OBSERVATION ONLINE FIRST Vulvar Verruciform Xanthoma Ten Cases Associated With Lichen Sclerosus, Lichen Planus, or Other Conditions Charlotte Fite, MD; Franc¸oise Plantier, MD; Nicolas Dupin, MD, PhD; Marie-Franc¸oise Avril, MD; Micheline Moyal-Barracco, MD Background: Verruciform xanthoma (VX) is a rare be- acanthosis without atypia, and elongated rete ridges. nign tumor that usually involves the oral cavity. Since Xanthomatous cells were aggregated in the papillary the first report of this tumor in 1971, only 9 cases have dermis. been reported on the vulva, and 3 of these were associ- ated with another vulvar condition. We describe the clini- Conclusions: Vulvar VX is a benign tumor with mis- copathologic features of 10 patients with vulvar VX and leading clinical features. All 10 cases were associated with focus on their associated conditions. a vulvar condition, mainly a lichen sclerosus. There- fore, VX might represent a reaction pattern induced by Observation: The mean age of the patients was 68 years different conditions, mainly characterized by damage to (range, 51-80 years). The VX lesions were asymptom- the dermoepidermal junction. When confronted with the atic, yellowish-orange verrucous plaques. The diagno- diagnosis of vulvar VX, clinicians may look for an asso- sis was clinically suspected in 2 cases; other suggested ciated vulvar condition. diagnoses were condyloma or squamous cell carci- noma. All of the patients had an associated vulvar con- dition: lichen sclerosus (6 patients), lichen planus (2 Arch Dermatol. 2011;147(9):1087-1092. patients), Paget disease, or radiodermatitis. Under mi- Published online May 16, 2011. croscopy, the VX lesions displayed parakeratosis, doi:10.1001/archdermatol.2011.113 ERRUCIFORM XANTHOMA location, histologic findings, history of dyslip- (VX) is a rare benign tu- idemia, treatment, follow-up, and associated mor which was first vulvar conditions. described in the oral cav- ity by Shafer in 1971.1 RESULTS VOnly a few cases of vulvar VX have been reported since its first description in CLINICAL DATA 1979,2-8 and the pathogenesis of this con- dition remains unclear. Anecdotal cases of During the study period, 10 patients (mean mucosal or skin VX associated with an un- age, 68 years [range, 51-80 years]) were reg- derlying disorder have been reported, and istered with a diagnosis of VX (Table 1), it has been suggested that oral VX might which presented as a single lesion in 9 of the be secondary to an underlying inflamma- cases and as multiple lesions in 1 case. Le- 9 tory disorder. Herein, we describe the sions were asymptomatic, slow-growing, clinicopathologic features of 10 cases of sharply demarcated, indurated, yellowish- vulvar VX and focus on their associated orange verrucous plaques (Figure 1 and vulvar conditions. Figure 2).Thesizeofthelesionsrangedfrom 2 to 20 mm. Three of the lesions were located METHODS on the labia minora, 3 were on the labia ma- jora, 2 were on the clitoris, and 2 were on the fourchette. One patient had 3 concomitant All consecutive cases of vulvar VX encoun- VX lesions, all located on the labia minora. Author Affiliations: tered between February 1989 and March 2010 Three patients complained of vulvar itching Departments of Dermatology by 1 pathologist (F.P.) working in 2 public hos- related to lichen sclerosus (2 patients) and (Drs Fite, Dupin, Avril and pitals of Ile de France and a private pathology Pagetdisease(1patient).DiagnosisofVXwas Moyal-Barracco) and Pathology center were listed. Biopsy specimens were fixed (Dr Plantier), Assistance in acetic-formaldehyde-alcohol solution, par- clinicallysuspectedinonly2cases,othersug- Publique des Hoˆpitaux de Paris, affin embedded, and routinely processed. For gested diagnoses being condyloma (3 pa- Hospital Cochin, Paris each case, we retrospectively collected the fol- tients), squamous cell carcinoma (SCC) (2 Descartes University, lowing information from the patients’ medi- patients), or “leucoplasia” (1 patient). In 2 Paris, France. cal records: age, clinical description of VX, cases, there was no recorded diagnosis but ARCH DERMATOL/ VOL 147 (NO. 9), SEP 2011 WWW.ARCHDERMATOL.COM 1087 ©2011 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/29/2021 Table 1. Characteristics of the Vulvar Verruciform Xanthoma (VX) of the Present Series Recurrence/ Patient No./ Vulvar Associated Clinical Description History of Follow-up, Age, y Conditiona or Hypothesis Diameter, mm Location No. Dyslipidemia mo 1/75 LS clinically obvious Condyloma 10 Fourchette Unique No NA 2/80 Vulvar Paget disease None 2 Labia majora Unique Yes No/14 3/77 LS “Keratotic papule”b 2 Clitoris Unique Yes NA 4/63 LS Condyloma, SCC, VX 5 Labia minora Unique No No/17 5/51 LP “Verrucous lesion”b NA Labia minora Unique No No/108 6/51 LS VX 4 Clitoris Unique No No/60 7/57 LS SCC 20 Labia minora Multiple Yes NA/died 8/77 LP Condyloma 15 Labia majora Unique No Yes/96 9/79 Radiodermatitis None 3 Fourchette Unique Yes NA 10/73 LS “Leucoplasia”b 4 Labia majora Unique No NA Abbreviations: LP, lichen planus; LS, lichen sclerosus; NA, not