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GPNMB is involved in multiple cellular functions including cell adhesion, stress protection and stem cell maintenance. In skin, melanocyte-GPNMB is suggested to mediate pigmentation through melanosome formation, but details of keratinocyte-GPNMB have yet to be well understood. We confirmed the expression of GPNMB in normal human epidermal keratinocytes (NHEKs) by reducing the expression using siRNA. A higher calcium concentration of over 1.25 mM decreased the GPNMB expression. Histological staining showed that GPNMB was expressed in the basal layer of normal skins but completely absent in vitiligo skins. The normal expression of GPNMB in nevus depigmentosus skin suggested that lack of GPNMB is characteristic of vitiligo lesional skins. IFN-γ and IL-17A, two cytokines with possible causal roles in vitiligo development, inhibited GPNMB expression in vitro. Approximately 4–8% of the total GPNMB expressed on NHEKs were released possibly by ADAM 10 as a soluble form, but the process of release was not affected by the cytokines. The suppressive effect of IFN-γ on GPNMB was partially via IFN-γ/JAK2/STAT1 signaling axis. Decreased GPNMB expression in keratinocytes may affect melanocyte maintenance or survival against oxidative stress although further studies are needed. These findings indicate a new target for vitiligo treatment, focusing on the novel role of IFN-γ and IL-17 in downregulating keratinocyte-GPNMB.

Introduction: Hypopigmented or depigmented lesions are considered as important dermatoses in daily practice. Their accurate diagnosis is very much crucial because of social stigma attached to these diseases in Indian subcontinent. Authors have proposed basic guidelines for dermoscopic assessment in terms of approach to a given hypopigmented/depigmented lesion. The following conditions are dermoscopically described here, they are vitiligo, idiopathic guttate hypomelanosis, lichen sclerosus, pityriasis alba, leprosy, pityriasis versicolor and nevus depigmentosus. Aim: To propose basic dermoscopic guidelines in terms of approach of a hypopigmented/ depigmented lesion in skin of color. Summary: Dermoscopic patterns highly depend on melanin skin layers and thus analysis of dermoscopic patterns in hypopigmented/depigmented lesions is difficult owing to the reduction or absent melanin in the epidermis. All hypopigmented/depigmentary conditions are described based on the 5 dermoscopic parameters which include; i) background colour, ii) vessels, iii) scales, iv) follicular findings and v) specific clues. Glowing white color in vitiligo and pale or dull white color in IGH and white with little brownish color in PA and PV are the different shades of color in the background in dermoscopy. In leprosy, PMH and ND, background color is brownish-white rather white. LSEA, color is white with bluish or pinkish hue is observed. Furthermore, dermoscopic diagnosis should be based on every parameter visible in a given lesion and not in isolation as each pattern or parameter is immensely contributory to the final diagnosis. Conclusion: Thus, dermoscopy aids in the distinction of hypopigmented/depigmentary lesions by demonstrating characteristic and definitive patterns.

Segmental nevus depigmentosus and segmental vitiligo can be difficult to differentiate from each other. Differential diagnosis of these two diseases is important because they have significantly different prognoses and psychological effects. The purpose of this study is to identify clinical clues that may be helpful in differentiating these two diseases. We enrolled 63 patients with segmental nevus depigmentosus and 149 patients with segmental vitiligo. Sex, age of onset, sites involved, dermatomal distribution, margin of lesion and presence of poliosis were evaluated in both groups. The age of onset was less than 10 years in 96.8% of segmental nevus depigmentosus and 28.9% of segmental vitiligo cases. Trunk (36.5%) and cervical (38.1%) dermatomes were the most commonly involved in segmental nevus depigmentosus and face (67.1%) and trigeminal (64.4%) dermatomes in segmental vitiligo. The average number of dermatomes involved in truncal lesions was different in segmental nevus depigmentosus and segmental vitiligo (2.71 vs 1.62, P = 0.001). Segmental vitiligo on the face, neck and trunk appeared closer to the axis than segmental nevus depigmentosus (P < 0.001). Segmental nevus depigmentosus and segmental vitiligo showed significantly different margins (90.5% and 41.6% serrated, respectively; P < 0.001). We observed clinical differences between patients with segmental nevus depigmentosus and those with segmental vitiligo. Distribution (site, distance to axis, dermatome), vertical width, margin of lesion and presence of poliosis can be helpful in differentiating segmental nevus depigmentosus and segmental vitiligo.

