An Algorithmic Approach Towards Diagnosis of Patients with Hereditary Reticulate Pigmentary Disorders- A narrative review.

Hereditary reticulate pigmentary disorders include a group of genetic disorders with net-like pigmentation as their predominant presentation. Many of these hereditary reticulate pigmentary disorders have a wide array of cutaneous presentations with overlapping features. Furthermore, some of these disorders also have systemic manifestations. The overlapping features often add confusion and cause delay in the diagnosis. Based on the literature search, we propose an easy-to-follow concise diagnostic algorithm for the same. This would aid in ordering a definite genetic test. A thorough data search was done using data base PubMed using the following keywords. It included "'inherit*' OR 'genetic'" AND "reticulate AND pigment*"'. Thereafter, an individual disease search was done using keywords 'Dowling-Degos disease', 'Dyschromatosis Hereditaria Symmetrica', 'Acropigmentation of Kitamura', 'Dyschromatosis Universalis Hereditaria', 'Naegeli-Franceschetti-Jadasssohn syndrome', 'X-linked reticulate pigmentary disorder' and 'Dyskeratosis congenita'. The search included case reports, series, observational studies, narrative and systematic reviews, clinical trials. Acquired pigmentary disorders were excluded. A total of 1994 articles were retrieved. Finally, 625 articles were included for the review. The articles were narrative review articles (40), case series (23), observational studies (44), and case reports (518). An easy-to-follow clinical diagnostic algorithm based on age of onset, distribution, and other parameters would definitely aid in reaching a provisional diagnosis. And further this approach will help in the genetic workup of a case of hereditary reticulate pigmentary disorder.

The effects of systemic immunomodulatory treatments on COVID-19 outcomes in patients with atopic dermatitis: Results from the global SECURE-AD registry.

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.

Comparison of efficacy of diode laser in patients of facial hirsutism with normal dehydroepiandrosterone sulfate and dihydrotestosterone versus deranged dehydroepiandrosterone sulfate and dihydrotestosterone level-A longitudinal study.

Hirsutism is defined as an excessive terminal hair growth in female over male pattern distribution areas. Traditional methods of hair removal have been replaced by lasers. The aim of the study was to observe the effect of diode laser in hirsutism patient with normal and deranged dehydroepiandrosterone sulfate (DHEAS) and dihydrotestosterone (DHT) level. Fifty patients of facial hirsutism with skin type IV and V were enrolled. Eighteen patients with deranged DHEAS and DHT level were allocated in group A whereas 16 patients with normal level were allocated in group B. Three sittings of diode laser was done in all subjects with varying session within a period of 6 months. At first follow-up visit, the percentage of hair reduction was fair in 62.5% of patients in group B and 16.7% of patients in group A, whereas at second follow-up visit, it was good in 56.3% of patients in group B and 11.1% of patients in group A. Increase in vellus hair and reduction in percentage of terminal hair were more significant in group B. Hair free interval and satisfaction level was significantly higher in group B. Diode laser is effective mode of treatment in hirsutism patient irrespective of hormone level. But, the effect is observed to be more efficacious in patients with normal DHEAS and DHT level.

Tofacitinib in paediatric dermatoses: a narrative review.

Tofacitinib has revolutionized the treatment of numerous dermatological conditions in different age groups. However, evidence for its effectiveness, safety and tolerability in the paediatric population is limited. We performed a literature search, which showed that oral tofacitinib is a reliable option in refractory juvenile dermatomyositis, severe alopecia areata, atopic dermatitis and psoriasis. Topical tofacitinib is an effective option in vitiligo and halo naevus. The risk-benefit ratio should be assessed prior to consideration of this molecule. In this narrative review, we have attempted to present a summary of the evidence of using tofacitinib (oral and topical) in paediatric dermatoses.

In Silico Identification of Potential Insect Peptides against Biofilm-Producing Staphylococcus aureus.

