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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.

Objectives: Chronic spontaneous urticaria (CSU) is defined as short lived (< 24h) wheals occurring spontaneously without any triggering factors, daily or almost daily for at least 6 weeks. Though CSU is mainly a Type 1 hypersensitivity reaction, earlier studies suggested contact allergy may have some role to play an important role in the etiopathogenesis of CSU. The objective of the study is to find out the relevance of patch test in the etiopathogenesis of and its correlation with serum IgE level. Materials and Methods: Thirty-one patients of CSU were thoroughly evaluated for clinical and laboratory parameters. We conducted patch testing with Indian Standard Series (ISS) in all of them and tried to find out the relevance of every positive reaction. Relevant positive cases were asked to avoid exposure for 2 months and were maintained only in breakthrough antihistamines. Furthermore, correlation of positive patch test reaction with high IgE and eosinophil count were studied. Results: Seventeen (55%) cases came positive for patch test. Potassium dichromate, lanolin, benzocaine, and fragrance mix were the most common offenders. Patients with very high IgE count had patch test positivity with multiple allergens. Seven of the fifteen patients who had relevant positive patch results could be followed up to remission; three were lost to follow-up. Conclusion: Patch test with ISS may give important clue to in a subset of patients of CSU and thus may be considered an important part of routine investigations of CSU.

Atopic dermatitis (AD) is a chronic, relapsing inflammatory skin disorder affecting 15-20% of children and 1-10% of adults.

There is a dearth of data regarding the safety and timing of the severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) vaccination of patients on immunosuppressive or immunomodulatory therapies. However, data from other vaccine trials may be extrapolated to get an idea regarding the recommendation of SARS-COV-2 vaccines. All the novel SARS-COV-2 vaccines are non-live, thus ensuring the safety of the vaccines. However, the vaccines may not be able to generate an equipotent immunogenic response in patients receiving immunotherapeutics, in comparison to those who are not. We have attempted to put forward certain statements, with respect to SARS-COV-2 vaccination of patients who are on treatment for different dermatological conditions. However, the risk-benefit ratio must be discussed between the patient and the physician, and the final call should be individualized.

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.

BACKGROUND: Limited data are available on the effects of systemic immunomodulatory treatments on COVID-19 outcomes in patients with atopic dermatitis (AD).OBJECTIVE: To investigate COVID-19 outcomes in patients with AD treated with or without systemic immunomodulatory treatments, using a global registry platform.METHODS: Clinicians were encouraged to report cases of COVID-19 in their patients with AD in the Surveillance Epidemiology of Coronavirus Under Research Exclusion for Atopic Dermatitis (SECURE-AD) registry. Data entered from 1 April 2020 to 31 October 2021 were analysed using multivariable logistic regression. The primary outcome was hospitalization from COVID-19, according to AD treatment groups.RESULTS: 442 AD patients (mean age 35.9 years, 51.8% male) from 27 countries with strongly suspected or confirmed COVID-19 were included in analyses. 428 (96.8%) patients were treated with a single systemic therapy (n = 297 [67.2%]) or topical therapy only (n = 131 [29.6%]). Most patients treated with systemic therapies received dupilumab (n = 216). Fourteen patients (3.2%) received a combination of systemic therapies. Twenty-six patients (5.9%) were hospitalized. No deaths were reported. Patients treated with topical treatments had significantly higher odds of hospitalization, compared with those treated with dupilumab monotherapy (odds ratio (OR) 4.65 [95%CI 1.71-14.78]), including after adjustment for confounding variables (adjusted OR (aOR) 4.99 [95%CI 1.4-20.84]). Combination systemic therapy which did not include systemic corticosteroids was associated with increased odds of hospitalization, compared with single agent non-steroidal immunosuppressive systemic treatment (OR 8.09 [95%CI 0.4-59.96], aOR 37.57 [95%CI 1.05-871.11]). Hospitalization was most likely in patients treated with combination systemic therapy which included systemic corticosteroids (OR 40.43 [95%CI 8.16-207.49], aOR 45.75 [95%CI 4.54-616.22]).CONCLUSIONS: Overall, the risk of COVID-19 complications appears low in patients with AD, even when treated with systemic immunomodulatory agents. Dupilumab monotherapy was associated with lower hospitalization than other therapies. Combination systemic treatment, particularly combinations including systemic corticosteroids, was associated with the highest risk of severe COVID-19.

Cyclosporine (CsA) is a calcineurin inhibitor that acts selectively on T cells. It has been used in dermatology since 1997 for its US Food and Drug Administration indication of psoriasis and off-label for various other inflammatory skin conditions, including atopic dermatitis, alopecia areata, urticaria, lichen planus and many others in pediatric, adults as well as in pregnant women. However, clinicians' preferences for management differ, which may have a bearing on the treatment selection. Hence, the purpose of this review is to outline the role of CsA in various skin conditions through consensus statement from six national experts in the field of dermatology.

