Mast-cell expressed membrane protein-1 is expressed in classical monocytes and alveolar macrophages in idiopathic pulmonary fibrosis and regulates cell chemotaxis, adhesion, and migration in a TGFß-dependent manner.

Mast-cell expressed membrane protein-1 (MCEMP1) is higher in patients with idiopathic pulmonary fibrosis (IPF) with an increased risk of death. Here we aimed to establish the mechanistic role of MCEMP1 in pulmonary fibrosis. We identified increased MCEMP1 expression in classical monocytes and alveolar macrophages in IPF compared with controls. MCEMP1 is upregulated by transforming growth factor beta (TGFß) at the mRNA and protein levels in monocytic leukemia THP-1 cells. TGFß-mediated MCEMP1 upregulation results from the cooperation of SMAD3 and SP1 via concomitant binding to SMAD3/SP1 cis-regulatory elements within the MCEMP1 promoter. We also found that MCEMP1 regulates TGFß-mediated monocyte chemotaxis, adhesion, and migration. Our results suggest that MCEMP1 may regulate the migration and transition of monocytes to monocyte-derived alveolar macrophages during pulmonary fibrosis development and progression.NEW & NOTEWORTHY MCEMP1 is highly expressed in circulating classical monocytes and alveolar macrophages in IPF, is regulated by TGFß, and participates in the chemotaxis, adhesion, and migration of circulating monocytes by modulating the effect of TGFß in RHO activity.

Risk of 30-Day All-Cause Readmission in Interstitial Lung Disease Patients after COVID-19: National-Level Data.

Rationale: Hospital readmission within 30 days poses challenges for healthcare providers, policymakers, and patients because of its impact on care quality, costs, and outcomes. Patients with interstitial lung disease (ILD) are particularly affected by readmission, which is associated with increased morbidity and mortality and reduced quality of life. Because small sample sizes have hindered previous studies, this study seeks to address this gap in knowledge by examining a large-scale dataset. Objective: To determine the rate and probability of 30-day all-cause readmission and secondary outcomes in patients with coronavirus disease (COVID-19) or ILD admitted to the hospital. Methods: This study is a nested cohort study that used the PearlDiver patient records database. Adult patients (age ⩾18 yr) who were admitted to hospitals in 28 states in the United States with COVID-19 or ILD diagnoses were included. We defined and analyzed two separate cohorts in this study. The first cohort consisted of patients with COVID-19 and was later divided into two groups with or without a history of ILD. The second cohort consisted of patients with ILD and was later divided into groups with COVID-19 or with a non-COVID-19 pneumonia diagnosis at admission. We also studied two other subcohorts of patients with and without idiopathic pulmonary fibrosis within the second cohort. Propensity score matching was employed to match confounders between groups. The Kaplan-Meier log rank test was applied to compare the probabilities of outcomes. Results: We assessed the data of 2,286,775 patients with COVID-19 and 118,892 patients with ILD. We found that patients with COVID-19 with preexisting ILD had an odds ratio of 1.6 for 30-day all-cause readmission. Similarly, an odds ratio of 2.42 in readmission rates was observed among hospitalized individuals with ILD who contracted COVID-19 compared with those who were hospitalized for non-COVID-19 pneumonia. Our study also found a significantly higher probability of intensive care admission among patients in both cohorts. Conclusions: Patients with ILD face heightened rates of hospital readmissions, particularly when ILD is combined with COVID-19, resulting in adverse outcomes such as decreased quality of life and increased healthcare expenses. It is imperative to prioritize preventive measures against COVID-19 and establish effective postdischarge care strategies for patients with ILD.

Mast-Cell Expressed Membrane Protein-1 (MCEMP1) is expressed in classical monocytes and alveolar macrophages in Idiopathic Pulmonary Fibrosis and regulates cell chemotaxis, adhesion, and migration in a TGFß dependent manner.