Biofilm-producing Staphylococcus aureus (SA) strains are frequently found in medical environments, from surgical/ wound sites, medical devices. These biofilms reduce the efficacy of applied antibiotics during the treatment of several infections, such as cystic fibrosis, endocarditis, or urinary tract infections. Thus, the development of potential therapeutic agents to destroy the extra protective biofilm layers or to inhibit the biofilm-producing enzymes is urgently needed. Advanced and cost-effective bioinformatics tools are advantageous in locating and speeding up the selection of antibiofilm candidates. Based on the potential drug characteristics, we have selected one-hundred thirty-three antibacterial peptides derived from insects to assess for their antibiofilm potency via molecular docking against five putative biofilm formation and regulated target enzymes: the staphylococcal accessory regulator A or SarA (PDB ID: 2FRH), 4,4'-diapophytoene synthase or CrtM (PDB ID: 2ZCQ), clumping factor A or ClfA (PDB ID: 1N67) and serine-aspartate repeat protein C or SdrC (PDB ID: 6LXH) and sortase A or SrtA (PDB ID: 1T2W) of SA bacterium. In this study, molecular docking was performed using HPEPDOCK and HDOCK servers, and molecular interactions were examined using BIOVIA Discovery Studio Visualizer-2019. The docking score (kcal/mol) range of five promising antibiofilm peptides against five targets was recorded as follows: diptericin A (-215.52 to -303.31), defensin (-201.11 to -301.92), imcroporin (-212.08 to -287.64), mucroporin (-228.72 to -286.76), apidaecin II (-203.90 to -280.20). Among these five, imcroporin and mucroporin were 13 % each, while defensin contained only 1 % of positive net charged residues (Arg+Lys) projected through ProtParam and NetWheels tools. Similarly, imcroporin, mucroporin and apidaecin II were 50 %, while defensin carried 21.05 % of hydrophobic residues predicted by the tool PEPTIDE. 2.0. Most of the peptides exhibited potential characteristics to inhibit S. aureus-biofilm formation via disrupting the cell membrane and cytoplasmic integrity. In summary, the proposed hypothesis can be considered a cost-effective platform for selecting the most promising bioactive drug candidates within a limited timeframe with a greater chance of success in experimental and clinical studies.

Combination of 1064-nm Q-switched neodymium-doped yttrium-aluminum-garnet laser with Modified Jessner's peel for the treatment of Nevus of Ota: A case series of seven patients.

1064-nm Q-switched Nd: YAG (neodymium-doped yttrium-aluminium-garnet) laser is widely used for the treatment of Nevus of Ota but multiple treatments are necessary for clinical improvement. Superficial chemical peeling using Modified Jessner's solution has been used for the treatment of facial pigmentation but repetitive chemical peeling can cause irritation and post-inflammatory hyperpigmentation. In this series, we evaluated seven patients who were treated with a combination of 1064-nm Q-switched Nd: YAG laser and Modified Jessner's peel for eight sessions with 85.7% patients showing more than 50% reduction in pigmentation. The added therapeutic benefit of the combination helped to achieve a significant reduction in pigmentation faster with a lesser number of sessions and reduced cost.

Rising trends of symmetrical drug related intertriginous and flexural exanthem due to Itraconazole in patients with superficial dermatophytosis: A case series of 12 patients from eastern part of India.

SDRIFE (symmetrical drug related intertriginous and flexural exanthem) is a benign self-limiting adverse drug reaction (ADR) primarily affecting the intertriginous, gluteal and flexural regions symmetrically in the absence of systemic involvement. It is considered to be a variant of baboon syndrome. Pathogenesis is poorly understood but is thought to be result of a delayed hypersensitivity response after exposure to the drug. Commonly implicated drugs are beta-lactam antibiotics. With the rising incidence of dermatophytosis in the Indian subcontinent, the use of oral anti fungals has become rampant. SDRIFE due to itraconazole has been rarely reported in literature. We hereby present a series of 12 patients presenting with SDRIFE due to itraconazole, which is by far the largest reported in existing literature. This case series highlights the importance of identifying these cutaneous adverse drug reactions in a setting where itraconazole is commonly being used.

Systemic Sclerosis: A Clinical Analysis of Foot Findings in 52 Cases.

