Halo nevi, also known as Sutton's nevi, leukoderma acquisitum centrifugum, or perinevoid vitiligo, are melanocytic nevi surrounded by an acquired ring of hypopigmented or depigmented skin (Balin & Barnhill, 2018). Although halo nevi themselves are generally benign, they may be associated with other conditions such as vitiligo (Patrizi et al., 2013), and the halo phenomenon can occur around other concerning neoplasms including cutaneous melanoma and basal cell carcinomas (Basak et al., 2015; Suzuki et al., 2016). Thus, the diagnosis and management of these lesions can be challenging. This review provides a synopsis of the epidemiology, pathophysiology, diagnosis, and management of halo nevi in the pediatric population.
EPIDEMIOLOGY AND PATHOPHYSIOLOGY
Halo nevi are most commonly observed during childhood and adolescence, with a mean age of presentation of 15 years (Balin & Barnhill, 2018). The incidence of halo nevi has been estimated at about 1% of the population (Herd & Hunter, 1998). Although uncommon, familial cases have been reported (Herd & Hunter, 1998), and no gender or race predilection has been observed (Aouthmany et al., 2012; Weyant et al., 2015). The halo phenomenon can occur at the site of other benign conditions including seborrheic keratoses (Vural & Güleç, 2022), dermatofibromas (Schofield et al., 2012), and molluscum (Le Treut et al., 2015) as well as around malignant tumors including cutaneous melanoma (Suzuki et al., 2016) and basal cell carcinoma (Basak et al., 2015). In this review, we focus on the characteristics of halo nevi and the halo phenomenon most relevant to pediatric patients.
Although the exact pathophysiologic mechanism associated with halo nevi remains unknown, they are thought to arise from a cell-mediated or humoral immune response directed against antigens within nevomelanocytes because of a break in autoimmunity (Balin & Barnhill, 2018; Zeff et al., 1997). This immune response results in nevomelanocyte regression and destruction, producing the characteristic ring of hypopigmentation observed in halo nevi.
PRESENTATION AND DIAGNOSIS
The halo nevus typically presents as a pink to dark-brown central nevus surrounded by a “halo” of hypopigmentation or depigmentation. Patients may have one halo nevus or multiple halo nevi. Halo nevi are most frequently observed on the upper back but can occur anywhere on the body (Zeff et al., 1997). Notably, halo nevi on the scalp or other hair-bearing areas may present as poliosis, a focal patch of white hair, if the autoinflammatory response associated with the halo phenomenon also affects melanocytes within hair follicles (Kay et al., 2010; Sleiman et al., 2013). As with other benign nevi, the central melanocytic nevus is generally less than 5 millimeters in diameter, has a homogenous color, and has well-defined, symmetric borders (Stefanaki, 2019).
Dermoscopy, a noninvasive, bedside technique of examining cutaneous lesions with a dermatoscope, can assist in evaluating and diagnosing halo nevi. On dermoscopic evaluation of a halo nevus, the central nevus may show a globular, homogenous, or reticular pigment pattern—features commonly seen in other benign nevi in pediatric patients—and the surrounding hypopigmented ring is generally pale, homogenous, symmetric, and well circumscribed and does not contain any significant structures or features (Figure 1; Haliasos et al., 2013; Kolm et al., 2006; Nedelcu et al., 2021).
Histologic examination of the central nevus reveals dense lymphocytic and histiocytic infiltrates, and the presence and arrangement of nevus cells varies based on the degree of regression (Balin & Barnhill, 2018); in completely regressed halo nevi, inflammatory infiltrates without any nevus cells may be observed (Nedelcu et al., 2021). Halo nevi are most frequently associated with compound or intradermal nevi, although the halo phenomenon has been observed with other nevomelanocytic lesions including Spitz nevi and blue nevi (Paller & Mancini, 2021). Although less common, halos can develop spontaneously around congenital melanocytic nevi of any size, resulting in significant or complete regression (Cusack et al., 2009; Margileth, 2019). Reassuringly, one study of 124 halo nevi excised in pediatric and adult patients found that most lesions were not dysplastic (Weyant et al., 2015), and halo melanomas remain exceedingly rare in the pediatric population (Lai et al., 2001).
The clinical appearance of a halo nevus evolves over time, and four distinct stages have been described (Aouthmany et al., 2012; Huynh et al., 2001). Initially, a hypopigmented halo appears around a normal-appearing central nevus (Stage I). The central nevus slowly regresses (Stage II) and eventually disappears (Stage III), leaving behind a hypopigmented macule. Eventually, repigmentation of the skin occurs (Stage IV). Significant variation in clinical course may occur. This process is often prolonged, requiring years to decades for complete resolution (Figure 2).
