Halo Nevi Research Articles

New insights in segmental vitiligo: case report and review of theories

Segmental vitiligo and generalized vitiligo are in general considered to be separate entities. The aetiopathogenesis of segmental vitiligo remains unclear, although several hypotheses have been put forward including mainly neuronal mechanisms. The typical association with other autoimmune diseases, as seen in generalized vitiligo, seems to be significantly less in segmental vitiligo, although recent insights point towards a possible immune-mediated overlap between the two subtypes. In this article, we describe a case with simultaneous presence of segmental vitiligo, alopecia areata, psoriasis and a halo naevus. To our knowledge, this is the first case with this exceptional combination. This concomitant presence could support the involvement of a shared autoimmune-mediated process, and may provide new insights into the pathogenesis of segmental vitiligo and direct future research. In the light of this remarkable case, different possible aetiopathogenetic mechanisms leading to the clinical presentation of segmental vitiligo are discussed and a new three-step theory is proposed.

Clinical Features of Vitiligo in Children and Adolescents

Objective – The research was implemented in order to determine the epidemiological and clinical characteristics of vitiligo in children and adolescents. Material and methods – The study was conducted as a crosssectional study of patients aged 0 to 18 years, treated at the Department of Dermatology at the Institute for Children and Youth Health Care of Vojvodina in Novi Sad, from January 1996 to January 2009. The study included 75 children and adolescents with vitiligo, 47 girls and 28 boys. Results – The average age of the patients was 10.81 ± 4.06 years, and their age at the time of occurrence of vitiligo was 8.46 ± 4.19 years. The most common “trigger” for the occurrence of vitiligo was stress in 18 (13.5%) children. 19 (25.3%) had a positive family history of vitiligo, and 29 (38.6%) patients for other endocrine and/or autoimmune diseases, mostly for thyroid disorders - in 25 (33.3%) patients. The initial vitiligo lesions were most often on the head and neck - in 33 children (44.0%). Non segmental vitiligo was diagnosed in 69 (92.0%), and segmental vitiligo in 6 children and adolescents (8.0%). The most common type of vitiligo was generalized, in 32 (42.7%) patients. 28 (34.1%) had polyosis, 35 (42.7%) halo nevus, and Koebner’s phenomenon was found in 19 (23.2%) children and adolescents with vitiligo. Conclusion – A higher incidence of vitiligo was confirmed in girls, the most common type of vitiligo was generalized, the most common sites of the initial lesions were the head and neck, which is consistent with previous studies on children’s vitiligo. The lower frequency of segmental and a higher incidence of acrofacial vitiligo is probably linked to the fact that 42.7% of the sample consisted of adolescents.

Childhood Vitiligo in China

Childhood vitiligo is a common pediatric skin disorder. The pathogenesis of vitiligo is unclear, and immunological dysfunction may play an important role. This prospective study aimed to profile childhood vitiligo and to discuss its correlation with immunological dysfunction. All of the 620 enrolled patients were aged younger than 14 years, and were assessed with a standard questionnaire. The levels of immunoglobulins, complement, and T-lymphocyte subsets were measured in 270 of these 620 patients. Of the 620 children, 302 (48.71%) were boys and 318 (51.29%) were girls, with an average disease onset age of 7.57 years. The average duration was 13.45 months. 453 (73.06%) children had head and neck involvement and 160 (25.81%) children had segmental vitiligo. 84 (13.55%) children had a family history. There was a correlation between the disease and seasons. The onset or progression usually occurred in summer and spring. Halo nevus was seen in both segmental and non-segmental vitiligo. Precipitating factors such as stress appeared more commonly in segmental vitiligo. As to the immunological findings, in segmental vitiligo, the levels of C3 and C4 were lower in the active relative to the quiescent stage (p < 0.05); and in non-segmental vitiligo, the percentages of CD3+ and CD4+ lymphocytes and the CD4+/CD8+ ratio were lower in the active relative to the quiescent stage (p < 0.01). Childhood vitiligo has its own clinical features. The different types of vitiligo have different characteristics. There is immunological dysfunction in children with vitiligo. Dysfunction of humoral immunity may play a role in the progression of segmental vitiligo, while non-segmental vitiligo is more related to cellular immunity.

