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Introduction Deep mycoses acquired by penetrating trauma to the skin can have varied and sometimes atypical morphological presentations resulting in diagnostic dilemmas and delay in treatment onset. Histopathology can be a useful tool in not only diagnosing but also differentiating various deep mycoses. Aims and Objectives To observe various morphological presentations and histopathological features of deep fungal infections. Materials and Methods A retrospective multi-centric study was conducted from 2010 to 2020 at 16 centres. The cases with diagnoses of various deep mycoses were included in the study. The patients presenting with cutaneous manifestations were included in the study. Their demographic details, history, presenting signs and symptoms, morphological presentations, histopathological features and treatment details were collected from the case sheets. Results A total of 124 cases were found from the case records. The most common type was chromoblastomycosis (42) followed by mycetoma (28) and rhinosporidiosis (17). The mean age was 43.76 ± 5.44 years. The average duration of symptoms before presentation was between 2 months to 10 years (average 2.5 ± 1.33 years). Male to female ratio was 1:0.7. Prior history of trauma was recorded in 36% of cases. Chromoblastomycosis cases presented with verrucous to atrophic plaques with black dots on the surface and histopathology findings included pesudoepitheliomatous hyperplasia, epithelioid cell granulomas, copper penny bodies within granulomas and abscesses. Rhinosporidiosis cases had polypoid grape-like lesions in the nose and eyes most commonly with histopathology findings of abundant thick-walled sporangia in dermis packed with thousands of spores. Eumycetoma patients had pigmented, indurated swelling with multiple sinuses discharging black granules and histopathology showed dermal abscesses and foreign body granulomatous reaction with PAS-positive hyphae. Histoplasmosis patients presented with few to multiple nodulo-pustular lesions on skin and palatal ulcers while small basophilic bodies packed in the cytoplasm of histiocytes were noted in histopathology. Phaeohyphomycosis cases presented as deep-seated cystic lesions and biopsy revealed deepithelialized cysts in the dermis or hypodermis with lumen showing necro inflammatory debris and fungal hyphae. Sporotrichosis cases had erythematous, tender nodules and papules either as single lesions or as multiple lesions arranged in a linear fashion and histopathology showed pseudoepitheliomatous hyperplasia of epidermis, loose to well-defined epithelioid cell granulomas and microabscesses. Spores were found in two cases. Cryptococcosis patient had multiple umbilicated lesions resembling giant molluscum contagiosum loose epithelioid cell granulomas and medium-sized spores lying in both intra and extracellularly on histopathology. Penicilliosis patients had nodulo-pustular lesions and histopathology showed an admixture of histiocytes, epithelioid cells, plasma cells, lymphocytes and polymorphs in the dermis with the presence of yeast-like spores in the cytoplasm of histiocytes and epithelioid cells. Entomophthoromycosis cases presented with asymptomatic subcutaneous firm swelling with loss of skin pinchability. Conclusion Though clinical findings of deep fungal infections are characteristic similar morphology and atypical presentations can be sometimes confusing. Histopathology is useful for confirming the diagnosis.

Introduction: Nitric oxide (NO) is an important biological mediator of inflammation which is exhaled after being produced in the lungs. Fractional exhaled NO (FENO) is a new tool which measures the amount of NO exhaled. Aims and Objective: The objective of our study was to find the correlation between the FENO levels between atopic dermatitis and asthma patients in comparison to healthy controls and hence identify steroid responsiveness. Methodology: Our study was a cross-sectional observational study performed in a tertiary care hospital involving 150 children (>5–