available; SCC, squamous cell carcinoma. a There was histologic confirmation in all patients listed except patient 1. b Clinical description is given rather than diagnosis. Figure 1. Clinical photograph of a condyloma-like verruciform xanthoma of Figure 2. Clinical photograph of a condyloma-like verruciform xanthoma of the left labia minora associated with a lichen sclerosus (pallor, architectural the right labia minora associated with an erosive lichen planus (erosions, changes). architectural changes). only the clinical descriptions “keratotic papule” and “ver- pathologically confirmed in 7 cases. One patient had la- rucous lesion.” ser ablation, but the lesion recurred 16 months later, and All of the patients had an associated vulvar condition: she was subsequently treated with surgery. However, re- lichen sclerosus (6 patients), lichen planus (2 patients), moval was incomplete, and the VX recurred 2 years later. Paget disease (1 patient), or radiodermatitis consecutive to Two patients were not treated by surgery; both of these cervical cancer treatment (1 patient). All of the associated were lost to follow-up, and we were informed that 1 of diagnoses were histologically confirmed except for 1 case them had died of myocardial infarction at the age of 63 of lichen sclerosus that was clinically obvious. Indeed, this years, 6 years after diagnosis of VX. The mean duration patient was diagnosed as having chronic recurrent vulvar of follow-up for 4 of the patients was 48 months (range, pruritus with typical architectural changes in the vulva. 14-108 months); 5 of the patients were lost to follow- In 5 cases, VX was diagnosed in patients whose asso- up. In 1 patient, local recurrence occurred first within ciated condition had been previously followed up for a 16 months after laser destruction of the lesion and then mean period of 30 months (range, 24-48 months). For 2 years after the surgical procedure. 2 patients, VX and lichen sclerosus were diagnosed si- multaneously. The time delay between the respective di- PATHOLOGIC RESULTS agnosis of VX and the associated vulvar condition was unavailable for the last 3 patients. A medical history of Pathologic examinations were performed in specimens high lipid levels was reported in 4 patients. Of these, 2 from 4 partial biopsies and in 8 surgical samples after ex- patients were receiving specific treatment. cision. The VX lesions were usually well demarcated from Verruciform xanthoma was unresponsive to topical the adjacent normal epithelium. The epithelium showed steroids prescribed for lichen sclerosus (in 5 patients). hyperkeratosis, acanthosis, and elongation of the rete Surgical excision of the VX was offered to and per- ridges (Figure 3). A wedge-shaped hyperkeratosis formed formed in 8 patients. Complete removal of the lesion was invaginating crypts extending deep into the acanthotic ARCH DERMATOL/ VOL 147 (NO. 9), SEP 2011 WWW.ARCHDERMATOL.COM 1088 ©2011 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/29/2021 Figure 3. Characteristic wedge-shaped parakeratosis with an orange hue and Figure 4. High magnification shows an inflammatory infiltrate of neutrophils, xanthomatous cells aggregated between the rete ridges (hematoxylin-eosin, with microabcesses at the junction with the parakeratosis original magnification ϫ200). (hematoxylin-eosin, original magnification ϫ100). epithelium and exhibited a characteristic orange hue. The granular layer was absent. A neutrophilic infiltrate of vary- ing density was noted at the junction of the parakera- totic layer and the stratum spinulosum (Figure 4). No atypia, mitosis, or koı¨locytes were seen. Aggregates of xanthomatous cells were confined to the papillary dermis, between the rete ridges (Figure 5). These aggregates did not usually extend deeper into the connective tissue. Xanthomatous cells were lipid-laden histiocytes—also called foam cells—and displayed a single small central vesicular nucleus, abundant lipid vacu- oles, and tiny granules in their cytoplasm that were pe- riodic acid–Schiff positive and diastase resistant. We did not observe any multinuclear Touton giant cells. Figure 5. High magnification shows xanthomatous cells aggregated in the The papillary dermis showed an increased number of papillary dermis (hematoxylin-eosin, original magnification ϫ400). prominent and often tortuous thin-walled vessels. Vary- ing degrees of acute or chronic inflammatory infiltrate or an SCC. The outstanding histopathologic features were (lymphocytes, plasma cells, neutrophils, and a few eo- the wedge-shaped, orange-colored parakeratosis invagi- sinophils) were present in the subepithelial connective nating into the papillomatous epithelium and the pres- tissue. Fat stains were precluded because specimens were ence of xanthomatous cells in the papillary dermis not frozen. (Figure 3). No case of transformation into SCC was Four partial biopsies of VX were performed in pa- observed. tients with previously histologically confirmed lichen scle- To our knowledge, only 9 cases of vulvar VX have been rosus or planus.