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Table of Contents
Year : 2020  |  Volume : 38  |  Issue : 3  |  Page : 186-187

Verruciform xanthoma in recessive dystrophic epidermolysis bullosa

1 Department of Dermatology, National Taiwan University Hospital, Taipei, Taiwan
2 Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan

Date of Submission16-Sep-2019
Date of Decision20-Feb-2020
Date of Acceptance01-Mar-2020
Date of Web Publication03-Jun-2020

Correspondence Address:
Dr. Hsien-Ching Chiu
Department of Dermatology, National Taiwan University Hospital, Taipei
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ds.ds_6_20

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How to cite this article:
Chiang CY, Chiu TS, Liau JY, Chiu HC. Verruciform xanthoma in recessive dystrophic epidermolysis bullosa. Dermatol Sin 2020;38:186-7

How to cite this URL:
Chiang CY, Chiu TS, Liau JY, Chiu HC. Verruciform xanthoma in recessive dystrophic epidermolysis bullosa. Dermatol Sin [serial online] 2020 [cited 2022 Dec 2];38:186-7. Available from: https://www.dermsinica.org/text.asp?2020/38/3/186/285720

Dear Editor,

We present a case of verruciform xanthoma (VX) in recessive dystrophic epidermolysis bullosa (RDEB). A 17-year-old female suffering from RDEB had been regularly followed at the Dermatology Department of National Taiwan University Hospital. She complained of an asymptomatic, chronic wound on her left knee for around 2 years. On physical examination, there was a 2-cm, well-demarcated, orange-red, verrucous plaque on the left knee [Figure 1]a. Squamous cell carcinoma (SCC) was suspected, and a fusiform skin biopsy was performed in the central part of the lesion. Microscopic examination revealed significant epidermal verrucous hyperplasia, elongated rete ridges, and clusters of foamy macrophages in the dermal papillae [Figure 1]b, [Figure 1]c, [Figure 1]d. The immunohistochemistry staining revealed that those foamy macrophages were positive for CD68 [Figure 1]e. There was no koilocyte or atypical keratinocyte. Based on the histopathological and immunohistochemical findings, a diagnosis of VX was established. Because of the benign nature of VX, the patient was kept under observation.
Figure 1: (a) One erythematous, well-circumscribed, verrucous plaque on the left knee. (b) A low power pathological view of the verrucous plaque revealing verrucous epidermal hyperplasia (H and E, ×40), (c) a moderate power pathological view showing elongated rete ridges (×100), (d) a high power view showing aggregation of foamy macrophages in the dermal papillae (×400), (e) immunohistochemistry stain showing foamy macrophages positive for CD68 (×400)

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VX, which was first reported by Shafer in 1971, usually appears as a solitary, well-demarcated, yellowish to erythematous, verrucous plaque or pedunculated tumor.[1] It typically occurs on the oral mucosa, and the most common extraoral location is the anogenital area. The mean age of patients with VX is 50 years, and there is a slight male predominance.[1],[2] The typical histological findings of VX are acanthosis, hyperkeratosis, parakeratosis, uniformly elongated rete ridges, and aggregation of foamy macrophages in the dermal papilla.[3]

The etiology of VX remains unclear. Due to the verrucous surface of VX, human papillomavirus infection was suspected as a possible cause. However, the results of different studies were controversial.[4],[5] Some authors proposed that the formation of VX may be a reactive change to local physical irritation and epithelial damage, with the damaged epithelial cells releasing lipid material and the dermal cells engulfing it to form foamy macrophages in the dermal papillae.[4] Different modalities have been tried to treat VX, and complete surgical excision showed the lowest recurrence rate.[5],[6] Other successful treatment choices include CO2 laser ablation and 5% imiquimod cream.