Halo naevi with associated vitiligo‐like depigmentations: pathogenetic hypothesis

In analogy with melanoma-associated leucoderma, halo naevi may trigger in some patients the development of additional depigmentations which are in distribution, extent and prognosis not in accordance with classic vitiligo. The aim of this study was to support the hypothesis that in a subset of halo naevi patients vitiligo-like lesions develop directly linked to the halo phenomenon. Forty-one patients with halo naevi were examined for the development of depigmentations not corresponding to typical vitiligo lesions. We identified a subset of five halo naevi patients with additional subtle depigmentations. After the occurrence of multiple halo naevi, they developed leucoderma that showed a different disease pattern than vitiligo (variable asymmetric distribution, limited extent and lack of progression). Moreover, the characteristics of these halo naevi patients with associated leucoderma were different from classic vitiligo patients (high number of halo naevi, absence of family history for vitiligo and absence of autoimmune diseases) and the timing of occurrence of the leucoderma suggested a direct relation with the halo phenomenon. In this article, we describe in a limited subset of patients with multiple halo naevi discrete depigmentations at distance from halo naevi which may result from a temporary autoimmune process directly linked to the halo phenomenon. This finding illustrates the collateral damage resulting from skin immunosurveillance and may have clinical consequences as the evolution pattern in this subset of patients is less progressive compared with vitiligo. We present clinical data that support this hypothesis and suggest to call it 'halo naevi-associated leucoderma'.

Multivariate analysis of factors associated with early-onset segmental and nonsegmental vitiligo: a prospective observational study of 213 patients

Although mixed forms have been described recently, segmental (SV) and nonsegmental vitiligo (NSV) are considered as clinically distinct. However, limited epidemiological data are available to help distinguish associated factors, and recent genome-wide association studies have been restricted to NSV. The higher prevalence of SV in children is helpful when comparing the two major presentations of the disease. To compare factors associated with SV and NSV, especially for markers of autoimmunity or autoinflammation. We conducted a single-centre prospective observational study in patients aged 17 years or under with a confirmed diagnosis of SV or NSV at the vitiligo clinic between 1 January 2006 and 1 July 2010. The Vitiligo European Task Force questionnaire was completed for each patient, and thyroid function and antithyroid antibodies were screened if not obtained in the previous year. Other forms of vitiligo (focal, mucosal, not classifiable) were excluded. A total of 213 children were included, 142 with NSV, 59 with SV and 12 with mixed vitiligo. There was no significant statistical difference for sex or age at onset between patients with SV and NSV. Halo naevi were significantly more frequent in NSV than in SV [odds ratio (OR) 7·58, P < 0·01). Patients with NSV more frequently had a positive family history of vitiligo (OR 2·25, P=0·02) and a marked familial autoimmunity background (OR 2·22, P = 0·01). Our study clearly shows that features of inflammation (pruritus)/autoimmunity (halo naevi, thyroid antibodies) are strongly linked to NSV, together with a familial background of vitiligo and autoimmunity.

Estudio descriptivo dermatoscópico de los nevos melanocíticos con despigmentación sin halo clínico

Background and objectives There are few cases described in the literature in which depigmentation of melanocytic nevi occurs without the appearance of halos. The aim of this study was to analyze the correlation between clinical and dermoscopic findings and to assess the usefulness of dermoscopy to identify possible markers of complete regression in melanocytic lesions. Materials and methods A prospective, observational, descriptive study of 77 melanocytic lesions in 52 patients was undertaken over a 5-year period. Melanocytic lesions from patients who underwent periodic follow-up in the digital dermoscopy unit were analyzed if they had exhibited partial or total, permanent depigmentation without a clinically apparent halo. Results We observed substantial variation in the time taken for pigmentation to disappear and the morphological characteristics of the nevi during the depigmentation process. Female sex and dermoscopic evidence of melanophage activity or of a halo were all associated with more rapid involution of pigmented lesions. The only variable which displayed a statistically significant association with complete depigmentation of melanocytic nevi was the presence of vascular proliferation. Fibrosis was the only variable that displayed a statistically significant association with heterogeneous depigmentation of melanocytic nevi. Conclusions In this study, we have identified patterns of depigmentation in melanocytic lesions that differ from the classic halo nevus.

