Transcript Photo Album - Pacific Dermatologic Association

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
Several-year history of an asymptomatic
enlarging plaque on the left inguinal area
September 2005
November 2007
November 2007
Epidermal acanthosis
Papillomatosis
Hyperkeratosis
Neutrophilic infiltrate
Papillary dermis with foamy xanthoma cells
Verruciform xanthoma (VX)
Verruciform Xanthoma
Presentation
• Uncommon benign entity
• Primarily found in oral mucosa
Also reported in anogenital and periorificial
sites
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
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
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
Proposed Mechanism
Parakeratosis
Neutrophils
Keratinocyte
Damage
Dendritic
Cells
Traumatic Event/
Inciting Agent
Foam Cells
Moshin SK et al, Am J Surg Pathol. 1998 Apr;22(4):479-87
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
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
Verruciform Xanthoma
Summary
• Uncommon association with RDEB
• Clinically can mimic SCC
• Benign entity, but requires close follow-up
Acknowledgements
• Dr. Anna Bruckner
• Dr. Uma Sundram