JEADV

DOI: 10.1111/jdv.13115

SHORT REPORT

Genital melanocytic naevus on lichen sclerosus: an uncommon occurrence and a management proposal I. Neri,† E. Dika,*,† P.A. Fanti, C. Misciali, A. Patrizi Dermatology, Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy *Correspondence: E. Dika. E-mail: [email protected]

Abstract Background Melanocytic naevi located in special sites, such as the vulvar mucosa might present peculiar clinical and dermatoscopic features. Objectives We describe a management proposal of a genital naevus associated with inflammatory disorders that aims to facilitate the clinical and pathologic diagnosis. Methods Videodermoscopy of a genital naevus associated with lichen sclerosus of an 8-year-old girl, was carried out before and 2 months after treatment with topical steroids. An excisional biopsy and immunohistochemical studies with HMB-45, MART -1 and molecular studies with p 16 staining were performed. Results The features of the melanocytic lesion associated with lichen sclerosus were troublesome on the basis of clinical and videodermoscopic evaluation. Histopathologic and immunohistochemical examination performed after topical treatment, showed a compound melanocytic naevus with an underlying inflammation consistent with lichen sclerosus. Conclusions The evaluation of genital naevi should take into account the presence of inflammatory disorders, not uncommon in such location. Treatment of the latter and short follow-up of the patients, can avoid over-diagnosis of malignancies and extensive surgical procedures. Received: 28 January 2015; Accepted: 23 February 2015

Conflicts of interest The authors declare no conflicts of interest.

Funding sources The authors declare no funding sources supporting the work and no conflicts of interest.

Introduction

Case report

The characteristics of genital melanocytic nevi (GMN) in children have not been widely reviewed. Most reports are single institution experiences and there is a lack of large scale studies on the prevalence of GMN.1–3 Lichen sclerosus (LS), on the other hand, is an uncommon inflammatory dermatitis, often misdiagnosed, with a predilection for the anogenital area. The estimated prevalence in girls is 1 in 900.4 Despite the rarity of both entities, a few cases of GMN superimposed on LS have been described, giving rise to atypical clinical and pathological features.5,6 We herein report an additional case of a worrisome genital melanocytic naevus associated with lichen sclerosus and a management proposal, together with a short review of the current literature.

An 8-year-old girl was referred to the Pediatric Dermatology Unit, Department of Experimental, Diagnostic and Specialty Medicine, for the occurrence of a pigmented lesion on the vulvar mucosa. The young patient’s mother was concerned about the possibility of a pigmented tumour or a melanoma. The mother had noticed the lesion 3 months before. Familial history was negative for melanoma and non-melanoma skin cancers and the patient was otherwise healthy. Dermatological examination revealed a flat asymmetric pigmented macule, 4 mm in diameter, extending over both the right labium minus and majus (Fig. 1a) on an underlying clinically evident LS with well-demarcated white plaques on the vulvar and perianal area. The LS was asymptomatic. The remainder of the skin and physical examination showed normal findings. On dermatoscopy, the pigmented lesion showed a diffuse pigmentation without a pigmented network, ‘a multicomponent



These authors contributed equally to this work.

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Figure 1 (a, b) Clinical and dermatoscopic presentation of a pigmented lesion of an 8year-old girl on underlying lichen sclerosus, showing a multicomponent pattern. (c, d) The same pigmented lesion 2 months after the treatment of lichen, showing a typical parallel pattern.

pattern’ with black dots of pigment, surrounding a white-greyish veil that extended from the interlabial furrow (Fig. 1b). We decided to treat the LS with 0.1% methylprednisolone aceponate cream once daily for 1 month and one application every 48 h for the second month. A short-term follow-up (2 months) was recommended, in order to observe the evolution of the pigmented lesion and of the underlying LS. At the follow-up visit, the clinical manifestations of genital LS showed an almost complete remission. Dermatoscopy showed remarkable changes on the pigmented lesion, presenting a typical parallel pattern with an accentuated central hyperpigmentation, conservation of the glandular dots (Fig. 1c,d), absence of the whitish-grey veil and the globular-like structures observed at the first visit. The clinical and dermatoscopic features were consistent with a diagnosis of benign melanocytic naevus. Due to the anxiety of the parents, the lesion was subsequently excised. Histopathology showed a symmetric architecture of the lesion, no melanocytic atypia and a sparse infiltrate of lymphocytes surrounding and permeating the melanocytic nests, with oedema and fibrosis in the papillary dermis (Fig. 2a,b). Immunohistochemical studies with HMB-45, MART-1 and molecular studies with p 16 staining confirmed the diagnosis of a compound melanocytic naevus with an underlying inflammation consistent with LS (Fig. 2c,d). After 6 months of follow-up no signs of recurrence of the GMN were observed and the LS maintained complete remission.