Prognostic value and clinical significance of halo naevi regarding vitiligo

Vitiligo and halo naevi can present together or separately. Whether they are different entities remains unclear. To assess the clinical significance of halo naevi, both with respect to the future development of vitiligo, and to the clinical profile and course of vitiligo. In total, 291 patients were included in this study: patients with only halo naevi (group 1; n=40), patients with generalized vitiligo without halo naevi (group 2; n=173) and patients with generalized vitiligo with halo naevi (group 3; n=78). Patients with only halo naevi (group 1) reported significantly less associated autoimmune disease (P=0·001), were less likely to have a family history of vitiligo (P=0·013) and were less likely to have presence of Koebner phenomenon (P<0·001) compared with patients with generalized vitiligo (groups 2+3). Multiple halo naevi (≥3) were significantly more frequently observed (P=0·002) in patients from group 1 compared with patients from group 3. In group 3, halo naevi were reported prior to the development of vitiligo in 61% (mean±SD time interval of 33·7±5·17months). No significant correlation was observed between the presence of halo naevi and the extent, activity or subtype of vitiligo. However, halo naevi in patients with vitiligo significantly reduced the risk for associated autoimmune diseases, and age at onset of vitiligo was significantly lower compared with patients with vitiligo without halo naevi (P<0·001). Our results support the hypothesis that halo naevi can represent a distinct condition. In a subset of patients, the occurrence of halo naevi may be an initiating factor in the pathogenesis of vitiligo.

Relevance of autoimmune thyroiditis in children and adolescents with vitiligo

Vitiligo is the most common pigmentation-related disorder worldwide. An autoimmune etiology is widely considered, and genetic factors may play an important role in its pathogenesis. The purpose of this study was to assess the incidence of thyroid dysfunctions and autoimmune thyroiditis in children with vitiligo and to identify related factors. Fifty children with vitiligo and 50 control children were enrolled. Data on age, onset, duration, disease activity, presence of thyroid disorder, other autoimmune diseases, halo nevi, poliosis, and mucosal vitiligo were determined. Serum free triiodothyronine, free thyroxine, total T3, total T4, thyroid-stimulating hormone, and antibodies to thyroperoxidase and thyroglobulin were measured. Thyroid gland efficiency was evaluated. The mean age at onset of vitiligo was 7.26 ± 4.43 years. The duration of vitiligo was 2.26 ± 2.95 years. Vulgaris-type vitiligo was the most common form in our patients (56%), and 42% reported at least one family member with thyroid disorder, autoimmune disease, or both. Overt hypothyroidism or hyperthyroidism were not detected. We found a significant association between autoimmune thyroiditis and both sex and disease duration (P = 0.046 and P = 0.07, respectively), but no association between autoimmune thyroiditis and age, age at onset of vitiligo, halo nevi, poliosis, mucosal involvement, disease activity, or family history of vitiligo, autoimmunity, or thyroid disorders. Children with vitiligo show an increased incidence of autoimmune thyroiditis. Children with vitiligo, especially girls and subjects with generalized/vulgaris-type vitiligo, should be screened annually for thyroid function and antithyroid antibodies to assist in the early diagnosis and therapy of autoimmune thyroiditis.

Vitiligo: A review of the published work

Vitiligo is a common depigmenting skin disorder, characterized by acquired, idiopathic, progressive, circumscribed hypomelanosis of the skin and hair, with total absence of melanocytes microscopically. It occurs worldwide, with an incidence rate of between 0.1% and 2%. Vitiligo is an important skin disease having a major impact on the quality of life of the patient suffering from it. The causes of this condition are uncertain but seem to be dependent on the interaction of genetic, immunological and neurological factors. Vitiligo coexists with other autoimmune disorders, Sutton or halo nevus, and malignant melanoma. The substantial disfigurement associated with vitiligo can cause serious emotional stress for the patient, which necessitates treatment. Because its pathogenesis is still not understood, there is a plethora of different treatments. Among them, topical steroids and narrowband ultraviolet B monotherapy were the most common as current treatments for localized and generalized vitiligo, respectively. Cosmetic improvement can be achieved by camouflage products and self-tanning dyes. The course of vitiligo is unpredictable, but often progressive. Spontaneous repigmentation may occur in a few people (10–20%), mainly in children, but this tends to be only partial and on sun-exposed areas. In this article, we review vitiligo as a whole, including epidemiology, pathogenesis and etiology, histopathology, clinical manifestations, classification, clinical variants, diagnosis and differential diagnoses, specific investigation, treatment, prognosis, psychosocial view and its association with other disorders.