Background Nevus depigmentosus (ND) is an uncommon congenital nonprogressive hypopigmented skin disorder that can be seen anywhere on the body. We considered other depigmenting disorders and focused on distinguishing ND from vitiligo and tuberous sclerosis complex in infancy. Methods The diagnosis of patients with nevus depigmentosus was made differentiating it from nevus anemicus, pityriasis alba, tuberous sclerosis complex, vitiligo and other depigmenting disorders. Results Of the 37 individuals with nevus depigmentosus evaluated, 36 were children, twenty-two (59.4%) were males and 15 (40.5%) were females, with male to female ratio 1.4:1. Conclusions Distinguishing ND from other disorders with depigmentation can be challenging, in particular in case of tuberous sclerosis complex and vitiligo in infancy.

Nevus depigmentosus (ND), also known as nevus achromicus or achromic nevus, is an uncommon congenital hypomelanosis of the skin that is often characterized as being nonprogressive and having serrated borders. It needs to be distinguished from other hypopigmented skin conditions such as nevus anemicus, hypomelanosis of Ito, Fitzpatrick patches (ash leaf spots) of tuberous sclerosis, vitiligo, indeterminate leprosy, and pigment demarcation lines. Treatment may be desired for aesthetic and possible psychosocial considerations. We review and update knowledge about ND and its simulants.

Background: Although vitiligo shows a similar clinical picture to other disorders in which patients exhibit depigmented spots, there are few reports on the differentiation of these conditions from each other. Purpose: We aimed to investigate the differences and similarities between vitiligo and nevus depigmentosus in children based on the clinical course and characteristics. Materials and Methods: We conducted an examination of the disease type, treatment, progress, and differentiation of 85 cases of vitiligo and nevus depigmentosus that developed before 15 years of age among patients who visited the Osaka University Department of Dermatology with a chief complaint of white patches from January 2010 to March 2017. Results: The median age at the onset of symptoms of 79 patients with vitiligo was 4.5 years and while that of 6 patients with nevus depigmentosus was 0.8 years, indicating that the onset of nevus depigmentosus occurred earlier. While vitiligo often developed in the head and neck regions, with a tendency for depigmented spots in multiple regions, nevus depigmentosus did not show this tendency in these regions. Upon performing Wood’s lamp observation in 38 cases of vitiligo, 35 cases were found to be fluorescent chalky white and in 3 cases histologically diagnosed as vitiligo, no clear fluorescent color was observed. All 6 patients diagnosed with nevus depigmentosus exhibited a non-fluorescent off-white color under a Wood’s lamp. Conclusions: This study indicates that non-invasive observation using a Wood’s lamp is useful in the differential diagnosis of pediatric leukoderma, in addition to the subsequent clinical course, such as the presence or absence of lesional repigmentation and/or enlargement.

Childhood vitiligo differs from adult-onset vitiligo for several features including increased incidence of the segmental variant, higher prevalence of halo nevi, and more common family history for autoimmune diseases and atopic diathesis. The major differential diagnoses are the postinflammatory hypomelanoses for nonsegmental vitiligo and nevus depigmentosus for segmental vitiligo. From a therapeutic standpoint, early awareness of the diagnosis seems to correlate with a good treatment outcome in this age group.