Atopic dermatitis (AD) is a chronic, relapsing inflammatory skin disease. Hanifin and Rajka's criteria is the most common diagnostic criteria used for the clinical diagnosis of this condition. However, many find that it is too exhaustive to be used in routine practice, and the specificity of many of the minor criteria poses challenges, particularly from Asian countries with type III, IV, and V skin.The aim of the study is to evaluate the effectiveness of the minor features of the Hanifin and Rajka criteria for AD in comparison to the UK working party's diagnostic criteria in pediatric populations of India.A hospital-based cross-sectional study of 100 patients in the pediatric age group (3 months-12 years) with AD was conducted based on history, clinical, and laboratory evaluation. An age-matched control group of 100 pediatric patients who did not have a personal or family history of atopic dermatitis was included after obtaining informed consent to find out the prevalence of minor criteria among the control group.Mean of the number of minor clinical criteria found positive in our study population in the infantile and toddler (below 2 years) and childhood groups (2-12 years) was (4.72 ± 1.75) and (5.67 ± 1.78), respectively. Early-onset of disease was the most consistent feature among the minor criteria found in 83% of patients, followed by xerosis (71%), hyperlinearity of palm (56%), pityriasis alba (54%), Denny Morgan fold (52%), elevated serum IgE (47%), perifollicular accentuation (37%), and tendency toward cutaneous infections (37%).We found that though some of the minor criteria are highly sensitive and specific to the diagnosis of AD (xerosis, ichthyosis, palmar hyperlinearity, tendency of cutaneous infections, Dennie-Morgan infraorbital fold, pityriasis alba, and perifollicular accentuation), some other criteria were either very rare or nonspecific for AD. We suggest that many of the minor criteria of Hanifin and Rajka may not have much significance for Indian patients and a multicentric nationwide study with a larger patient pool is required to create a trimmed and improved version of Hanifin and Rajka criteria.

Integration of automated cardiac analysis techniques with the personal healthcare devices can aid in the timely diagnosis of myocardial infarction (MI) to reduce the risk of mortality. The use of electrocardiogram (ECG) along with advanced signal processing tools limits the application of the available techniques for such portable devices having limited processing ability. Here we propose a novel MI screening technique based on photoplethysmographic (PPG) data, which is the most widely monitored signal in everyday health monitoring. Two simple statistical features ideally reflecting the MI-induced variability in the PPG data are identified. To combat the noise sensitivity of the PPG acquisition process, the same statistical parameters are employed for eliminating the noise corrupted data segments. Classification of MI data is then performed using a simple threshold-based binary classification approach. The technique is evaluated with PPG records collected from 62 hospitalized MI patients and 65 healthy subjects. The robustness of the algorithm is cross-validated with both MI and non-MI records collected from the Multiparameter Intelligent Monitoring in Intensive Care (MIMIC) II database. The algorithm achieves an average detection accuracy of 96.25% with sensitivity and specificity of 96.36% and 96.15%, respectively. The promising results establish the utility of PPG as an alternate MI diagnosing tool. Apart from using the less obtrusive PPG signal, the proposed technique relies on simple feature extraction and classification tools, thus making it a promising alternative to the ECG based techniques for implementation on the personal healthcare devices.

Systemic mastocytosis with associated clonal hematologic non-mast cell lineage disease (SM-AHN) represents a specific subtype of mastocytosis and is extremely rare in children. We describe a 4-year-old child with systemic mastocytosis associated with Hodgkin's lymphoma. The child had cutaneous mastocytosis and lymphadenopathy without other clinical features of SM, which was diagnosed only by bone marrow examination.

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.

Summary Patients with atopic dermatitis (AD) have an increased risk of bacterial skin infections, which cause significant morbidity and, if untreated, may become systemic. Staphylococcus aureus colonizes the skin of most patients with AD and is the most common organism to cause infections. Overt bacterial infection is easily recognized by the appearance of weeping lesions, honey‐coloured crusts and pustules. However, the wide variability in clinical presentation of bacterial infection in AD and the inherent features of AD – cutaneous erythema and warmth, oozing associated with oedema, and regional lymphadenopathy – overlap with those of infection, making clinical diagnosis challenging. Furthermore, some features may be masked because of anatomical site‐ and skin‐type‐specific features, and the high frequency of S. aureus colonization in AD makes positive skin swab culture of suspected infection unreliable as a diagnostic tool. The host mechanisms and microbial virulence factors that underlie S. aureus colonization and infection in AD are incompletely understood. The aim of this article is to present the latest evidence from animal and human studies, including recent microbiome research, to define the clinical features of bacterial infections in AD, and to summarize our current understanding of the host and bacterial factors that influence microbial colonization and virulence.

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.

The progression of allergic diseases with the development of atopic dermatitis and food allergy in infancy and subsequent asthma and allergic rhinitis in the later childhood is known as 'atopic march'. There have been many arguments in favour of and against this concept. This article reviews the latest epidemiology, immunological mechanisms and translational implications in clinical practice and research, which is relevant to the dermatologists. The role of skin as a site of initiation and the potential for interventions on skin that may prevent subsequent allergic diseases is also highlighted.

Summary Background Guidelines discourage the use of systemic corticosteroids for atopic dermatitis (AD), but their use remains widespread. Objectives To reach consensus among an international group of AD experts on the use of systemic corticosteroids for AD. Methods A survey consisting of statements accompanied by visual analogue scales ranging from ‘strongly disagree’ to ‘neutral’ to ‘strongly agree’ was distributed to the International Eczema Council (IEC). Consensus was reached in agreement on a statement if, What's already known about this topic? Despite recommendations against their use in practice guidelines, systemic corticosteroids are commonly used for atopic dermatitis (AD). What does this study add? The International Eczema Council reached consensus on circumstances in which systemic corticosteroids can be used for AD, including a lack of other treatment options, as a bridge to other systemic therapies or phototherapy, during acute flares in need of immediate relief, in anticipation of a major life event, or in the most severe cases.Clinicians should limit the use of systemic corticosteroids for severe AD to those circumstances. https://doi.org/10.1111/bjd.16385 available online https://goo.gl/Uqv3dl