Vitiligo in an Italian Outpatient Center

Vitiligo is a depigmentation disorder resulting from destruction of cutaneous melanocytes that affects 0.1-2% of the world's population, irrespective of sex and race. To investigate the clinical and immunopathologic characteristics of a series of Italian vitiligo patients. We examined clinical and immunopathologic data of 204 patients affected by vitiligo at a university-based dermatology outpatient hospital (second clinic) between January 1998 and March 2008. In particular, the clinical-epidemiologic characteristics of our patients, serologic parameters suggestive of immune/autoimmune activity (autoantibodies, immune complexes, complement, immunoglobulins), and the association between vitiligo and HLAs were investigated. Upon comparison of our results with control and literature values, the following aspects appeared to be in complete agreement: the frequency of clinical subtypes of vitiligo, an earlier onset of segmental compared with non-segmental vitiligo, the association of familial vitiligo with other autoimmune diseases, the greater association of non-segmental vitiligo than segmental vitiligo with autoimmune diseases, and some features of pediatric vitiligo. Other data were partially consistent with the literature, such as the association between vitiligo and autoimmune diseases/autoantibody activities, and the association between vitiligo and HLAs. Finally, a portion of our data did not concur with the literature, including the sex distribution and mean age of onset, the lack of association between halo nevi and autoimmune diseases, and some aspects of pediatric vitiligo. This study provides novel information regarding clinical features and serologic parameters in different subgroups of vitiligo, namely a significant association between active vitiligo and autoantibody activities, and significant clinical differences (i.e. activity of disease, age of onset, and coexistence of other autoimmune diseases) between vitiligo associated with autoantibodies and vitiligo negative for autoantibodies.

Extralesional vitiligo over knee following halo nevus with poliosis on the scalp

Extralesional vitiligo over knee following halo nevus with poliosis on the scalp

Descriptive Dermoscopic Study of Depigmentation in Melanocytic Nevi Without a Visible Halo

Background and objectives There are few cases described in the literature in which depigmentation of melanocytic nevi occurs without the appearance of halos. The aim of this study was to analyze the correlation between clinical and dermoscopic findings and to assess the usefulness of dermoscopy to identify possible markers of complete regression in melanocytic lesions. Materials and methods A prospective, observational, descriptive study of 77 melanocytic lesions in 52 patients was undertaken over a 5-year period. Melanocytic lesions from patients who underwent periodic follow-up in the digital dermoscopy unit were analyzed if they had exhibited partial or total, permanent depigmentation without a clinically apparent halo. Results We observed substantial variation in the time taken for pigmentation to disappear and the morphological characteristics of the nevi during the depigmentation process. Female sex and dermoscopic evidence of melanophage activity or of a halo were all associated with more rapid involution of pigmented lesions. The only variable which displayed a statistically significant association with complete depigmentation of melanocytic nevi was the presence of vascular proliferation. Fibrosis was the only variable that displayed a statistically significant association with heterogeneous depigmentation of melanocytic nevi. Conclusions In this study, we have identified patterns of depigmentation in melanocytic lesions that differ from the classic halo nevus.

Immune reactions in benign and malignant melanocytic lesions: lessons for immunotherapy

Spontaneous regression of benign and malignant melanocytic lesions can be a visible sign of immunosurveillance. In this review, we discuss different immune reactions against melanocytic lesions: halo nevus, Meyerson's nevus, regression in melanoma and melanoma-associated depigmentation. These entities present with particular clinical aspects, histology and evolution. In all entities, a melanocyte-specific T-cell reaction has been assumed but a different degree of melanocyte destruction is present. A focus on the immune responses in melanocytic lesions reveals several aspects of an adequate skin immunity and may help to identify the key points in the immune destruction of melanocytes. These insights can add to the knowledge of how to optimize immunotherapeutic strategies in melanoma.