INTRODUCTION: The diagnosis of systemic sclerosis is mostly clinical and is identified by the various cutaneous changes. The cutaneous changes in systemic sclerosis are already well established and supported by a large number of studies and textbooks. Very few literatures describe foot findings in patients with systemic sclerosis. This study was aimed at identifying the various changes in plantar aspect of foot of patients with systemic sclerosis. METHODS: All patients diagnosed with systemic sclerosis were recruited. Apart from clinical examination, X - ray of both feet was done in all the patients. RESULTS: A total of 52 patients were recruited in the study period. Most of the patients (86.54%) were females and the mean age at presentation was 34.19 years. A history of Raynaud's phenomenon was present in 47(90.38%) patients. The foot findings were digital pitted scars in 44(84.61%) patients, macular hyperpigmentation in 40(76.92%) patients and corns in weight bearing areas were found in 40 (76.92%) cases. CONCLUSIONS: In our study corn was a common finding in the foot of patients of systemic sclerosis which has not been routinely documented.

RASopathies: Evolving Concepts in Pathogenetics, Clinical Features, and Management.

RASopathies refers to the group of disorders which are caused by a mutation in various genes of the RAS/MAPK (RAT sarcoma virus/Mitogen activated protein kinase) pathway. It includes many genes with varied functions, which are responsible for cell cycle regulation. As the mutation in one gene affects the entire pathway, there are many overlapping features among the various syndromes which are included under an umbrella term "RASopathies." However, neuroectodermal involvement is a unifying feature among these syndromes, which are caused by germline mutations affecting genes along this pathway. Recently, many other RASopathies have been described to involve blood vessels, lymphatics, and immune system. Also, many cutaneous mosaic disorders have been found to have mutations in the concerned pathway. The purpose of this article is to briefly review the pathogenesis of RASopathies with cutaneous manifestations, and summarise the features that can be helpful as diagnostic clues to dermatologists. As we understand more about the pathogenesis of the pathway at the cellular level, the research on genotype-phenotype correlation and therapeutic options broadens. Targeted therapy is in the clinical and preclinical trial phase, which may brighten the future of many patients.

Patch Testing in Toilet Seat Dermatitis: A Case Series of Six Patients with Paediatric Atopic Dermatitis.

Background: Toilet seat dermatitis (TSD) is a localized form of dermatitis that commonly occurs in the buttocks and upper posterior aspect of the thigh in school-going children. Previously, it was mostly seen due to allergic contact dermatitis to wood, but recently, many other allergens in toilet seats along with the possibility of irritant contact dermatitis (ICD) to detergents were reported. Materials and Methods: Six cases diagnosed clinically as atopic dermatitis per Hanifin and Rajka criteria presented with posterior thigh dermatitis. Patch testing using the Indian Standard Series, toilet seat scrapings, and detergents was conducted. Results: Patch testing showed negative results at 48, 96, and 168 hours, including lesional site testing. Symptomatic management, including topical steroids and later tacrolimus, was administered, coupled with counselling on toilet seat cover use, with a follow-up after four weeks. Lesions showed resolution and reduced itching. Limitation: This study's limitation lies in its small sample size from a single geographic area. Conclusion: Lesion resolution through topical treatment and toilet seat avoidance, support a diagnosis of contact dermatitis; however, the role of atopy remains crucial, suggesting a complex interplay. Larger studies are needed for a comprehensive understanding of this dermatological condition.

Algorithmic approach toward diagnosis of patients with congenital photosensitivity disorders and review of literature.

The congenital photosensitivity disorders present as cutaneous signs and symptoms secondary to photosensitivity, extracutaneous manifestations, and a predisposition to malignancy. Diagnosis of these conditions mainly depend on clinical findings as the molecular analysis is not always feasible. A review of all the related articles collected after a thorough literature search using keywords, "congenital AND photosensitivity NOT acquired" and the individual diseases was done. A total of 264 articles were included in the review. An algorithm for diagnosis of the different congenital photosensitivity disorders based on the various clinical presentations has been proposed. An early suspicion and diagnosis of the different congenital photosensitivity disorders is the cornerstone behind prompt institution of prevention and treatment, and decreasing the associated morbidity.

Vascular Malformations.