Background: Mast-Cell Expressed Membrane Protein-1 (MCEMP1) is higher in Idiopathic Pulmonary Fibrosis (IPF) patients with increased risk of death and poor outcomes. Here we seek to establish the mechanistic role of MCEMP1 in pulmonary fibrosis. Methods: MCEMP1 expression was analyzed by single-cell RNA sequencing, immunofluorescence in Peripheral Blood Mononuclear Cells (PBMC) as well as in lung tissues from IPF patients and controls. Chromatin Immunoprecipitation (ChiP) and Proximity Ligation Assay (PLA) were used to study the transcriptional regulation of MCEMP1 . Transient RNA interference and lentivirus transduction were used to knockdown and knock-in MCEMP1 in THP-1 cells to study chemotaxis, adhesion, and migration. Bulk RNA sequencing was used to identify the mechanisms by which MCEMP1 participates in monocyte function. Active RHO pull-down assay was used to validate bulk RNA sequencing results. Results: We identified increased MCEMP1 expression in classical monocytes and alveolar macrophages in IPF compared to controls. MCEMP1 was upregulated by TGFß at the mRNA and protein levels in THP-1. TGFß-mediated MCEMP1 upregulation results from the cooperation of SMAD3 and SP1 via concomitant binding to SMAD3/SP1 cis -regulatory elements within the MCEMP1 promoter. In terms of its function, we found that MCEMP1 regulates TGFß-mediated monocyte chemotaxis, adhesion, and migration. 400 differentially expressed genes were found to increase after TGFß stimulation of THP-1, further increased in MCEMP1 knock-in cells treated with TGFß and decreased in MCEMP1 knockdown cells treated with TGFß. GO annotation analysis of these genes showed enrichment for positive regulation of RHO GTPase activity and signal transduction. While TGFß enhanced RHO GTPase activity in THP-1 cells, this effect was attenuated following MCEMP1 knockdown. Conclusion: MCEMP1 is highly expressed in circulating classical monocytes and alveolar macrophages in IPF. MCEMP1 is regulated by TGFß and participates in the chemotaxis, adhesion, and migration of circulating monocytes by modulating the effect of TGFß in RHO activity. Our results suggest that MCEMP1 may regulate the migration and transition of monocytes to monocyte-derived alveolar macrophages during pulmonary fibrosis development and progression.

Sarcoidosis-associated pulmonary fibrosis: joining the dots.

Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology. A minority of patients with sarcoidosis develop sarcoidosis-associated pulmonary fibrosis (SAPF), which may become progressive. Genetic profiles differ between patients with progressive and self-limiting disease. The mechanisms of fibrosis in SAPF are not fully understood, but SAPF is likely a distinct clinicopathological entity, rather than a continuum of acute inflammatory sarcoidosis. Risk factors for the development of SAPF have been identified; however, at present, it is not possible to make a robust prediction of risk for an individual patient. The bulk of fibrotic abnormalities in SAPF are located in the upper and middle zones of the lungs. A greater extent of SAPF on imaging is associated with a worse prognosis. Patients with SAPF are typically treated with corticosteroids, second-line agents such as methotrexate or azathioprine, or third-line agents such as tumour necrosis factor inhibitors. The antifibrotic drug nintedanib is an approved treatment for slowing the decline in lung function in patients with progressive fibrosing interstitial lung diseases, but more evidence is needed to assess its efficacy in SAPF. The management of patients with SAPF should include the identification and treatment of complications such as bronchiectasis and pulmonary hypertension. Further research is needed into the mechanisms underlying SAPF and biomarkers that predict its clinical course.

Dermatological adverse drug reactions to tyrosine kinase inhibitors: a narrative review.

Tyrosine kinase inhibitors (TKIs) target the signal transduction pathways of protein kinases by several modes of inhibition. Adverse effects are generally dose dependent, with certain side-effects unique to each drug. However, due to similarities in target sites, different classes of TKIs may have identical or overlapping side-effect profiles. This narrative review is an attempt to summarize the common and uncommon adverse effects of different classes of TKIs.

