Langerhans cell histiocytosis in children: A retrospective case series of 126 cases.

BACKGROUND/OBJECTIVES: Langerhans cell histiocytosis (LCH), a rare neoplasm of hematopoietic myeloid precursor cells, is clinically characterized by spontaneously resolving lesions to a progressive life-threatening multisystem disorder. Diagnosing LCH in children is challenging as it mimics other skin disorders. This study describes the varied clinical presentation and disease course in children less than 18 years diagnosed with LCH. METHODS: We performed a retrospective observational study of all cases diagnosed with LCH presenting to a children's hospital in the last 26 years. Data on history, cutaneous and systemic examination, and laboratory evaluation performed, were recorded. RESULTS: A total of 126 children diagnosed with LCH were included in the study. There were 68% cases limited only to skin, and 32% children with multisystem involvement at the initial presentation. Scaly papules were the most common morphologic finding in skin. The skeletal system was the second most common organ system to be affected. Failure to thrive was a common symptom. Progression of skin to systemic involvement was seen in 27.9%. In 76.7%, skin lesions cleared over a period of 2 to 4 years. Complete remission was seen in 56.9% of children over a period of 3 to 7 years, while 8.1% children died of complicationsand 31.8% were lost to follow-up. CONCLUSIONS: Long-term follow-up in this study has shown cutaneous LCH without systemic involvement has a good prognosis. Skin involvement,along with failure to thrive, was the most common clinical presentation in our study. The skeletal system was the second most common organ system involved.

A Clinicopathological and Immunohistochemical Profile of Primary Extra Mammary Paget's Disease in Skin of Colour: A Case Series.

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.

Filaggrin gene polymorphisms in Indian children with atopic dermatitis: A cross-sectional multicentre study.

Background Filaggrin (FLG) gene encoding the protein filaggrin plays an important role in barrier function of the skin and its alteration is a predisposing factor for atopic dermatitis. FLG gene variants result in absent or decreased filaggrin protein. Worldwide, the prevalence of FLG variants ranges from 14 to 56%. FLG null variants are distinct in each population. Objectives To study the FLG gene polymorphisms in Indian children and attempt a genotype-phenotype correlation in atopic dermatitis. Methods This was a cross-sectional, multicentre study conducted on 75 Indian children. Demographic details, clinical features and identified FLG null variants were recorded. We performed a whole gene sequencing of the entire FLG coding region using next-generation sequencing technology. Results The prevalence of FLG null variants was 34.7%. A total of 20 different FLG loss of function variants in 26 children were documented. Sixteen (80%) variants were novel and four (20%) were previously reported in Asian and European populations. We found a statistically significant association between FLG variants with early age of onset of atopic dermatitis (P = 0.016) and elevated serum IgE levels (P = 0.051). There was no significant difference between atopic dermatitis phenotypes in children having one variant as compared to children harbouring two or more null variants. Limitation Small sample size. Conclusion Our study reports a unique set of FLG variants different from Asian and European populations, with these variants being significantly associated with an early age of onset of atopic dermatitis and elevated serum IgE levels.

Hypomelanosis of ito-whorled hyperpigmentation combination: a mirror image presentation.

We hereby report a 2-year-old boy who presented with a peculiar combination of hyperpigmented and hypopigmented skin lesions along the lines of Blaschko, sharply demarcated at the midline, both on the anterior and posterior trunk. Although combinations of hyperpigmented and hypopigmented skin lesions distributed along the lines of Blaschko have been described, the combination of skin lesion comprised hypomelanosis of Ito and whorled hypermelanosis distributed in the extremely unusual manner described in this patient has not been reported previously.

Correlation of the severity of atopic dermatitis with absolute eosinophil counts in peripheral blood and serum IgE levels.