We have completed a comprehensive review of the English literature regarding VX in RDEB, and only six cases have been reported. The mean age of RDEB patients with VX (7 cases, including the present case) is 20 years [Table 1],[2],[7],[8],[9],[10],[11] much younger than the general VX patient group. The possible reason of younger prevalence may be due to skin fragility, frequent trauma, and impaired wound healing in RDEB patients.[2] There is no obvious gender preference (3 males vs. 4 females) [Table 1].
Table 1: A review of verruciform xanthoma in recessive dystrophic epidermolysis bullosa patients

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Because VX can appear as an acquired, quickly enlarging, verrucous, or ulcerative plaque in the eroded skin areas, a skin biopsy must be done to rule out the possibility of SCC. Although VX is thought to be a benign tumor, rare case reports have shown that SCC coexisted with or developed from it.[12],[13] Since RDEB patients are prone to develop cutaneous SCC, a cautious watch for atypical keratinocytes or early SCC hidden in the background of VX is mandatory. Differing from other patients, RDEB patients seldom received surgical excision for the VX lesion due to skin fragility and impaired wound healing. Only one RDEB patient was reported to receive the operation to remove the VX lesion,[2] while conservative treatments such as observation, topical steroid, or antibiotics were applied for others [Table 1].

In conclusion, VX is a rare, benign tumor, and when occurring in patients with RDEB, is often a mimicker of cutaneous SCC. A skin biopsy is almost always needed to establish the diagnosis. If keratinocyte atypia is present in the specimen of VX, complete surgical excision should be considered to prevent further evolution into SCC. Otherwise, conservative treatment or observation could be taken to prevent inappropriate aggressive intervention in RDEB patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Shafer WG. Verruciform xanthoma. Oral Surg Oral Med Oral Pathol 1971;31:784-9.  Back to cited text no. 1
Curto-Barredo L, Segura S, Barranco C, Bellosillo B, Lloreta J, Toll A, et al. Verruciform xanthoma developing in recessive dystrophic epidermolysis bullosa: A sheep in wolf's clothing. Am J Dermatopathol 2014;36:506-9.  Back to cited text no. 2
Al-Nafussi AI, Azzopardi JG, Salm R. Verruciform xanthoma of the skin. Histopathology 1985;9:245-52.  Back to cited text no. 3
Mohsin SK, Lee MW, Amin MB, Stoler MH, Eyzaguirre E, Ma CK, et al. Cutaneous verruciform xanthoma: A report of five cases investigating the etiology and nature of xanthomatous cells. Am J Surg Pathol 1998;22:479-87.  Back to cited text no. 4
Fite C, Plantier F, Dupin N, Avril MF, Moyal-Barracco M. Vulvar verruciform xanthoma: Ten cases associated with lichen sclerosus, lichen planus, or other conditions. Arch Dermatol 2011;147:1087-92.  Back to cited text no. 5
Stiff KM, Cohen PR. Vegas (verruciform genital-associated) xanthoma: A comprehensive literature review. Dermatol Ther (Heidelb) 2017;7:65-79.  Back to cited text no. 6
Cooper TW, Santa Cruz DJ, Bauer EA. Verruciform xanthoma. Occurrence in eroded skin in a patient with recessive dystrophic epidermolysis bullosa. J Am Acad Dermatol 1983;8:463-7.  Back to cited text no. 7
Murat-Susić S, Pastar Z, Dobrić I, Camino Varela A, Hutinec Z, Husar K, et al. Verruciform xanthoma in recessive dystrophic epidermolysis bullosa Hallopeau-Siemens. Int J Dermatol 2007;46:955-9.  Back to cited text no. 8
Orpin SD, Scott IC, Rajaratnam R, Colloby PS, Heagerty A. A rare case of recessive dystrophic epidermolysis bullosa and verruciform xanthoma. Clin Exp Dermatol 2009;34:49-51.  Back to cited text no. 9
Huang KP, Mattoch IW, Sundram UN, Bruckner AL. Warty plaque in a boy with recessive dystrophic epidermolysis bullosa. Pediatr Dermatol 2009;26:747-8.  Back to cited text no. 10
Fuyuno Y, Mitoma C, Ito S, Uchi H, Okura M, Yamashita T, et al. Verruciform xanthoma developing in eroded skin of recessive dystrophic epidermolysis bullosa. Eur J Dermatol 2015;25:509-10.  Back to cited text no. 11
Takiwaki H, Yokota M, Ahsan K, Yokota K, Kurokawa Y, Ogawa I. Squamous cell carcinoma associated with verruciform xanthoma of the penis. Am J Dermatopathol 1996;18:551-4.  Back to cited text no. 12
Mannes KD, Dekle CL, Requena L, Sangueza OP. Verruciform xanthoma associated with squamous cell carcinoma. Am J Dermatopathol 1999;21:66-9.  Back to cited text no. 13


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