One of the most frequent complaints in dermatology clinics is the eruption of hypopigmented patchy skin lesions. The aim of the study was to investigate the utility of dermoscopy in common hypopigmented macular diseases. Patients with the followings diseases were examined by dermoscopy: vitiligo, pityriasis alba, nevus depigmentosus, achromic pityriasis versicolor, idiopathic guttate hypomelanosis, and extragenital guttate lichen sclerosus. This study showed that these hypopigmented macular diseases might display specific dermoscopic features. In vitiligo, the mean dermoscopic features were the presence of a diffuse white glow with perifollicular pigment, perilesional hyperpigmentation, leukotrichia and the pigmentary network. In idiopathic guttate hypomelanosis, the characteristic features were the presence of multiple, shiny, scaly macules with welland ill-defined edges borders that coalesced into polycyclic macules. For nevus depigmentosus, the mean features were hypopigmented patches with irregular border with a faint reticular network. For pityriasis alba, the fairly ill-demarcated hypopigmented macules with fine scales were the mean feature. In lichen sclerosus, there were white structureless areas, perilesional erythematous halo, follicular plugging and white chrysalis like structures. Dermoscopy of achromic pityriasis versicolor showed a fairly demarcated white area with fine scales localized in the skin creases.

The condition often starts in childhood and may spread, ultimately causing total depigmentation, or persist, with irregular remissions and relapses. Excessive pigmentation is known as hyperpigmentation and decreased pigmentation is known as hypopigmentation. Nascent patches of vitiligo are more easily identified if the skin is examined with UV light illumination. UV light may also enhance the appearance of follicular pigmentation within patches of vitiligo. Vitiligo may occasionally be associated with regression of pigmentation in malignant melanoma and it is wise to examine the entire skin surface of patients presenting with the condition. Examination in long-wave UVR enhances loss of a epidermal pigment and helps identify areas of developing depigmentation. Hypomelanosis of Ito is identified by the typical whorled patches of a hypopigmentation along the lines of Blaschko. Nevus depigmentosus manifests as a circumscribed area of depigmentation on the posterior trunk.

Amyand’s hernia is a very rare clinical condition characterized by the presence of a normal or inflammed appendix within the inguinal hernia sac. It may be present as an acute apendicitis inside the sac or incarcerated hernia. Sometimes it may be asymptomatic. We report a case of 70-year-old male patient that was presented to our emergency service with a huge right inguinal mass that was diagnosed as an incarcerated inguinal hernia and underwent operation. The intraoperative findings included small intestinal segment, large omental tissue and mobile cecum with healthy appendix inside the sac. There was no sign of strangulation. Lichtenstein herniorhaphy was done without appendectomy.

Acquired or genetic condition and localized or generalized hypomelanosis may have similar clinical presentation with vitiligo. Common differential diagnoses of vitiligo are nevus depigmentosus, idiopathic guttate hypomelanosis, and tinea versicolor. Various conditions have similar clinical presentation with vitiligo. The differential diagnosis of vitiligo is broad which ranges from occupational iatrogenic chemical leukoderma, genetic syndromes, malformations, idiopathic, infections, neoplastic, and postinflammatory hypopigmentation. Good history taking, a thorough physical examination, and the judicious use of histopathology generally yield a straightforward diagnosis.

This chapter provides an overview of the disorders of pigmentation that are most commonly seen by pediatricians, and highlights those conditions specifically requiring additional laboratory screening. The chapter is divided into four sections: congenital localized alterations in pigmentation (e.g. nevus depigmentosus), congenital generalized alterations in pigmentation (e.g. albinism), acquired localized alterations in pigmentation (e.g. tinea versicolor) and acquired generalized alterations in pigmentation (e.g. vitiligo). The first two categories are separated from the latter two by onset before the age of 2 years, in most cases.