Background: Atopic dermatitis (AD) has a complex etiology that includes Th2 polarization, which is accompanied by the cytokines IL4, IL-5, IL-13, and IL-31, as well as Th17 and Th22, and in chronic lesions, Th1 cells. Tofacitinib inhibits Th1-, Th2-, and Th17-associated cytokines by selectively blocking JAK1 and JAK3 receptors. We conducted a multicentric, retrospective chart analysis to study the efficacy and safety of tofacitinib in patients with moderate to severe refractory AD. Materials and Methods: We included 16 adult patients (aged >18 years) with moderate to severe AD who had previously undergone systemic therapy with inadequate response. In the baseline, demographic data, previous treatment history, severity scores (eczema area and severity index [EASI] and SCORing Atopic Dermatitis [SCORAD]), and quality of life score (Dermatology Life Quality Index [DLQI]) were noted. Baseline blood investigations, including complete blood count, liver function test, renal function test, lipid profile, and interferon gamma release assay for tuberculosis, were done. Patients were followed up every month for 6 months that included documentation of severity scores, blood investigations, and DLQI. Any adverse events, if reported, were noted. Result: All 16 patients completed the 6-month trial. Our patients were previously treated with cyclosporine (n = 10), methotrexate (n = 3), or both (n = 3). The mean EASI scores improved from 23.38 ± 9.56 at baseline to 8.50 ± 7.57 at the end of 6 months. The mean SCORAD score improved from 41.25 ± 8.69 at baseline to 14.93 ± 7.82 at the end of 6 months. Quality of life also improved as the mean DLQI improved from 15.18 ± 2.73 at baseline to 5.31 ± 4.11 at the end of the study period. No severe adverse reactions were noted, but 3 patients experienced dyslipidemia and 2 patients had altered bleeding time. Conclusion: Tofacitinib is a safe and effective treatment option for recalcitrant moderate to severe adult AD.

Background: Xeroderma pigmentosa (XP) is a rare inherited (autosomal recessive) disease, resulting from impairment in DNA repair that involves recognition and repair of ultraviolet radiation (UVR)-induced DNA damage in the nucleotide excision repair pathway. This results in increased photosensitivity, UVR-induced damage to skin and eye, increased susceptibility to cutaneous and ocular cancers, and progressive neurodegeneration in some patients. Aims and Objective: The objective of the study was to describe the clinicopathological spectrum of eight cases of XP. Materials and Methods: An ambispective case series was conducted in a pediatric tertiary care hospital in eastern India during a 10-year period from 2013 to 2022. Results: All the children of our cohort were born of consanguineous marriage. The mean age of presentation was 1.2 years (range: 7 months–3 years), whereas three children presented during their infancy. The male-to-female ratio was 5:3. The most common findings were cutaneous (100%), followed by ophthalmic (75%) and/or neurological symptoms (25%). Patients had normal skin at birth but soon developed extreme photosensitivity followed by abnormal skin pigmentation and subsequently progressive xerosis, atrophy, wrinkling, and poikiloderma over time. Six patients had varied degrees of ocular involvement, whereas three of them had severe manifestations including madarosis, tylosis, ectropion, lagophthalmos, phthisis bulbi, clouding and scarring of the cornea with complete or partial loss of vision, and ophthalmic malignancies. Fifty percent (n = 4) of cases had cutaneous and ocular premalignant (actinic keratosis) and malignant lesions including melanoma and nonmelanoma skin cancer such as squamous cell carcinoma (SCC) and basal cell carcinoma (BCC) in their early childhood. One patient had simultaneous occurrence of multiple malignancies together (SCC, BCC, and melanoma). Neurological abnormalities (subnormal intelligence) were found in two cases. There was no evidence of sensory neural hearing loss, microcephaly, neuroregression, or neurodeficits. Conclusion: Although XP is associated with increased mortality and morbidity, early diagnosis followed by persistent vigorous photoprotection and regular screening for early detection of malignancies along with psychological support can drastically improve patients' quality of life and life expectancy. Besides genetic counseling, further research is required on formulating optimal management of XP, specifically the role and possibilities of gene therapy in XP.

Introduction: Atopic dermatitis (AD) is a chronic, inflammatory skin disorder that significantly impairs life quality and mostly affects children. Although classical approaches of treatment have been effective in mitigating the symptoms of AD, novel remedial measures based on a better understanding of immunopathogenesis are needed to target the underlying etiology. Aims and Objectives: Probiotics improve the intestinal micro environment and restore the balance of immune responses, thereby, emerging as a novel therapeutic option in the management of AD. Taking into account the updated information available, this review aims to discuss the beneficial effects of probiotics on gut microbiota, their role in strengthening the epidermal barrier and immunological responses, and hence their clinical usage in AD. Results: Classically, AD has been understood as a complex interplay between genetic susceptibility, barrier dysfunction, immune dysregulation, and exogenous factors. Current research has demonstrated that AD is associated with changes in both skin and gut microflora. The “gut-skin” axis hypothesis and the interaction between them have been demonstrated in research. Patients with AD have demonstrated gut dysbiosis, which leads to immune dysregulation and can serve as a potential trigger for AD. Probiotics, by their ability to put right gut dysbiosis, have demonstrated modest efficacy in reducing disease activity in clinical studies. Conclusion: Although multiple randomized controlled trials and meta-analyses have demonstrated the utility of probiotics as adjuvant therapy in the management of AD, the need of the hour is to develop precise guidelines pertaining to their usage in AD.