Long-term results of noncultured epidermal cellular grafting in vitiligo, halo naevi, piebaldism and naevus depigmentosus

Several surgical techniques are available for the treatment of stable leucoderma. The use of noncultured epidermal cellular grafting was introduced in 1992. Data on long-term follow-up regarding stability of the repigmented area, time to achieve the final repigmentation, colour matching, reaction to sun exposure and patient satisfaction with treatment have been reported only a few times previously. To evaluate the long-term results of the noncultured epidermal cellular grafting technique in patients with different types of leucoderma, including segmental vitiligo (n = 33), generalized vitiligo (n = 33), mixed vitiligo (n = 6), halo naevi (n = 11), piebaldism (n = 3) and naevus depigmentosus (n = 1). Patients were evaluated by examination and questionnaire in a retrospective setting after transplantation by autologous noncultured cellular grafting. Percentage of repigmentation was evaluated in 82 patients using a digital imaging analysis system (mean follow-up 15 months). Long-term results were evaluated by 54 patients using a questionnaire up to 7·7 years after treatment (mean 4 years). More than 75% repigmentation was achieved in 71% of patients. Best results were obtained in segmental vitiligo, halo naevi and piebaldism, whereas results in generalized or mixed vitiligo were inferior. According to the patients, final repigmentation was achieved after a mean of 10 months post-treatment. In 80% some colour mismatch (hyperpigmentation and hypopigmentation) was reported between the treated area and the surrounding skin, although this was not disturbing for the majority. This colour mismatch was reported significantly less after sun exposure (P = 0·012). During follow-up 7% of patients, all with generalized vitiligo, observed some loss of the achieved repigmentation. Autologous epidermal cellular grafting achieved a high percentage of repigmentation, which was maintained during follow-up in the majority of patients. Although it improved quality of life, a perfect colour match was seldom obtained.

Frühes Melanom neben einem benignen Nävus, nävusassoziiertes Melanom oder regressiver Nävus?

A 43-year-old woman, in whom a melanoma associated with a melanocytic nevus had been removed 2 years previously, came for a follow-up examination presenting with two light brown to medium brown maculae measuring approximately 7x5 mm with a flat papular center. One of the lesions was surrounded by a large white halo about 2 mm in size. The differential diagnosis on clinical grounds included a halo nevus with a second common nevus, a nevus with regression, and a melanoma associated with a nevus. Under the dermatoscope, an unremarkable homogeneous/reticular pattern could be seen and in one portion hallmarks of regression, namely gray tones and distinct gray-black dots. The diagnosis based on histopathological analysis was that of an atypical congenital nevus with regression.

The application of dermal papillary rings in dermatology by in vivo confocal laser scanning microscopy

Confocal laser scanning microscopy (CLSM) allows noninvasive visualization of human skin in vivo, without needing to fix or section the tissue. Melanocytes and pigmented keratinocytes at the level of the basal layer form bright dermal papillary rings which are readily amenable to identify in confocal images. Our purpose was to explore the role of dermal papillary rings in assessment of lesion location, the diagnosis, differential diagnosis of lesions and assessment of therapeutic efficacy by in vivo CLSM. Seventy-one patients were imaged with the VivaScope 1500 reflectance confocal microscope provided by Lucid, Inc. The results indicate that dermal papillary rings can assess the location of lesion; the application of dermal papillary rings can provide diagnostic support and differential diagnosis for vitiligo, nevus depigmentosus, tinea versicolor, halo nevus, common nevi, and assess the therapeutic efficacy of NBUVB phototherapy plus topical 0.1 percent tacrolimus ointment for vitiligo. In conclusion, our findings indicate that the dermal papillary rings play an important role in the assessment the location of lesion, diagnosis, differential diagnosis of lesions and assessment of therapeutic efficacy by in vivo CLSM. CLSM may be a promising tool for noninvasive examination in dermatology. However, larger studies are needed to expand the application of dermal papillary rings in dermatology.

