Validation of the diagnostic criteria for segmental vitiligo.

BACKGROUND: Segmental vitiligo has a different clinical course and prognosis as compared to nonsegmental vitiligo, which necessitates its correct diagnosis. It may be difficult to distinguish segmental vitiligo from the limited or focal types of nonsegmental vitiligo. OBJECTIVE: To validate the previously proposed diagnostic criteria for segmental vitiligo. METHODS: This was a cross-sectional validation study involving patients with limited vitiligo. The diagnostic criteria were used to classify vitiligo lesions as segmental or nonsegmental, and was compared with the experts' diagnosis, which was considered as the "gold standard". RESULTS: The study included 200 patients with 225 vitiligo lesions. As per the diagnostic criteria, 146 vitiligo lesions were classified as segmental and 79 as nonsegmental. The experts classified 147 vitiligo lesions as segmental and 39 as nonsegmental, while the diagnosis either was labeled "unsure" or could not be agreed upon for 39 lesions. As compared with the experts' opinions ("for sure" cases, n = 186), the sensitivity and specificity of the diagnostic criteria was 91.8% (95% confidence interval [CI]: 86.2%-95.7%) and 100% (95% CI: 91%-100%), respectively. The positive predictive value was 100% (95% CI: 97.3-100%), while the negative predictive value was 76.5% (95% CI: 62.5%-87.2%). There was a 93.5% agreement between the clinical criteria and experts' opinions (k = 0.83, P < 0.001). LIMITATION: The diagnostic criteria were compared with the experts' opinion in the absence of an established diagnostic "gold standard". CONCLUSIONS: The proposed diagnostic criteria for segmental vitiligo performed well, and can be used in clinical practice, as well as in research settings.

Classification of facial vitiligo: A cluster analysis of 473 patients.

Vitiligo has a substantial negative impact on quality of life in affected patients, especially those with the involvement of the face. However, the current system can barely distinguish between specific patterns of facial involvement except for the segmental type when focusing only on facial lesions. We classified facial vitiligo into three distinct subtypes using cluster analysis based on facial topography (n = 473): centrofacial vitiligo (72.9%), panfacial vitiligo (18.0%), and hairline vitiligo (9.1%). Centrofacial vitiligo was the most common type and is thought to comprise the typical facial involvement of generalized vitiligo. Panfacial vitiligo was a distinct subtype with onset in old age and less involvement of other body parts. Hairline vitiligo was another distinct subtype with onset in old age and a poor response to conventional phototherapy. A relevant classification system could help us to explore the causes, anticipate the prognosis, and manage the condition in patients with vitiligo.

Predictive Model for Response Rate to Narrowband Ultraviolet B Phototherapy in Vitiligo: A Retrospective Cohort Study of 579 Patients.

Vitiligo is an acquired depigmenting disorder. To date, there is no predictive model for its response rate to narrowband ultraviolet B (NBUVB) phototherapy. The aim of this study was to investigate the different types of response of patients with non-segmental vitiligo undergoing NBUVB 3 times a week. Many patients who were previously considered non-responders were given the opportunity to continue the treatment. Long-term maintenance of treatment and follow-up of a cohort of 579 patients enabled different subtypes of response (very rapid, rapid, average, slow and "non-responders") to be described for the first time, and a predictive model of response to be constructed based on repigmentation rate in the first 48 sessions of NBUVB. Among those patients who did not respond during the first 48 sessions, a new subgroup of patients was found, termed "very-slow" responders, who achieved a low, but significant, level of repigmentation after 96 sessions of NBUVB.

New discoveries in the pathogenesis and classification of vitiligo.

Vitiligo is a common autoimmune disease that progressively destroys melanocytes in the skin, resulting in the appearance of patchy depigmentation. This disfiguring condition frequently affects the face and other visible areas of the body, which can be psychologically devastating. The onset of vitiligo often occurs in younger individuals and progresses for life, resulting in a heavy burden of disease and decreased quality of life. Presentation patterns of vitiligo vary, and recognition of these patterns provides both diagnostic and prognostic clues. Recent insights into disease pathogenesis offer a better understanding of the natural history of the disease, its associations, and potential for future treatments. The first article in this continuing medical education series outlines typical and atypical presentations of vitiligo, how they reflect disease activity, prognosis, and response to treatment. Finally, we discuss disease associations, risk factors, and our current understanding of disease pathogenesis.