Background Nevus depigmentosus (ND) is frequently confused with vitiligo. Differential diagnosis can be difficult. In vivo reflectance confocal microscopy (RCM) is a noninvasive technique for real-time en face imaging of the superficial layers of the skin down to the superficial dermis with cellular level resolution close to conventional histopathology. In this study, we tried to use this new technology to study the features of the distribution of pigment cells of these two hypopigmentation disorders and then concluded the differential features. Methods Sixty vitiligo patients and 62 ND patients were enrolled in the study. Three points in each patient (lesional, margin of the lesions and adjacent non- lesional points) were examined with RCM. The gray value of image was quantified using software, and we calculated the relative gray value. Results The RCM image feature was different between vitiligo and ND patients. The differential diagnosis was made based on the following four RCM features: complete absence of pigment cells; the distribution of pigment cells; the margins; and the relative gray value. Conclusion RCM can be used as an auxiliary diagnostic tool for the differential diagnosis between vitiligo and ND.

Background/purpose: Hypopigmentary skin disorders such as vitiligo, nevus depigmentosus and nevus anemicus are common diseases in clinic. The lesions of these diseases could be similar to some extent, although each of them has its own characteristic clinical appearance and histological features. Clinically, the atypical lesions are often difficult to be differentiated. In vivo reflectance confocal microscopy (RCM) is a non-invasive, repetitive imaging tool that provides real-time images at a nearly cellular histological resolution. Our aim was to investigate the RCM features of vitiligo, nevus depigmentosus and nevus anemicus. Subjects and Methods: A total of 135 patients with a clinical diagnosis of the aforementioned diseases were included in this study. The RCM images from depigmented skin, border of the white macules, adjacent normal-appearing skin and distant normal skin for all patients at the dermo-epidermal junction (DEJ) level were investigated. Results: In the active phase of vitiligo (AVP), the RCM demonstrated a complete loss of melanin in lesional skin in eight (53; 15.1%) patients. In 45 patients (53; 84.9%) of the AVP, part of the bright dermal papillary rings normally seen at the DEJ level disappeared or part of the rings lost their integrity and the content of melanin decreased obviously. In 20 patients (53; 37.7%) of the AVP, highly refractile inflammatory cells could be seen within the papillary dermis in the lesional and adjacent normal-appearing skin, which may indicate the lesion progresses. In addition, part of the dermal papillary rings showed lack of integrity or their brightness decreased in adjacent normal-appearing skin in all the patients of the AVP. It is important to know that the RCM demonstrated an ill-defined border. In the stable phase of vitiligo (SPV), the RCM demonstrates a complete loss of melanin in lesional skin and a clear border in 31 (41; 75.6%) patients; the content of melanin and dermal papillary rings in adjacent normal-appearing skin show no changes. In 10 (41; 24.4%) patients, the dendritic and highly refractile melanocytes arose in the recovery phase of vitiligo, which may indicate the repigmentation of vitiligo. There are three kinds of repigmentation patterns under RCM: marginal, perifollicular and diffuse. Distant normal skin showed no difference from controls in both the active and the SPV. In all the patients with nevus depigmentosus, the content of melanin decreases obviously but the dermal papillary rings are intact. The dermal papillary rings show no differences between lesional skin and adjacent normal-appearing skin of nevus anemicus. Conclusion: Considering our results, RCM may be useful to non-invasively discriminate vitiligo, nevus depigmentosus and nevus anemicus in vivo.

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.

1 Journal of Investigative Dermatology (2015), Volume 135 © 2015 The Society for Investigative Dermatology QUESTIONS 1. A 37-year-old man with history of diabetes mellitus type 1 presented for evaluation of the lesions pictured above. They are asymptomatic and have been slowly increasing in size and number over the past few years. What is your diagnosis? a. Tinea versicolor. b. Vitiligo. c. Nevus depigmentosus. d. Postinflammatory hypopigmentation. Cells to Surgery Quiz: June 2015 Leyre Falto-Aizpurua1, Mohammad-Ali Yazdani Abyaneh1, Brian J. Simmons1, Fleta N. Bray1, Mohammed Alsaidan1 and Keyvan Nouri1 Journal of Investigative Dermatology (2015) 135, e14. doi:10.1038/jid.2015.136