Halo Nevus of the Choroid in 150 Patients

To evaluate choroidal halo nevus. We performed a retrospective medical record review on all patients with a clinical diagnosis of choroidal halo nevus treated at the Ocular Oncology Service at Wills Eye Institute from April 1, 1974, through June 30, 2008. Their clinical characteristics and natural history were studied. The choroidal halo nevus showed 2 components, including a distinct central pigmented region surrounded by a yellow halo. Of the 150 patients, 107 (71.3%) were women and 43 (28.7%) were men; and 149 (99.3%) were white, with a median age at presentation of 54 years. Autoimmune disorders were found in 4 patients (2.7%), a rate similar to the prevalence in the US population (2.7% vs 3.1%, P = .74). Preexistent cutaneous melanoma was found in 5 patients (3.3%), which was significantly more prevalent than the rate for the US population (3.3% vs 0.3%, P < .001). The halo was peripheral in 139 patients (92.7%) and slightly internal in 11 (7.3%). Two patients (1.3%) had multifocal halo nevi. The nevus location was superior in 31 patients (20.7%), temporal in 43 (28.7%), inferior in 29 (19.3%), nasal in 27 (18.0%), and macular in 20 (13.3%). Related features included drusen in 85 patients (56.7%), subretinal fluid in 21 (14.0%), orange pigment in 13 (8.7%), and retinal pigment epithelial atrophy in 15 (10.0%). There were no intraocular inflammatory findings. Of the 110 patients with nevi with follow-up, growth into melanoma occurred in 4 patients (3.6%) at a median interval of 41 months. Halo nevus is a variant of choroidal nevus that has a brown center and yellow halo. No relationship was found with autoimmune disorders, but a relationship with previous cutaneous melanoma is possible.

Long term results of non cultured epidermal cellular grafting in vitiligo, halo nevi, piebaldism and nevus depigmentosus

Long term results of non cultured epidermal cellular grafting in vitiligo, halo nevi, piebaldism and nevus depigmentosus

Cutaneous Metastases of Malignant Melanoma: A Clinicopathologic Study of 192 Cases With Emphasis on the Morphologic Spectrum

Metastatic melanoma represents one of the most common types of cutaneous metastases. In up to 5% of patients, metastatic melanoma can be the first manifestation of the disease. For the most part, the histologic diagnosis of metastatic melanoma poses little diagnostic difficulty; however, some metastases may adopt unusual or unfamiliar appearances mimicking other benign and malignant conditions. We present a study of 192 cases of cutaneous metastatic melanomas with special emphasis on their spectrum of morphologic features. The patients were 115 men and 77 women, 23-91 years of age (mean 57 years). Most tumors were located on the proximal legs, scalp, and arms and ranged from 0.8 to 3.0 cm. One hundred ten cases showed the classic morphologic appearance of melanoma (well-circumscribed epithelioid dermal/subcutaneous nodule), 82 cases showed unusual histologic appearances that mimicked other benign and malignant neoplasms. In 16 patients (8.3%), there was no evidence of primary melanoma and the cutaneous metastasis was the only manifestation of the disease. The histologic diagnosis of cutaneous metastatic melanoma can pose difficulties for diagnosis, especially in the face of an unknown primary neoplasm. Unusual features observed in this series included examples of cutaneous metastatic melanoma that closely simulated metastatic carcinoma, dermatofibroma, leiomyosarcoma, angiosarcoma, nevoid melanoma, halo nevus, blue nevi, and atypical fibroxanthoma. Several cases also showed rhabdoid, balloon cell, and alveolar features. Immunohistochemical stains plus careful clinical history helped to establish the correct diagnosis. Our series illustrates that the differential diagnosis of cutaneous metastatic melanoma can be broad and difficult. To the best of our knowledge, this is the largest series of cutaneous metastatic melanomas reported in the literature.

Halo nevi related to treatment with imatinib in a dermatofibrosarcoma protuberans patient

Halo nevi related to treatment with imatinib in a dermatofibrosarcoma protuberans patient