Segmental Vitiligo.

Segmental vitiligo is characterized by its early onset, rapid stabilization, and unilateral distribution. Recent evidence suggests that segmental and nonsegmental vitiligo could represent variants of the same disease spectrum. Observational studies with respect to its distribution pattern point to a possible role of cutaneous mosaicism, whereas the original stated dermatomal distribution seems to be a misnomer. Although the exact pathogenic mechanism behind the melanocyte destruction is still unknown, increasing evidence has been published on the autoimmune/inflammatory theory of segmental vitiligo.

Combination therapy of orally administered glycyrrhizin and UVB improved active-stage generalized vitiligo.

Glycyrrhizin has been used clinically for several years due to its beneficial effect on immunoglobulin E (IgE)-induced allergic diseases, alopecia areata and psoriasis. In this study, glycyrrhizin, ultraviolet B light (UVB) or a combination of both were used to treat active-stage generalized vitiligo. One hundred and forty-four patients between the ages of 3 and 48 years were divided into three groups: group A received oral compound glycyrrhizin (OCG); group B received UVB applications twice weekly, and group C received OCG+UVB. Follow-ups were performed at 2, 4, and 6 months after the treatment was initiated. The Vitiligo Area Scoring Index (VASI) and the Vitiligo Disease Activity (VIDA) instrument were used to assess the affected body surface, at each follow-up. Results showed that 77.1, 75.0 and 87.5% in groups A, B and C, respectively, presented repigmentation of lesions. Responsiveness to therapy seemed to be associated with lesion location and patient compliance. Adverse events were limited and transient. This study showed that, although the three treatment protocols had positive results, OCG and UVB combination therapy was the most effective and led to improvement in disease stage from active to stable.

Involvement of T cells in early evolving segmental vitiligo.

Recent studies have suggested an overlapping autoimmune mechanism between segmental vitiligo (SV) and nonsegmental vitiligo (NSV). Although T-cell infiltration is observed in the margins of active lesions in NSV, the histopathological characteristics of the active margin of SV are not well known. To determine if T-cell inflammatory responses are present in the active margin of SV lesions, biopsies were taken from the active margin of a lesion in 12 patients with early or actively spreading SV and compared with a normal control sample (on the symmetrical, opposite site of the same dermatome). The samples were stained for CD4, CD8, CD25 and interferon-γ. Lymphocytic infiltration was seen in 70% of patients. CD4+ T cells infiltrated the dermis, while CD8+ T cells were present in the epidermis or attached to the basal layer. The increase in the number of CD8+ T cells was significant (P < 0.04), while CD4+ or CD25+ T cells also appeared to be increased in number, but this was not significant. These results suggest that SV also has an autoimmune mechanism in the early evolving stage.

Clinicopathologic Analysis of Stable and Unstable Vitiligo: A Study of 66 Cases.

Vitiligo is an acquired skin disorder characterized by milky-white macules and absence of functioning melanocytes. The cornerstone of its management is the correct categorization of a case into its 2 broad types-stable and unstable vitiligo. This distinction is at present based mainly on clinical criteria because the histopathological features are not fully established. This study was thus undertaken to examine histopathological features of vitiligo and to come up with a reliable and systematic approach toward this diagnostic challenge. All patients presenting with clinical features of vitiligo at our institution were included in the study. A 3-mm punch biopsy was taken from 3 sites-lesional, perilesional, and normal skin. Histopathological examination was primarily focused on evaluating 5 histopathological variables-spongiosis, epidermal lymphocytes, basal cell vacuolation, dermal lymphocytes, and melanophages. A total number of 66 patients were included in the study. There were 30 patients in stable and 36 in unstable vitiligo groups. It was observed that all 5 histopathological pattens were associated with unstable vitiligo. All the cases were then scored using a scoring system devised by the authors and the scores obtained were correlated with clinical categorization. It was observed that while there is a definite overlap in histological findings in the 2 groups, adoption of a systematic reporting system brings more consistency and objectivity in the diagnosis. The authors have recommended diagnoses that should be reported for the various scores. This in turn will help us to more reliably and confidently manage these patients.