Vitamin K deficient bleeding (VKDB) disorder is a rare but fatal disorder that needs prompt diagnosis and timely intervention. Among its varied clinical manifestations, nodular purpura is rare one. Proper knowledge of this presentation helps clinicians to exclude other close differentials and avoid unnecessary delays in diagnosis. Though bleeding from the vaccination site could be a manifestation of VKDB, the concomitant presence of nodular purpura and vaccine-induced panniculitis in the same patient is rare. We report a case of a 4-month-old baby presenting with both, posing a diagnostic dilemma.

The time-centered routine consultations have been shown to be inadequate in imparting the necessary knowledge and practical skills for the children and the family suffering from atopic dermatitis. There is a need to complement medical treatment with adequate counseling or focused psychological and educational interventions, directed at the patient and the parents. The authors discuss the various approaches in “counseling” that has been used worldwide and propose a practical approach for the same in the Indian scenario.

Psoriasis is a common, chronic, immune-mediated, multisystem, inflammatory disorder. It affects all age groups, including infancy. In one-third of the cases, the onset of the disease is in the first and second decades of life. Childhood psoriasis significantly affects the quality of life of the child as well as that of the entire family. Pediatric psoriasis has distinct clinical presentations and evolves with time. Like in adults, chronic plaque psoriasis has been found to be the most common type of childhood psoriasis. Psoriatic plaques in children are less pruritic, smaller and thinner with less prominent scaling. In pigmented skin, the erythema is less prominent and plaques appear violaceous or hyperpigmented. Pediatric psoriasis can be associated with arthritis, metabolic syndrome, depression and anxiety. Hence all children should be screened routinely for associated comorbidities. Management of pediatric psoriasis is challenging owing to the limitation of approved therapies. 'Proactive therapy' is a recent approach in childhood-onset psoriasis that would help to prevent the severity of flare-ups, thus improving the quality of life.

Triple-A syndrome (Allgrove syndrome) is a rare, autosomal recessive disorder classically described with the triad of alacramia, achalasia, and ACTH-resistant adrenal insufficiency. Very few cases have been reported so far in the literature. Rarity leads to misdiagnosis. Phenotypic heterogeneity further adds to the confusion as classical triads are not constantly present in each and every case. The presence of additional autonomic disturbances in some cases brings about the proposal of the name 4A syndrome instead of 3A syndrome. Basic defect is mutation of achalasia, adrenocortical insufficiency, and alacramia syndrome gene. The entity needs multidisciplinary approach including endocrinologist, pediatrician, dermatologist, and ophthalmologist for best possible outcome. Although there is no definitive treatment, early diagnosis is extremely important to save life and prevent neurological sequel from adrenal crisis, to avoid unnecessary investigations and inappropriate treatment, and to improve quality of life. We report a 13-year-old boy with triple-A syndrome diagnosed clinically presented with repeated episodes of seizure, Addisonian pigmentation, the absence of tear, and difficulty of swallowing.

Lynch syndrome or hereditary nonpolyposis colorectal cancer (HNPCC) is a type of inherited cancer syndrome with a genetic predisposition to different types of cancer. There is an increased predisposition to cancers in the endometrium, colon, stomach, ovary, uterus, skin, kidney, and brain in patients of Lynch syndrome. We are reporting a 48-year-old male who presented with a pea-sized growth in his left arm which was found to be sebaceoma on histopathology. On further detailed history, examination, and genetic study, it was proved to be a familial case of Lynch syndrome. The case is being reported to stress the importance of knowledge about clinical manifestation, associated neoplasms, and molecular genetic profile of Lynch syndrome which will enable physicians and pathologists to provide highly targeted surveillance and management for patients with high cancer risk.

Infantile systemic hyalinosis (ISH; MIM #236490) and juvenile hyaline fibromatosis (MIM #228600) represent two spectrums of the rare autosomal recessive disorder, the hyaline fibromatosis syndrome caused by mutations in ANTXR2/CMG2 encoding capillary morphogenesis protein-2. Herein, we report two cases of ISH with different clinical presentations confirmed by CMG2 gene mutations. Homogenous 79 bp deletion of the entire exon 11 reported in one of the cases has not been reported previously.