Background/purpose: Vitiligo and nevus depigmentosus (ND) present similar hypopigmented macules with significantly different prognoses. Although the distinction between the two diseases is important, differential diagnosis relies on medical history and physical examination, which is far from decisive in some cases. The Mexameter® is an objective skin color-measuring device, and has been reported to provide a reproducible and sensitive means of quantifying small skin color differences. In this study, we investigated the usefulness of a Mexameter® for discriminating these diseases. Methods: A selection of 202 hypopigmented skin lesions (182 from vitiligo and 20 from ND) were the objects of this study. Using a Mexameter, MIs were obtained from lesions and symmetrically located control skin. RMIs, ratios of the MIs of lesional skins to control skins, were calculated. Results: The mean MIs and RMIs were significantly different for vitiligo and ND. The mean RMI of ND lesions was 74±13, which was significantly higher than that of vitiligo lesions (50±24). No ND lesion had an RMI of

Vitiligo is a refractory skin disease. To investigate the risk factors and treatment responses of patients with vitiligo in Japan, we recorded and analyzed the details of 713 vitiligo patients (comorbidity, treatment responses, family history, age, and sex) who visited the dermatology clinic of the Nagoya City University Hospital, Nagoya, Japan between January 2004 and August 2010 (mean age, 35.2 years; 302 men, 411 women) using logistic regression analysis. The results are expressed as odds ratios (OR) with 95% confidence interval (CI). Patients were diagnosed with vitiligo [n = 644; 338 generalized type (47.4%), 170 segmental type (23.8%), and 136 localized type (19.1%)], nevus depigmentosus (n = 53, 7.4%), halo nevus (n = 14, 2.0%), and hypomelanosis of Ito (n = 2, 0.3%). For generalized and localized types, none of the analyzed factors were statistically significant. For the segmental type, antinuclear antibody (OR = 1.005; 95% CI, 1.00–1.01; p 55 years (OR = 0.0171; 95% CI, 0.00333–0.0879; p

The use of confocal laser scanning microscopy (CLSM) may be an eligible alternative for confirmation of the diagnosis of hypopigmented macules. Our purpose was to evaluate CLSM features for non-invasive imaging of vitiligo, nevus depigmentosus and postinflammatory hypopigmentation in vivo. A total of 68 patients with a clinical diagnosis of the aforementioned diseases were included in this study. CLSM was performed on lesional and adjacent normal appearing skin for all patients. In the active and stable phases of vitiligo, CLSM demonstrated a complete loss of melanin in lesional skin in 14 of 25 patients (56.0%) and 16 of 20 patients (80.0%), respectively. In 11 of 25 (44.0%) patients, the amount of melanin in lesional skin decreased in the active phase of vitiligo, but it is noteworthy to know that the melanin was distributed homogeneously in the dermal papillary rings. In four of 20 patients (20.0%), the dermal papillary rings disappeared completely, but some refractile granules and dendrites could be seen in the stable phase of vitiligo, which may indicate the start of vitiligo repigmentation. Although, in 20 of 20 patients (100%) with nevus depigmentosus, the dermal papillary rings lost their integrity and the content of melanin decreased obviously, there must have been melanin in the dermal papillary rings during its development in all patients. Simultaneously, the melanin was distributed heterogeneously in the dermal papillary rings. The content of melanin and dermal papillary rings in postinflammatory hypopigmentation probably depend on the depth and site of the inflammation; moreover, melanophages were observed in postinflammatory hypopigmentation but did not exist in vitiligo and nevus depigmentosus. In addition, the content of melanin and dermal papillary rings in adjacent normal appearing skin showed changes in the active phase of vitiligo but showed no changes in any of the patients in the stable phases of vitiligo, nevus depigmentosus and postinflammatory hypopigmentation. Differences based on CLSM in the aforementioned diseases were the content of melanin and its distribution pattern. CLSM may be useful to discriminate vitiligo, postinflammatory hypopigmentation and nevus depigmentosus in a non-invasive fashion.