Vascular malformations are intricate anomalies of the circulatory system, presenting a diverse array of clinical manifestations, and posing significant challenges in diagnosis and treatment. The pathogenesis of vascular malformations is explored through the lens of genetic and molecular mechanisms, shedding light on the pivotal role of somatic mutations and dysregulated signaling pathways. Clinical presentations of vascular malformations are widely variable, ranging from cosmetic concerns to life-threatening complications. The utility of imaging techniques, such as magnetic resonance imaging (MRI), computed tomography (CT), and angiography, are discussed in detail, emphasizing their role in precise delineation and characterization. Therapeutic strategies for vascular malformations are multifaceted, considering factors such as lesion size, location, potential complications, and patient-specific factors. Traditional interventions, including surgical excision and embolization, are appraised alongside emerging approaches like targeted molecular therapies and minimally invasive procedures. The manuscript underscores the need for an individualized treatment approach, optimizing outcomes while minimizing risks and complications. In summation, this manuscript offers a comprehensive analysis of vascular malformations, encompassing their underlying pathogenesis, clinical nuances, diagnostic methods, and therapeutic considerations. By synthesizing current knowledge and highlighting gaps in understanding, this review serves as a valuable resource for clinicians, researchers, and medical practitioners, fostering an enhanced comprehension of vascular malformations and paving the way for improved patient care and innovative research endeavors.

Prescribing Patterns in the Treatment of Topical Steroid Damaged Face Patients: A Cross-Sectional, Survey-Based Observational Study among Practicing Dermatologists in an Eastern State of India.

Introduction: Topical steroid damaged face (TSDF) is a common occurrence in the current scenario, where darker - skinned people believe having lighter-colored skin improves their prospects in all aspects of life. This has led to the abuse of topical corticosteroids with unrealistic expectations driven by ignorance of the adverse effects, particularly in the Indian population. As TSDF is virtually an epidemic in India and there is no treatment protocol for dermatologists, we conducted this study to find out what dermatologists prescribe and, in turn, serve as a foundation to develop guidelines in the future. Materials and Methods: Forty dermatologists from Odisha, India, were selected to complete the questionnaire, containing 18 questions about diagnosis, treatment and necessary referral for ocular complications. Results: All participants were frequently diagnosing and treating TSDF. A total of 92.5% of the participants preferred initiating systemic therapy with isotretinoin or doxycycline as the drug of choice. Clindamycin and tacrolimus were preferred by 55% and 50% of participants, respectively, as the topical drug of choice. A total of 62.5% of the participants advised physical sunscreens and mild cleansers to be used with water at room temperature to wash their face. To reduce facial erythema, brimonidine was advised by 45% of participants. Conclusion: Standardized guidelines for managing TSDF are the need of the hour.

Trichoscopic Features of Scalp Discoid Lupus Erythematosus versus Lichen Planopilaris: A Systematic Review.

Introduction: Lichen planopilaris (LPP) and discoid lupus erythematosus (DLE) are primary scarring alopecias that pose diagnostic challenges clinically, where trichoscopy features may provide benefit in delineating these two cicatricial alopecia, and also helps in assessing the evolution and therapeutic response. To date, there are few reviews on dermoscopic findings in differentiating these two alopecias. Methods: A systematic literature review was conducted using the PubMed and Google Scholar databases. The search terms included for scalp DLE were 'lupus' OR 'discoid lupus' OR "scalp lupus" and for scalp LPP were "lichen planopilaris" OR "scalp follicular lichen planus" OR "lichen planus follicularis" and were combined with "dermoscopy" OR "dermatoscopy" OR "videodermoscopy" OR "video dermatoscopy" OR "trichoscopy". The differences in the prevalence of dermoscopic features in scalp DLE and LPP were calculated using the Chi-square test. Results: Of 52 articles, 36 (17 LPP, 19 DLE) were eligible for quantitative analysis. We found predominant peripilar tubular casts and perifollicular erythema with the presence of arborizing vessels in the vicinity of these changes, indicating early LPP. In contrast, follicular red dots, speckled brown pigmentation, and hair diameter variability indicated active DLE. Shiny white areas were common in both the groups in late stages. The target pattern of distribution of blue-grey dots, milky red areas, and irregular white fibrotic dots were seen in LPP, and pink-white background, follicular plugs, perifollicular and interfollicular scale, rosettes, chrysalides, and red spider on yellow dots were detected in DLE. Features such as yellow dots and blue-grey structureless areas were nonspecific and did not have a major role in differentiating DLE from LPP. Conclusion: This article provides a comprehensive review of the literature and delineates the trichoscopic differences and peculiarities of scalp DLE and LPP, including the correlation of dermoscopic features with histopathological findings.

Methotrexate monotherapy versus methotrexate and apremilast combination therapy in the treatment of palmoplantar psoriasis: A prospective, randomised, assessor-blinded, comparative study.