Introduction: Treatment of moderate to severe atopic dermatitis (AD) is a real challenge for the dermatologists. Dupilumab is the first targeted biologic therapy approved for the treatment of children and adults with moderate-to-severe AD. The efficacy and safety of dupilumab in Indian patients is limited to date, it is necessary to assess the performance of this treatment in real clinical practice in the Indian context. Methodology: Patients from three centers of India, two from Kolkata and one from Bangalore were included in the study for retrospective chart analysis. Efficacy was assessed by comparing the SCORAD and EASI and impact on quality of life was assessed by DLQI scores. All patients received standard doses of Dupilumab. Any side effect of the treatment was noted in the bi-weekly follow-up visit. Results: Twenty-five patients who were treated with dupilumab for at least 6 months were retrospectively included to study. The mean EASI score improved from 19.48 at baseline to 4.84 at six months. Seventeen patients (68%) achieved EASI 75 (≥75% improvement from baseline) at the end of 6 months of treatment. All these patients were earlier treated with at least one systemic immunomodulator without any significant improvement. The mean SCORAD score also improved with dupilumab treatment from 37.32 at baseline to 8.04 at six months. The improvement were found to be statistically significant (P < 0.001). The quality of life also improved significantly (P < 0.001) from a baseline mean of 17.08 at baseline to 6.52 at 6 months. Conclusions: We observed significant efficacy, tolerability, and safety of dupilumab in Indian patients with AD in a real-world setting, which was similar to that shown in clinical trials in the western populations.

Extramammary Paget's disease is an intraepithelial neoplasm, usually found in areas rich in apocrine gland concentration. The clinical features, histopathology, immunohistochemistry and management details of five patients (F = 3, M = 2) have been described here. While a well-defined persistent plaque with crusting and erosion was the most common presentation, hyperpigmentation, hypopigmentation and depigmentation were also observed in two patients. Characteristic Paget's cells with cytokeratin 7 and EMA positivity were seen on histopathology examination. Authors conclude that pigmentary alterations may be under-reported in extra mammary Paget's disease in the skin of colour.

Psoriasis is a chronic, inflammatory, papulosquamous disorder with a variable clinical spectrum. Although it affects population of all age groups, the significance and negative impact of childhood psoriasis is often neglected worldwide. In nearly one-third of the psoriatic population, psoriasis appears during the childhood. In this article, we have focused on different issues and challenges faced during the management of childhood psoriasis. In addition to the varied clinical spectrum and associated comorbidities, in this article, we have given emphasis on the following real needs for childhood psoriasis: (i) psychological and social effects on the affected children, (ii) impact on their family members, and (iii) early diagnosis and management of psoriasis on children with psoriasis. We have concluded the article with the “concept of total care,” which is a third dimension for the management of psoriasis.

Background: Atopic dermatitis (AD) is a common and chronic, pruritic inflammatory skin condition that affects all age groups. There was a dearth of consensus document on AD for Indian practitioners. This article aims to provide an evidence-based consensus statement for the management of AD with a special reference to the Indian context. This guideline includes updated definition, etiological factors, classification, and management of atopic dermatitis. Methodology: The preparation of guidelines was done in multiple phases. Indian Dermatology Expert Board Members (DEBM), recommended by the Skin Allergy Society of India, prepared 26 evidence-based recommendations for AD. An extensive literature search was done in MEDLINE, Google scholar, Cochrane, and other resources. Articles published in the past 10 years were reviewed and recommendations were graded based on the quality of evidence as per GRADE. After forming the initial structure, DEBM met in Mumbai and gave their decisions on an agree and disagree scale with an Indian perspective. Finally, their suggestions were compiled for preparing the article. After DEBM finalized the draft, a treatment algorithm was formulated for the management of AD. Results: DEBM suggested a working definition for AD. The panel agreed that moisturizers should be used as mainstay of therapy and should be continued in all lines of therapy and in maintenance phase. Topical corticosteroids and topical calcineurin inhibitors should be considered as the first line of treatment. Among systemic therapies, cyclosporin should be considered first line, followed by azathioprine, methotrexate, and mycophenolate mofetil. Phototherapy can be an effecive alternative. Empirical food restriction was recommended against. Conclusion: These guidelines should form a reference for the management of patients with AD in an evidence-based manner.