Childhood vitiligo differs from adult onset vitiligo in several features, including more segmental forms, higher prevalence of halo nevi, and more common family history for autoimmune diseases. The major differential diagnoses are the post-inflammatory hypomelanoses for non- segmental vitiligo (NSV) and nevus depigmentosus for segmental vitiligo (SV). Thyroid anomalies are detected in NSV only, and actually The course of childhood vitiligo is not well-known, since there is a lack of long-term follow-up studies.

The most common disorders of hypopigmentation in children are pityriasis alba, vitiligo, nevus depigmentosus, and tinea versicolor. Pityriasis alba usually presents as ill defined, scaly patches of hypomelanosis on the cheeks of children with an atopic diathesis. The face is also a favored site for vitiligo, but the distribution is periorificial, and the pigment loss is complete because of a destruction of melanocytes. Vitiligo is an acquired, progressive disorder in contrast to nevus depigmentosus, which is a stable, congenital leukoderma. The localized form of nevus depigmentosus must be distinguished from an ash leaf spot, the earliest cutaneous manifestation of tuberous sclerosis, whereas the systematized form may be confused with hypomelanosis of Ito, another neurocutaneous disorder. The lesions of tinea versicolor favor the upper trunk of adolescents, and potassium hydroxide examination of the associated scale reveals hyphal and yeast forms of P. orbiculare. Any inflammatory process in the skin such as dermatitis or psoriasis can resolve with areas of hypopigmentation.

Summary Background. Naevus depigmentosus (ND) is a congenital, nonfamilial, well-circumscribed, uniformly hypopigmented macule, the relative size and distribution of which is stable throughout life. The aetiopathogenesis of ND is not yet fully understood, and reports about the clinical and histopathological characteristics of ND are few. Objective. To investigate the clinical and histopathological characteristics of ND, and to make it easier to diagnose ND clinically. Methods. A clinical survey on 38 patients with ND was performed according to the diagnostic criteria proposed by Coupe. Wood’s lamp examination was used to distinguish the different appearance of ND and vitiligo. Skin-biopsy specimens were stained with haematoxylin and eosin, silver, antibodies to S-100 protein, tyrosinase-related protein-1 and tyrosinase, then used for ultrastructural study. Melanocytes were also cultured. Results. Leucoderma was present at birth in 13 patients (34.2%), and appeared during the first 3 years of life in 15 patients. The trunk was the most commonly affected site, and the lesions usually had serrated, irregular borders. Under Wood’s lamp, lesions had an off-white accentuation without fluorescence. Immunohistochemistry showed that the melanin content of ND lesions was decreased compared with perilesional normal skin, but there was no change in the number of melanocytes. Ultrastructural study showed that some aggregated melanosomes were present in the affected keratinocytes. Conclusion. As a result of the above findings, we suggest changes to Coupe’s criteria for ND.

Nevus depigmentosus is an uncommon hypopigmented macule or patch that is congenital and stable in its relative size and distribution throughout life; it occurs sporadically and may be localized, segmental or, less often, systematized. We report the case of a 17-year-old girl with a segmental achromic nevus of the left leg and a patch on the lower back with late age of onset who developed lentigines after prolonged intense ultraviolet B exposure as a consequence of an incorrect diagnosis of segmental vitiligo.