Halo nevi may coexist with other autoimmune conditions affecting pediatric patients. Vitiligo, a disorder characterized by patches of skin pigment loss, is thought to arise from autoimmune processes closely related to yet distinct from that of halo nevi (Jouary & Taïeb, 2010; Stierman et al., 2009). Halo nevi are frequently observed in children with preexisting vitiligo (Cohen et al., 2016; Mazereeuw-Hautier et al., 2010), although it remains unclear if halo nevi are a risk factor for the development of new-onset vitiligo in children (Cohen et al., 2016; Patrizi et al., 2013; Van Geel et al., 2011). Notably, there has been one case report in which excision of a halo nevus in a pediatric patient led to the rapid improvement of their concurrent vitiligo, highlighting the relationship between these clinical entities (Wang et al., 2016). Cases of halo nevi presenting in association with autoimmune hyperthyroidism and hypothyroidism have also been reported (Drąg-Zalesińska et al., 2019; Zhou et al., 2016), and halo nevi associated with nonsegmented vitiligo seem to occur more frequently in patients with a family history of autoimmune disease (Ezzedine et al., 2012). Increased presence of halo nevi has also been observed in patients with Turner syndrome, a common sporadic genetic disorder characterized by short stature, webbed neck, and impaired gonadal and sexual development, suggesting a genetic basis for the tendency to develop halo nevi (Bello-Quintero et al., 2010; Brazzelli et al., 2004). Crucially, the sudden development of halo nevi has been associated with occult cutaneous or ocular melanoma at a distant site in adults (Balin & Barnhill, 2018; Haynes et al., 2021; Vyas et al., 2016), possibly because of cross-reactivity between antigens within the malignant tumor and benign nevomelanocytes elsewhere on the body (Zeff et al., 1997). However, the overall incidence of melanoma—and thus melanoma-associated halo nevi—remains extremely low among pediatric patients (Paulson et al., 2020). Interestingly, prior work have identified the presence of antibodies and immune cells toxic to melanoma cells in individuals with halo nevi (Zeff et al., 1997), and research investigating a potential therapeutic link between the local cellular and microenvironment of halo nevi and cutaneous melanoma is ongoing.
The management of halo nevi within the pediatric population should consider the specific needs and history of the patient. Healthcare providers should evaluate pediatric patients for a personal or family history of atypical nevi, cutaneous and ocular melanoma, and autoimmune disease including vitiligo and should perform a thorough examination of the skin and mucosal surfaces. A careful clinical and dermoscopic examination of each halo nevus is needed to distinguish benign halo nevi from more concerning entities, such as atypical nevi or melanoma. The “ABCDE” mnemonic for assessing melanoma (asymmetry, border irregularity, color variegation, diameter > 6 mm, evolving lesion; Tsao et al., 2015) can be used to assess the central nevus. If the nevus exhibits clinical features suggestive of atypia including asymmetry or abnormal coloration, or shows melanoma-specific dermoscopic patterns such as atypical network, negative network, crystalline structures, or atypical vessels (Haliasos et al., 2013), then biopsy and histopathologic evaluation is warranted. If the nevus appears benign, however, then serial monitoring is appropriate, and the halo nevus does not need to be excised. Given the rarity of ocular melanoma among pediatric patients (Singh et al., 2000), referral for general ophthalmic evaluation in the setting of benign halo nevi is currently not recommended (Paller & Mancini, 2021). In the literature, improvement of benign halo nevi have been reported after treatment with topical calcineurin inhibitors (Hossain et al., 2013), topical Janus kinase inhibitors (Hu et al., 2018), dermabrasion (Awad et al., 2019), and narrow-band ultraviolet B phototherapy (Shah et al., 2019). In general, however, no treatment is necessary for halo nevi in pediatric patients (Saleem et al., 2019). Because patients often seek dermatologic care for halo nevi because of changes in the appearance of the melanocytic lesion, healthcare providers should educate and counsel patients on the expected progression and lengthy time course associated with the resolution of benign halo nevi.
Halo nevi are a common dermatologic finding, particularly among children and adolescents, and may be associated with other conditions including vitiligo and Turner syndrome. In the pediatric population, halo nevi are generally benign; however, healthcare providers should perform a detailed clinical and dermoscopic examination to distinguish between benign halo nevi and more concerning or associated entities including vitiligo, halo nevi with atypia, and halo melanoma.
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