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Granuloma annulare (GA) is a benign granulomatous dermatosis. Clinically, it has been classified into localized, generalized, subcutaneous, macular, erythematous, and perforating types. Generalized GA (GGA) is an unusual pattern of GA reported mostly in adults aged 50 years or over and children aged about 10 years. Occurrence in infants is very rare and few cases of papular GGA have been reported in infants. We report a case of annular pattern of GGA in a 3-month-old infant. This is the first case report of annular pattern GGA in an infant.

Chronic mucocutaneous candidiasis (CMC) is a clinical entity where extensive fungal infection of skin, hair, nail, and mucosa with Candida sp. is seen. It has got association with several immunological and endocrinal dysfunctions. We report a case of a 14-year-old boy who presented with peculiar facies, gross failure to thrive, fungal granulomatous lesions with scaring alopecia, oropharyngeal candidiasis, ectodermal dystrophy, grade 3 clubbing of all four limbs, interstitial keratitis with leukoma due to recurrent corneal ulcerations, and persistent deciduous teeth. A diagnosis of CMC was made and evaluation of immunological pathways revealed a high titer of immunoglobulin E. CMC with overlapping features of autoimmune polyendocrinopathy candidiasis ectodermal dystrophy syndrome and hyper IgE syndrome is extremely rare in literature. Timely diagnosis of an underlying etiology and proper treatment of a case of CMC may provide a better quality of life of the patient.

Atopic dermatitis (AD) coexisting with allergic contact dermatitis (ACD) is not uncommon. There has been lot of controversies regarding this concept as the prevalence of ACD in AD is similar to that seen in nonatopics. There is increased susceptibility to ACD in AD as AD modulates the exposure to allergens. There is an impaired barrier function that predisposes to the development of ACD in AD. Patch testing is a standard test for detecting allergens in AD.

Introduction: Acanthosis nigricans (AN) is a frequently encountered entity. Facial AN (FAN) is a subset of AN which is being increasingly recognized. Recently, reports hypothesizing the association of FAN with features of metabolic syndrome have been published. Aims and Objectives: The aim of this study was to study the clinicodemographic profile of patients with FAN, and to assess the correlation of hypertension, increased waist–hip ratio (WHR), increased body mass index (BMI), type 2 diabetes mellitus, deranged lipid profile, serum insulin, and impaired oral glucose tolerance test (OGTT) (parameters of metabolic syndrome) in these patients, as well as to determine the most significant predictor (highest relative risk) of development of FAN. Methods: A multicentric case–control study was conducted (123 cases in each group) over a period of 2 years. Data were obtained on the basis of history, examination, and relevant laboratory investigations. Statistical analysis was done using Statistica version 6 (StatSoft Inc., 2001, Tulsa, Oklahoma, USA), SPSS statistics version 17 (SPSS Inc., 2008, Illinois, Chicago, USA), and GraphPad Prism version 5 (GraphPad Software Inc., 2007, San Diego, California, USA). Results: Mean age of the patients with FAN was 38.83 ± 8.62 years. Mean age of onset of the disease was 30.93 ± 8.18 years. The most common site of face involved was the forehead and temporal region. The most common pigmentation was brown-black. Male sex, positive OGTT, increased WHR, and increased BMI were most significantly related to FAN. Smoking was found to have a protective effect against the development of FAN. Conclusion: Here, we document a significant association between male patients with positive OGTT, increased WHR, and BMI and FAN. Thus, we propose that FAN could be considered a morphological marker of metabolic syndrome.

Multiple café au lait macules are one of the cornerstones in diagnosing neurofibromatosis Type 1 (NF 1). NF 1 is often a multisystem neurocutaneous disorder, plexiform NF being one of the most important variants of this phacomatosis. Here, a rare case of a 2.5-year-old male child without positive family history has been described who presented with multiple large café au lait spots, alopecia, dysmorphic facies, proptosis, protrusion and deviation of tongue, biphasic stridor, bilateral neck swelling, and abnormal gait due to foreshortened left leg. Magnetic resonance imaging revealed abnormal soft-tissue infiltration of cavernous sinus through sphenoid bone, retro-orbital, retropharyngeal, parapharyngeal spaces, floor of mouth, and encasement of unilateral neck vessels and compression over trachea. Indirect laryngoscopy revealed unilateral vocal cord palsy most likely due to recurrent laryngeal nerve involvement. Biopsy confirmed the diagnosis of NF. Pediatricians and dermatologists should be aware of syndromic causes and their varied presentations while encountering a child with multiple large café au lait spots.