Efficacy and safety of 250 mg versus 500 mg oral terbinafine in the treatment of tinea corporis and cruris: A randomised, assessor-blinded comparative study.

Background Though higher doses of terbinafine are often prescribed to treat dermatophyte infections, it is unknown if such doses are more effective than the conventional dose because comparative data are unavailable. Aim To compare the efficacy and safety of a once-daily dose of oral terbinafine 250 mg with 500 mg along with topical clotrimazole in the treatment of tinea infections. Methods A randomised, assessor-blinded, comparative study was carried out. Each group of subjects were administered either 250 mg or 500 mg oral terbinafine once daily for four weeks, along with topical clotrimazole. Clinical improvement was assessed after two weeks and again after four weeks from treatment initiation. Result A total of 60 patients with tinea corporis and cruris were randomised into two groups receiving either 250 mg (group A) or 500 mg (group B) oral terbinafine, along with clotrimazole cream in both groups. Baseline clinical parameters such as lesional activity (papules, vesicles and pustules), degree of erythema, scaling and severity of itching were comparable between both treatment arms. At the first and second follow-ups, no significant differences were found in the clinical parameters between the two groups. At the end of two weeks 13.8% of group A and 14.3% of group B and after 4 weeks 25.9% of group A and 33.3% of group B participants became KOH negative (P = 1.00 and 0.76, respectively). No significant difference in culture negativity was reported at the end of therapy (four weeks) between the two treatment arms (P = 0.78). Overall cure rates were 20% and 33.3% in the two treatment arms respectively at the end of the study (P = 0.82). Conclusion Oral terbinafine 250 mg daily yielded a poor cure rate in tinea cruris and corporis after 4 weeks of treatment and an increased dose of 500 mg did not have any additional benefit.

Evolution of pulmonary hypertension in interstitial lung disease: a journey through past, present, and future.

Interstitial lung diseases (ILD) are a spectrum of disorders often complicated by pulmonary hypertension (PH) in its course. The pathophysiologic mechanism of WHO group 3 PH is different to other forms of PH. The advent of PH is a harbinger for adverse events like mortality and morbidity, implying that the PH component of disease expedites deteriorated clinical outcomes. In fact, WHO group 3 PH due to ILD has the worse prognosis among all groups of PH. Hence, early detection of PH by a comprehensive screening method is paramount. Given considerable overlap in clinical manifestations between ILD and PH, early detection of PH is often elusive. Despite, the treatment of PH due to ILD has been frustrating until recently. Clinical trials utilizing PAH-specific pulmonary vasodilators have been ongoing for years without desired results. Eventually, the INCREASE study (2018) demonstrated beneficial effect of inhaled Treprostinil to treat PH in ILD. In view of this pioneering development, a paradigm shift in clinical approach to this disease phenotype is happening. There is a renewed vigor to develop a well validated screening tool for early detection and management. Currently inhaled Treprostinil is the only FDA approved therapy to treat this phenotype, but emergence of a therapy has opened a plethora of research toward new drug developments. Regardless of all these recent developments, the overall outlook still remains grim in this condition. This review article dwells on the current state of knowledge of pre-capillary PH due to ILD, especially its diagnosis and management, the recent progresses, and future evolutions in this field.

Evaluation of Neutrophil-to-Lymphocyte Ratio and Platelet-to-Lymphocyte Ratio in Chronic Plaque Psoriasis.