[Vitiligo: Clinical presentation and pathogenesis]. / Vitiligo : Klinik und Pathogenese.

BACKGROUND: Vitiligo is a chronic skin disorder with depigmentation of circumscribed areas due to structural and functional damage to melanocytes. RESULTS: There is international consensus on the classification in nonsegmental and segmental vitiligo. The influence of vitiligo on the quality of life is significant and is influenced by ethnic and sociocultural factors. There is a new insight into the genetic susceptibility, mechanisms and targets of the autoimmune inflammation, the altered morphology and function of melanocytes and into the association of vitiligo with other autoimmune diseases, skin cancer and skin cancer therapy. CONCLUSIONS: The recognition of associated autoimmune disorders is as important as is the assessment of changes in the quality of life. New insight into the pathogenesis may have therapeutic consequences. The relationship between vitiligo and skin cancer and between vitiligo and immunotherapies in patients with metastatic melanoma warrants close clinical monitoring of affected patients and further scientific studies.

Combination therapy of orally administered glycyrrhizin and UVB improved active-stage generalized vitiligo

Glycyrrhizin has been used clinically for several years due to its beneficial effect on immunoglobulin E (IgE)-induced allergic diseases, alopecia areata and psoriasis. In this study, glycyrrhizin, ultraviolet B light (UVB) or a combination of both were used to treat active-stage generalized vitiligo. One hundred and forty-four patients between the ages of 3 and 48 years were divided into three groups: group A received oral compound glycyrrhizin (OCG); group B received UVB applications twice weekly, and group C received OCG+UVB. Follow-ups were performed at 2, 4, and 6 months after the treatment was initiated. The Vitiligo Area Scoring Index (VASI) and the Vitiligo Disease Activity (VIDA) instrument were used to assess the affected body surface, at each follow-up. Results showed that 77.1, 75.0 and 87.5% in groups A, B and C, respectively, presented repigmentation of lesions. Responsiveness to therapy seemed to be associated with lesion location and patient compliance. Adverse events were limited and transient. This study showed that, although the three treatment protocols had positive results, OCG and UVB combination therapy was the most effective and led to improvement in disease stage from active to stable.

Hypochromic vitiligo: delineation of a new entity.

BACKGROUND: Hypochromic vitiligo is a rare entity that has been reported only twice under the term 'vitiligo minor', with an absence of clear delineation. OBJECTIVES: To delineate hypochromic vitiligo through a case series of patients with typical bilateral hypopigmented lesions affecting the face and trunk. METHODS: This is a retrospective multicentric evaluation study conducted in eight departments of dermatology in France, Belgium, Senegal and Saudi Arabia. RESULTS: Twenty-four cases of hypochromic vitiligo were identified. Fourteen were men and 10 women. The mean age at diagnosis was 35·4 years (range 8-66). Strikingly, all patients were dark skinned, with skin types V and VI. The pattern of distribution was highly similar in most of the patients (18 of 24), with involvement of the face and neck area predominating on seborrhoeic areas associated with multiple isolated hypopigmented macules involving predominantly the scalp. The retrospective nature of this study is its main limitation. CONCLUSIONS: Hypochromic vitiligo is not yet part of a conventional classification. The disease seems to be limited to individuals with dark skin types. Hypopigmented seborrhoeic face and neck involvement associated with hypopigmented macules of the trunk and scalp is the hallmark of the disease.

Vitiligo--Part 2--classification, histopathology and treatment.

In an unprecedented effort in the field of vitiligo, a global consensus resulted on a suggested new classification protocol for the disease. The main histopathological finding in vitiligo is the total absence of functioning melanocytes in the lesions, while the inflammatory cells most commonly found on the edges of the lesions are CD4+ and CD8+ T lymphocytes. Physical and pharmacological treatment strategies aim to control the autoimmune damage and stimulate melanocyte migration from the unaffected edges of lesions and the outer hair follicle root sheath to the affected skin; moreover, surgical treatments can be combined with topical and physical treatments.