Background: Vitiligo is a systemic autoimmune disease due to the loss of melanocytes from the epidermis. Very few reports in the literature are available about the ocular findings and associated cutaneous abnormalities in childhood vitiligo. Aims: Objective of this study was to evaluate the ocular findings, various clinical characteristics and associated cutaneous abnormalities of childhood vitiligo. Materials and Methods: We retrospectively analyzed the clinical data of children with vitiligo who presented to the Dermatology Outpatient Department from August 2011 to August 2014. All patients were assessed for the natural history, clinical characteristics, family history, and associated ocular and cutaneous abnormalities. Results: Of the total 180 children with vitiligo studied, 64 (35.6%) were boys and 116 (64.4%) were girls. The mean current age of the children visiting our hospital was 8 years. History of trauma prior to the onset of vitiligo was present in 13 patients (7.2%). The family history of vitiligo was present in 37 patients (20.6%). Most common clinical type of vitiligo seen in our patients was vitiligo vulgaris (n = 68, 37.8%), followed by segmental (n = 41, 22.8%). The most common site of initial lesion was head and neck followed by lower limbs. Leukotrichia was seen in 65 patients (36.1%), while Koebner phenomenon was observed in 48 children (26.7%). Vitiligo disease activity (VIDA) score of +4 was most commonly seen in 108 patients (60%), followed by +3 seen in 20 patients (11.1%). VIDA score 0 and −1 were seen in 15 (8.3%) and 22 (12.2%) patients, respectively. Cutaneous associations with vitiligo were found in 24 patients (13.3%). These were halo nevi in nine patients (5%), atopic dermatitis in six patients (3.3%), alopecia areata in four patients (2.2%), premature canities in three patients (1.7%), and nevus depigmentosus and lichen nitidus in one patient each (0.6%). Thirty-eight patients (21.1%) were found to have periocular depigmentation. Depigmented spots in the iris were seen in two patients (1.1%). Other findings were lamellar cataract and persistent papillary membrane in one patient each (0.6%). Conclusion: Childhood vitiligo in our study showed preponderance in females. Majority of patients (77.9%) had

Nevus depigmentosus (ND) is a congenital leukoderma that is, stable in its size and distribution and may present as isolated, segmental or systematized form. Segmental ND sometimes can pose a diagnostic difficulty with segmental vitiligo. Likewise, segmental vitiligo overlaps with the clinical features of ND to a greater degree. A 15-year-old boy presented with two distinct asymptomatic pigmentary lesions involving the right thigh. Based on the clinical features, Wood's lamp examination and histological features a diagnosis of vitiligo arising within the confines of segmental ND was entertained. Though there are reports of vitiligo coexistent with ND in two different sites, and development of lentigines within ND, vitiligo developing within the preexisting ND makes this case an interesting presentation.

Background The authors report a case of Hypomelanosis of Ito (HI), a rare neurocutaneous syndrome associated with musculoskeletal, neurological alteration and ophthalmologic abnormalities. Methods A 9 month old girl was brought for evaluation of hypopigmented skin lesions and strabismus. Clinical examination revealed whirled hypochromic skin lesions only in the left side of the body (neck and trunk) following the lines of Blaschko, compatible with HI and hypotonia. She presents also: strabismus, genu valgus and pubis pilosity. She attained head control at 4 month and failure to sit without support. Results The blood tests, hormonal tests, abdominal and cranial ultrasound were normal. Electroencephalogram revealed diffuse cortico-subcortical dysfunction. MRI of head was normal. Blood karyotyping showed mosaicism. Differential diagnosis: nevus depigmentosus, tuberous sclerosis, vitiligo. Treatment: no treatment for the cutaneous lesions. Appropriate specialty care for orthopaedic and visual problems. Conclusions Numerous complications have been associated with HI involving the central nervous system, musculoskeletal, ophthalmologic alteration and urogenital malformation. Many chromosomal abnormalities have been reported in association with HI. The prognosis of HI is determined by de associated abnormalities.