Discussion To date, the described cases of GMN in children are congenital or early-onset lesions.3 The most frequently reported

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dermatoscopic patterns are the parallel, globular and homogenous forms. As in adults, the main differential diagnoses in paediatric patients are melanotic macules or vascular tumours.7,8 The clinical, dermatoscopic and histopathologic characteristics of GMN may sometimes show peculiar characteristics and cause anxiety to parents and clinicians. Although melanoma in childhood is rare as it accounts for 0.3–0.5% of melanomas in childhood, it should be considered in the differential diagnosis of pigmented lesions, even in this peculiar setting.9–16 Genital melanocytic naevi on LS are rarely encountered, though the real incidence it is not well known. In the main reviews concerning LS in paediatric patients, the association with melanocytic naevi has not been mentioned.4 Vice versa, some cases of GMN associated with LS have been described with regard to the histopathologic changes.17–19 Moreover, three of the four cases of melanoma on vulvar mucosa reported in paediatric patients were associated with an underlying LS. A discussion on a possible pathogenetic role of the inflammation and the histopathologic changes in the tumoral stroma followed these reports. No description about the clinical and dermatoscopic features of the pigmented lesions was given, nor the anamnestic data on LS occurrence.20–22 In 2012, Pinto et al.23 described the atypical histopathologic features of a junctional naevus arisen on an LS background in the genital area and discussed the importance of the confounding features with melanoma derived from LS changes, such as dermal sclerosis and stromal changes that could be misinterpreted as regression. Other cases of GMNi associated with LS were reviewed by Carlton et al.17 They postulated that the histologic similarities

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between melanoma and naevi occurring on scar tissue or on an altered stromal background, might be due to an activation of melanocytes that proliferate contiguously to fibrotic or sclerotic collagen, containing a more abundant melanin. Regarding our patient, the decision to the treat the lichen and to perform a further evaluation of the pigmented lesion was made after the literature on the latter cases had been consulted by the chief clinician. Applying the dermoscopic algorithm proposed by Ronger Savle et al.24 for the evaluation of vulvar-pigmented lesions to our case, a total score of 5 would have been achieved at the first consultation as follows: the lesion was unilateral (1 point), flat (0 points), showed irregularity of the pattern (1 point), presented a whitish veil (2 points) and three colours (1 point). Instead, at the follow-up visit the score would have been 1, since, after the regression of the LS, the pigmented lesion showed remarkable changes, presenting unilaterality as the only variable correlated with malignancy (1 point). If only the clinical and dermatoscopic criteria had been followed, without a careful evaluation of confounding factors such as the presence of lichen-related changes, a misdiagnosis would likely have been performed, creating psychological discomfort to the family and perhaps difficulties in reaching the correct pathologic diagnosis. After the treatment and remission of the LS and the correlated inflammation, the diagnosis of GMN became easier on a clinical, dermatoscopic and histopathologic basis. In conclusion, the clinician and pathologist should not underestimate the possibility of melanoma occurrence, but they should also be aware that environmental factors,

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Figure 2 (a) Histopathologic features of the naevus showing a symmetric silhouette of the lesion (H&E 10 9: Original magnification). (b) Detailed images: no melanocytic atypia and a sparse infiltrate of lymphocytes surrounding and permeating the melanocytic nests, with oedema and fibrosis in the papillary dermis (H&E. 25 9: Original magnification). (c, d) Low power view of the lesion after staining for and HMB-45 and p16, confirming the diagnosis of a compound melanocytic naevus (109 Original magnification).

such as LS, could interact with GMN producing misleading features. The aim of this paper is to add a management proposal to the current literature. When GMN occur on LS, the treatment of the inflammation and a short follow-up (2–3 months) represent the best therapeutic approach. The avoidance of extensive surgical procedures and of melanoma overdiagnosis in childhood is one of the main purposes of our clinical practice and research.

Acknowledgements We thank the parents of the patient, who readily cooperated and respected our clinical decisions.

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Genital melanocytic naevus on lichen sclerosus: an uncommon occurrence and a management proposal.

Melanocytic naevi located in special sites, such as the vulvar mucosa might present peculiar clinical and dermatoscopic features...
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