Abstract:

Verruciform xanthoma (VX) is a rare lesion that presents as an exophytic, papillary growth in the oral mucosa or genitals, particularly the scrotum, typically in adults. A number of case reports describe VX in young patients with recessive dystrophic epidermolysis bullosa (RDEB), where it is thought to be caused by trauma or inflammation. As the literature is limited, the aim of this study was to evaluate the clinicopathologic features of a series of VX occurring in the setting of RDEB. We identified a total of 41 VX from our internal clinics over the past 20 years. A total of 11 biopsies of VX were from 4 patients with RDEB. Available pathologic material was reviewed. Additional clinical and outcomes data were pooled from the electronic medical record. We identified 11 VX occurring in 4 patients with RDEB. Anatomic sites involved included knee (1), buttock (1), calf (1), back (1), upper groin (1), lower abdomen (1), and thigh (4). Median age at time of diagnosis was 18.5 years (range 13 – 44). Clinical impressions included SCC (8), VV (5), VX (4), granulation tissue (2), infection (1), verrucous carcinoma (1), cutaneous horn (1). Median clinical size of lesions was 5 cm (range 2.5 – 5). All patients were male. Histologic examination of revealed skin with acanthosis associated with broad epidermal ridges and overlying parakeratosis with serum crust and neutrophils. Foamy histiocytes (xanthoma cells) were present in the papillary tips. A subepidermal split was noted in two cases. A subjacent inflammatory infiltrate was noted in two cases. Secondary impetiginization was noted in one case. Local recurrence occurred in one of the lesions (site: thigh). Here we present the largest series to date of VX in patients with RDEB. , Although the pathogenesis of VX is poorly understood, chronic inflammation and trauma due to skin fragility in RDEB may account for the occurrence of VX within this population. Awareness of the potential for local recurrence is merited.

Financial Disclosure:
No current or relevant financial relationships exist.

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Kathleen Sheahon, MD