BACKGROUND: Although a number of epidemiological studies, showing incidence and prevalence of atopic dermatitis, were available, scant attention has been paid to the correlation between the parameters of the disease like severity, absolute eosinophil count and IgE level, which has been known to be associated inconsistently. Hence this study was undertaken. METHODS: A total of 102 patients of atopic dermatitis, both children and adults, and 107 age matched controls were studied at the Pediatric Dermatology clinic, Institute of Child Health and department of Dermatology, AMRI-Apollo hospitals, Kolkata. RESULTS: The average age of onset of atopic dermatitis was observed to be 4.55 years. Both the average absolute eosinophil count and IgE levels in patients of atopic dermatitis were significantly higher than that of the controls. Each of these parameters showed significant correlation with severity of the disease and showed a nonhomogeneous distribution reflected by significant association with personal history of bronchial asthma and family history of atopy, when both parents were atopic. CONCLUSIONS: Our study shows that clinical activity of the disease as recorded by the "SCORAD" index can be used as an indicator of the hematological abnormalities as well as to some extent as a prognostic indicator. Family history of atopy correlates with the hematological abnormalities only if both parents are involved and bronchial asthma is the only associated atopic condition which correlates with the parameters of the disease .

Neonatal erythroderma: diagnostic and therapeutic challenges.

Erythroderma a life-threatening entity during the first one month, and many a time, a manifestation of genodermatosis, immune deficiency, psoriasis, metabolic diseases, and infections. Atopic dermatitis presenting as erythroderma is usually observed later, after this one-month period, and hence not a common differential for neonatal exfoliative dermatitis. Although a rare entity, there is a paucity of studies on this and in contrast to adults, some may manifest as cardinal signs of primary disease conditions.

Psoriasis in children: an insight.

Onset of psoriasis in childhood is quite common. Chronicity, inflammation and hyperproliferation are the cardinal features by which the condition establishes its uniqueness. Clearance of disease may be farfetched in most patients and relapse is frequent. Early recognition and management of psoriasis in children and adolescents is vital in therapy in children.

An uncontrolled open pilot study to assess the role of dietary eliminations in reducing the severity of atopic dermatitis in infants and children.

BACKGROUND: The severity of atopic dermatitis (AD) has been reported to be reduced by dietary eliminations in a subset of patients with AD. AIMS: To assess the reduction of the severity of atopic dermatitis in infants and children after eliminations of certain dietary items. MATERIALS AND METHODS: The study group comprised of 100 children with atopic dermatitis. Their severity of itching, surface area of involvement, and SCORAD index were measured. Patients who did not have any systemic disease or were not on systemic corticosteroids were included in the study. Selected patients were advised to strictly adhere to a diet excluding milk and milk products, all kinds of nuts and nut-containing foods, egg and egg-containing foods, seafish and prawns, brinjal and soyabean for a period of 3 weeks. Instead of these avoided items, the food items to be included freely to maintain proper nutrition were dal and dal products, rohu fish, chicken, and fruits. All the preintervention parameters were measured again after 3 weeks. RESULTS: There was a statistically significant reduction in severity scores after dietary elimination alone. CONCLUSION: Dietary elimination helped to alleviate symptoms and signs in a subset of infants and children with AD.

Epidemic of hand, foot and mouth disease in West Bengal, India in August, 2007: a multicentric study.

BACKGROUND: Hand, foot, and mouth disease (HFMD) is caused mostly by Coxsackievirus A16 (CA16) and enterovirus 71 (EV71). Epidemic of HFMD has occurred in India only once in Kerala in 2003. We report here a recent outbreak of HFMD in three districts of West Bengal, India. MATERIALS AND METHODS: A case detection system developed with 1) three private clinics in three districts; two at Howrah and one at Hooghly, 2) Pediatrics Department of two medical colleges in Kolkata, 3) 12 practioners of these three districts with 4) a central referral center at Department of Dermatology, NRS Medical College, Kolkata where all cases from this system were confirmed by a single observer. Pediatric Dermatology unit of the Institute of Child Health, Kolkata was another independent unit. RESULTS: A total of 38 cases of HFMD were reported till 08.10.07. Age group ranged from 12 months to 12 years (mean 40.76 months, SD 29.49). Males were slightly higher than females (M:F - 21:17). Disease was distributed mostly over buttocks, knees, hands, feet - both dorsum and palmar or the plantar surface and the oral mucosa. Highest severity noted over the buttocks and the knee. Healing time for skin lesions was 6-13 days (mean 9.13 days, SD 1.93). Oral lesions were found in 33 (86.8%) cases. CONCLUSION: This outbreak far away from the initial one confirmed regular outsourcing of the virus with possibilities of future epidemics. Also the fact that EV71 induced epidemic is on rise in this part of globe is alarming for India. We hope this early report will be of help for strategic planning for a better management of the disease and prevention of dreaded neurological complications in India.