Background: Nevus depigmentosus is defined as a congenital nonprogressive hypopigmented macule or patch that is stable in its relative size and distribution throughout life. The pathogenesis and histopathologic characteristics of nevus depigmentosus is not yet fully established. Objective: The purpose of this study was to investigate the clinical and histopathologic characteristics of nevus depigmentosus as well as its pathogenesis. Methods: A clinical survey was done with 67 patients diagnosed as having nevus depigmentosus. Two skin biopsy specimens each were taken from 18 patients: one from the central part of the depigmented lesion and another from the border of the lesion, including peri-lesional normal skin. The sections were stained with hematoxylin-eosin, Fontana-Masson, and S-100 protein. Ultrastructural evaluation was also done to detect changes of the melanocytes. Results: The lesions were mostly present before 3 years of age (92.5%), but some lesions also appeared later in childhood (7.5%). The back and buttocks were the most commonly affected sites, followed by the chest and the abdomen, the face, the neck, and the arms. Forty patients (59.7%) had the isolated type of nevus depigmentosus and 27 patients (40.3%) had the segmental type. Histopathologic studies showed that the staining ability of Fontana-Masson in nevus depigmentosus lesions decreased compared with perilesional normal skin. However, there were no changes in the numbers of melanocytes identified as S-100-positive cells in the basal layer. Electronmicroscopic studies revealed a great reduction in the number of melanosomes in melanocytes and some membrane-bound aggregated melanosomes were observed in keratinocytes. Conclusion: The results of this study support the hypothesis that nevus depigmentosus is caused by the functional defects of melanocytes and the morphologic abnormalities of melanosomes.(J Am Acad Dermatol 1999;40:21-6.)

A particular pigmentary disorder develops on the skin of Chinese children. The lesions are composed of whitish macules and patches arranged in linear bands on the extremities unilaterally. Histologically these lesions are hypopigmented chronic dermatitis. Although the lesions are arranged in a lichen striatus pattern, they differ clinically from lichen striatus by the absence of papules and plaques. These cases may represent a new entity. The differential diagnosis includes lichen striatus, vitiligo, piebaldism, nevus depigmentosus, and hypomelanosis of Ito. The differences between the disorder presented here and those of different diagnosis are discussed.

Nevus depigmentosus is a congenital disorder of pigmentation which occurs in all sexes and races. It commonly presents in childhood and is mostly nonprogressive. It is a form of cutaneous mosaicism with functionally defective melanocytes and abnormal melanosomes. Localized solitary lesions need to be differentiated from conditions like nevus anemicus, ash leaf macule, vitiligo, etc., and generalized lesions from hypomelanosis of Ito. Treatment is usually not required although cosmetic camouflage, phototherapy, grafting, and other modalities may be tried.

A 45-year-old Korean man had two distinct types of hypopigmented lesions on the forehead and back: vitiligo and nevus depigmentosus (ND). The hypopigmented macules on the forehead were incidentally discovered 15 days previously and responded well to steroid therapy. The hypopigmented macule on the back had been present since birth, was stable in size, and showed no response to steroid therapy. There were no remarkable differences between the two lesions in routine histopathology or Fontana-Masson staining. However, the lesion on the back was shown to contain melanocytes in electron microscopy (EM).

Nevus depigmentosus (ND) is a congenital, non-progressive, hypopigmented lesion that is usually stable throughout an affected individual's lifetime. The clinical features of vitiligo are similar to those of ND, but the two diseases have different treatment responses and prognoses. We report here on a rare case of vitiligo that was coexistent with ND. Both conditions were treated with narrow-band UVB. An 11-year-old boy presented with two distinct types of hypopigmented lesions, one on the forehead and the other on his back. The first was a hypopigmented patch with leukotrichia, and it was incidentally discovered 3 months before the child was examined at our clinic and it had rapidly increased in size. The second hypopigmented patch was detected at birth and it had slowly been increasing in size. The hypopigmented lesion on the child's forehead was diagnosed as vitiligo, and the one on his back as ND. Once- or twice-weekly narrow-band UVB treatment was initiated. Improvements in the two lesions were assessed with clinical photography and using a Mexameter® (Courage-Khazaka Electronic, Germany), which is a pigment-measuring device.