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.

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.

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.

Atopic dermatitis in infants and children in India.

Atopic dermatitis (AD) is a chronic relapsing eczematous skin disease characterized by pruritus and inflammation and accompanied by cutaneous physiological dysfunction, with a majority of the patients having a personal or family history of "atopic diathesis." The term "atopic diathesis" refers to the presence of allergic rhinitis, bronchial asthma or AD. The universal occurrence of AD is no longer debated. However, published material about its natural history, etiopathogenesis, epidemiology, clinical patterns and management leave a lot to be known in the Indian scenario. In the present write-up, we will try to explore the wealth of knowledge about the disease available in our country and try to unfurl the complex interplay of different factors that are implicated for the development of this condition. The diagnosis of AD is based on a constellation of signs and symptoms. There is no laboratory "gold standard" for the diagnosis of AD. In a majority of the cases, the diagnosis is quite easy. Topical corticosteroids form the mainstay of topical treatment and, along with emollient, are able to control the condition in more than 80% of the cases. However, as use of long-term topical corticosteroid has the potential to produce local and systemic adverse effects, topical tacrolimus has come up as a useful molecule for the long-term control of the disease.

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.

Management of erythema nodosum leprosum by mycophenolate mofetil.

Mycophenolate mofetil has been tried in 20 cases of chronic relapsing erythema nodosum leprosum reaction where long use of systemic steroid produce complications or are contraindicated. Excellent results have been observed in all the cases to arrest the reaction followed for a period of six to eight months duration.

Condom leukoderma.

Contact dermatitis from natural latex of condom has been reported and is attributed to latex sensitivity. Chemical leukoderma from rubber condom is probably not reported. Here we present a case of chemical leukoderma in a 32-year-old male who developed depigmentation around the shaft of the penis in a circumferential pattern. Since the lesion was solitary and the site corresponded to the point of maximum contact of the condom, a diagnosis of contact leukoderma due to latex condom was thought of. Patch testing was done with mercaptobenzothiazole (MBT), dusting powder present in the condom and condom latex as such. The patient tested positive (3+) with mercaptobenzothiazole and the condom latex. On discontinuation of condom use and with UVB phototherapy, lesions repigmented in eight weeks.

Amulet string contact leukoderma and its differentiation from vitiligo.

A young adult factory worker presented with a linear depigmented vitiliginous patch on his right arm at the site where a silver amulet had been fixed with a nylon thread. He claimed that it was occupational in origin and demanded compensation, but patch testing with the nylon thread of the amulet and its extracted dyes proved that the contact leukoderma was due to the thread.

Injuries and granuloma caused by water slider.

Bite of waters slider (Ranatra) producing granuloma, numbness of the arm followed by neurotoxicity in the form of acute abdomen is being reported.

Damodar river pollution and health hazards.

Damodar river is one of the most polluted rivers in this country. Chemical pollution and bacteriological pollution, both are high--leading to rising trend in different industrial health hazards in the Damodar basin.