Acknowledgements • Dr. Anna Bruckner • Dr. Uma Sundram

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