An 8-year-old male child presented with complaints of pain abdomen and appearance of rashes all over the body, with pain and swelling of the left knee joint. The child was a known case of developmental delay with seizure disorder, and the rashes had appeared just after starting oral valproate. On examination, the child appeared well except for the rashes and local swelling with decreased range of motion in the left knee joint. Redness around the urethral meatus was also noted. Skin biopsy revealed features suggestive of reactive arthritis. The child responded well to nonsteroidal anti-inflammatory drugs.

Ecthyma gangrenosum (EG) is a severe invasive cutaneous infection caused classically by Pseudomonas aeruginosa typically affecting immunocompromised patients especially those with neutropenia. We report the case of a 2-year-old boy with autoimmune lymphoproliferative syndrome presenting with solitary EG on the forehead. Blood culture, as well as culture from the lesion both, showed growth of P. aeruginosa. Pseudomonal sepsis responded well to piperacillin-tazobactam and amikacin therapy. Prompt diagnosis of the lesion is crucial as failure to start timely appropriate therapy may lead to fatal outcome.

Diapering practices vary among different countries from the use of cloth diapers to the modern disposable diaper. In the last few decades, diaper technology has advanced significantly thus decreasing the prevalence of diaper dermatitis. Recent innovative techniques used in the manufacturing of diapers include incorporation of superabsorbent polymer gel that can absorb 30 times its weight in liquid. Recently, smart diapers have been developed which not only prevents diaper dermatitis but significantly has reduced the burden on parents. This article reviews how changes in disposable diaper technology have improved diapering practices.

Erythrokeratodermia variabilis (EKV) are a rare heterogeneous group of inherited cornification disorders. They are characterized by two distinct morphological types of skin lesions: Fixed hyperkeratotic plaques and sharply marginated, pruritic, and migratory erythematous lesions. We report three cases of EKV along with a review of literature.

Food allergy in atopic dermatitis (AD) is debatable from decades. Role of diet in the cause and treatment of AD is controversial and is not well-defined. Allergists and pediatricians are convinced about the food allergy in AD whereas many dermatologists are contrary for this. However, there are studies in the Indian and western literature supporting the evidence that elimination diet may improve the severe type of AD. There is increasing awareness and lot of misconception among caregivers about food allergy and hence careful understanding about this concept is necessary to counsel parents. Recent evidence-based literature suggests avoidance of proven food allergens in AD could be beneficial in moderate to severe type of AD.

Bullous mastocytosis (BM) is a rare variant of diffuse cutaneous mastocytosis where there is disorderly infiltration of the skin by mast cells. We report a 4-month-old male infant with this rare disease who presented with wrinkled tough skin and generalized multiple bullous lesions. Diagnosis was done by serum tryptase level and histopathological examination. The patient responded well to oral corticosteroid and antihistaminic therapy. Although a congenital disorder, timely diagnosis and treatment can be potentially rewarding in patients with BM.

Henoch–Schonlein purpura (HSP) with facial involvement with bullous rashes are extremely rare. A 12-year-old boy presented with abdominal pain and features of arthritis. He also had multiple purpuric rashes over his lower limbs. Gradually, he developed bullous rashes which were seen on his legs and hands and progressed to involve the face. He was confirmed to be suffering from HSP from clinical presentation and skin biopsy. The child responded well to oral steroids. Bullous lesions may be seen in HSP. However, there is neither prognostic significance of this nor does it alter the management. Other causes of bullous lesions should be ruled out. As facial involvement is associated with renal and gastrointestinal involvement, these children should be monitored for sequelae.

Central nervous system involvement in segmental nevus depigmentosus (SND) is rare. A 7-month-old boy having convulsion and segmental hypopigmented patch in the right inguinal region. Magnetic resonance imaging of brain showed bilateral periventricular white matter hypoplasia with prominent subarachnoid spaces and mild dilation of ventricles with mild left cerebral hemispheric atrophy. Association of SND with seizure and white matter lesion has been rarely reported.