BACKGROUND: Palmoplantar psoriasis is a chronic debilitating condition which significantly impairs quality of life. OBJECTIVES: To assess the efficacy and safety of the combination of apremilast and methotrexate compared with methotrexate monotherapy in the treatment of palmoplantar psoriasis. Also, to study the impact on treatment on the Dermatology Life Quality Index and Palmoplantar Quality of Life Index. METHODS: A total of 64 patients were randomised to two groups in a 1:1 ratio - Group A received both methotrexate and apremilast in combination, while Group B received only methotrexate, for 16 weeks. The primary endpoints were the mean score of Modified Palmoplantar Psoriasis Area and Severity Index at week 16, the proportion of patients achieving modified palmoplantar psoriasis area severity index-75 and/or Palmoplantar Psoriasis Physician Global Assessment score 0/1 at week 16. RESULTS: A significantly higher proportion of patients in Group A achieved Modified Palmoplantar Psoriasis Area and Severity Index-75 at week 16 (43% in Group A vs 30% in Group B). The Modified Palmoplantar Psoriasis Area and Severity Index score was significantly lower in the combination group at week 16 (4.03 ± 2.05 in Group A and 5.89 ± 2.31 in Group B, P-value = 0.002). About 80% of patients in the combination group with baseline Palmoplantar Psoriasis Physician Global Assessment ≥3 achieved Palmoplantar Psoriasis Physician Global Assessment 0/1 compared to 60% in Group B. The combination group showed a significantly higher reduction in Dermatology Life Quality Index and Palmoplantar Quality of Life Index scores compared to the methotrexate alone group (P-value = 0.025). No notable adverse events were observed. LIMITATION: The limitations of the study were single blinding, small sample size and a lack of longer follow up to assess the rate of relapse. We did not account for attrition during sample size calculation. Also, due to the paucity of data regarding the use of apremilast in palmoplantar psoriasis, definitive comparisons could not be made with previous studies. CONCLUSION: The combination of apremilast and methotrexate has superior efficacy and a similar safety profile as compared to methotrexate monotherapy for the treatment of moderate to severe palmoplantar psoriasis.

Efficacy and Safety of Autologous Serum Therapy in Chronic Spontaneous Urticaria in the Pediatric Population: A Prospective Pilot Study.

Background: Chronic spontaneous urticaria (CSU) in children is mostly spontaneous in onset (57%). Treatment comprises long-term antihistaminic therapy without need for elaborate investigations. A subset of such patients don't respond to conventional treatment and novel therapies to help reduce pill burden is the need of the hour. Objectives: To determine the efficacy and safety of autologous serum therapy (AST) in pediatric patients with chronic spontaneous urticaria. Materials and Methods: All pediatric patients, aged between 6-16 years, attended to our OPD from March 2019 to March 2020 were recruited. Clinico-demographic data and baseline investigations of all patients were performed. Two-weekly AST therapy was given for 8 visits with levocetrizine tablet 5mg on an on-demand basis. Urticaria activity score (UAS) sheet was provided to record and return every 2 weeks. Statistical analysis was done using the IBM SPSS 26 software package. Results: Autologous serum skin test (ASST) was positive in 63% patients. Both the ASST positive and ASST negative group showed significant reduction in UAS7 score at week 14 compared to baseline. The reduction in mean UAS7 score was associated with a decreased pill burden and positive response in the patient and physician global assessment scale. No statistically significant difference between the two groups in terms of mean UAS7 reduction was found. Conclusion: This study has explored the efficacy and safety of autologous serum therapy in the pediatric CSU patients. Both ASST positive and ASST negative group respond to AST therapy.

Symplastic Glomus Tumor: Pathologist's Challenge and Physician's Dilemma: A Case Report with Review.

Glomus tumor is a distinct mesenchymal tumor of specialized perivascular smooth cell origin (pericyte), reminiscent of normal glomus body located in the fingers. It accounts for <2% of soft tissue tumors. Recently WHO categorized few unusual variants, of which symplastic glomus tumor is one. Histologic picture of the latter shows marked nuclear atypia in the absence of any other malignant features. They are exceedingly rare, hence their biological behavior, prognosis and treatment are little understood. But recognizing this entity would prevent misdiagnosis of malignancy. We report such a case in a 39-year-old female presented with excruciating pain in the left thumb.