Vitiligo - Part 2 - classification, histopathology and treatment

In an unprecedented effort in the field of vitiligo, a global consensus resulted on a suggested new classification protocol for the disease. The main histopathological finding in vitiligo is the total absence of functioning melanocytes in the lesions, while the inflammatory cells most commonly found on the edges of the lesions are CD4+ and CD8+ T lymphocytes. Physical and pharmacological treatment strategies aim to control the autoimmune damage and stimulate melanocyte migration from the unaffected edges of lesions and the outer hair follicle root sheath to the affected skin; moreover, surgical treatments can be combined with topical and physical treatments.

Latent class analysis of a series of 717 patients with vitiligo allows the identification of two clinical subtypes.

Non-segmental vitiligo (NSV) is an enigmatic disease with various clinical courses. To empirically identify underlying subtypes of NSV, we performed latent class analysis (LCA) of 717 consecutive patients with NSV seen between 2006 and 2012 and were analyzed. Median age was 32 yrs (14-45), median age at NSV onset was 18 yrs (8-32), and median NSV duration 5 yrs (0.75-78.5). A two-class model showed the best fit. Of the 717 patients, 280 (39%) belonged to LC1 and 437 (61%) to LC2. LC1 patients had high probabilities for early disease onset (<12 yrs), halo nevi, family history of premature hair greying, Koebner phenomenon, previous episodes of repigmentation, and family history of vitiligo. By contrast, LC2 patients were characterized by a late disease onset (after or at the age of 12 yrs, median age of 30 yrs) and acrofacial localization without any lesions on trunk or limbs. These two LCA classes (LC1, 'prepubertal onset'; LC2, 'post-pubertal onset') may help refining results from genome-wide association studies (GWAS) and allow a more accurate genotype-phenotype correlation and help defining more directed treatment protocols.

Classification of segmental vitiligo on the trunk.

BACKGROUND: Segmental vitiligo is characterized by a unilateral and localized distribution. A classification for segmental vitiligo on the face has been proposed, while this is still unavailable for lesions on the trunk. OBJECTIVES: To design a classification for segmental vitiligo lesions on the trunk by evaluating their distribution pattern. METHODS: In this retrospective observational study, the distribution pattern of 106 segmental vitiligo lesions on the trunk was analysed and classified into recurring subtypes. RESULTS: Segmental vitiligo was more frequently observed on the ventral side (85·8%) than the lateral side (52·8%) or the back (36·8%) of the trunk. Based on recurring similarities in distribution patterns, lesions were categorized into six distinct subtypes: types 1, 2 and 3 involved the upper part of the trunk, type 4 and 5 the middle part and type 6 the lower part of the trunk. In total, 67·9% of all segmental vitiligo lesions fitted into these subtypes. The most frequent type of lesion was subtype 3 (22·6%), which showed a characteristic V-shaped pattern on the upper trunk, followed by subtype 5 (17·9%), with a band-like pattern on the lateral side, and subtype 6 (13·2%), with a rectangular depigmentation on the lower abdomen. CONCLUSIONS: Based on our observations we suggest a new classification into six subtypes for segmental vitiligo lesions on the trunk, allowing categorization of the majority of segmental vitiligo patterns on the trunk. This may have important prognostic and diagnostic significance in early developing lesions.

Classification and comparative study of vitiligo in Southeast of Turkey with biochemical and immunological parameters.

OBJECTIVE: To investigate classification and comparative with biochemical and immunological parameters of vitiligo disease in Southeast of Turkey. PATIENTS AND METHODS: 148 patients who referred to the Department & Dermatology and Venereology with the complaint of vitiligo were selected. All patients gave informed consent for participation in the study. The patients were classified according to types of the skin. The complete blood cell and biochemical laboratory tests, ASO, CRP, RF, ANA, thyroid hormone levels were examined. RESULTS: In the evaluation of 148 vitiligo patients; we didn't observe any significant difference between age and sex groups. We observed the maximum number of patients in the ages of 20-29 years and the most common types of clinical and focal types. We compared the childeren and adult patients according to their skin types, the difference was significant. We viewed that the onset of the disease commonly first in the face area. Koebner phenomenon 17 (11.48%), halo nevus 5 (3.38%) and leucotrichia 5 (3.38%) of patients were positive. CONCLUSION: Vitiligo is associated with loss of color by the destruction of melanocytes. The incidence of vitiligo is 1-2% and is found in every race. This study is important for the reason of the first research which evaluates vitiligo patients in southeast of Turkey.