Background: Psoriasis is a common inflammatory dermatological condition and affects 2-3% population worldwide. Psoriasis area and severity index (PASI) and body surface area (BSA) are two commonly used scales used to measure disease severity in psoriasis patients. However, these scales are plagued by weaknesses as inter-observer variation and insufficient evaluation of micro-vascular inflammation. Thus, it is necessary to have an objective and simple measure of the severity of inflammation. The ratio of neutrophils-to-lymphocytes (NLR) and platelets-to-lymphocytes (PLR) are simple and inexpensive markers of systemic inflammatory response that can be measured as part of a complete blood count and are already used in the setting of inflammatory diseases. The utility of the NLR and PLR in psoriasis however, remains relatively unexplored. Aims and Objectives: The present study was undertaken to assess if NLR, PLR and C-reactive protein were altered in chronic plaque psoriasis patients as compared to controls and also to determine correlation of NLR and PLR values with disease severity as measured by PASI. Methods: This case control study consisted of equal numbers (45 each) chronic plaque psoriasis patients and control subjects. The subjects were evaluated by way of history taking, clinical and blood examination. Thereafter, the results were tabulated and examined statistically. Results: Our study results indicate that psoriasis patients tended to have a higher neutrophil count, lymphocyte count, NLR and C-reactive protein in comparison the control subjects (P < 0.05). Conclusion: We concluded that such easily available and low cost indices of systemic inflammation are raised in psoriasis patients and are positively correlated with the severity of involvement. They can thus not only be used to monitor the effect of systemic drugs in psoriasis.

Comparison of Effectiveness and Safety of Immunotherapy of Warts with Intralesional Versus Subcutaneous MMR Vaccine: An Open Label Randomized, Parallel Group, Clinical Trial.

Common wart, also known as verruca vulgaris is characterized by focal proliferation of keratinocytes caused by multiple strains of human papilloma virus (HPV). Conventional treatments like chemical cautery, cryotherapy, electro-cautery, etc often fail to cure verruca satisfactorily. The present work was a randomized, parallel-group, non-inferiority clinical trial with an objective of comparing the effectiveness and safety of subcutaneous MMR versus intralesional MMR vaccine in the treatment of multiple warts. Method: Consenting patients of both sexes of 18-65 years age, who have viral warts and did not receive anti-wart treatment in the last 4 weeks and devoid of any active bacterial or viral skin diseases were included in the study. Interventions: Eligible patients were randomized into either group A (receiving 0.3 ml of intralesional MMR) or group B (receiving 0.5 ml of subcutaneous MMR). A total of three injections were administered at two weeks interval. Outcome Measure: The response was considered complete if there was disappearance of the wart(s) and return of the normal skin markings, partial if the wart(s) was regression in size by 50-99% and no response if there was be 0-49% decrease in wart size. Results: Thirty patients were recruited in each group; 5 of group A and 3 of group B were lost to follow up. Modified intention to treat analysis was performed, so, the last observation of such patients was carried forward and all 60 participants were analysed. Number of warts and size of the largest wart were declined significantly (P < 0.001 and P = 0.001 respectively) in both the treatment arms. No significant difference between two groups were seen. Complete clearance including distant lesions was achieved in 22 patients; 12 (48%) in group A and 10 (37.04%) in group B, but the final outcome at the end of the study showed no significant difference between the two t groups. (P = 0.64). Adverse Events: Only one patient had developed mild fever with tender, enlarged parotid gland after first injection of subcutaneous MMR which resolved within two weeks. Conclusion: Efficacy and safety profile of Subcutaneous and intralesional MMR were almost same. Both can be considered as safe and cost effective treatment of warts while the subcutaneous route may be easier to administer.

Bacteriologic Profile Along With Antimicrobial Susceptibility Pattern of Pediatric Pyoderma in Eastern India.

Background Pyogenic skin infection (pyoderma) is a bacterial infection of the skin and its appendages. Primary pyoderma is caused by the direct invasion of healthy skin, whereas secondary pyoderma originates in diseased skin as superimposed conditions, such as scabies, pediculosis, wounds, insect bites, and eczema. This study aimed to identify the clinical patterns and risk factors of pyoderma in a pediatric population and to isolate various causative bacteria and determine their susceptibility patterns. Methodology A prospective study was performed at the Medical College and Hospital, Kolkata, India, for one year (from August 2016 to July 2017), which included all children younger than 12 years with pyoderma attending the outpatient dermatology department (as the study was conducted among the pediatric population, only children below 12 years of age were included). Sterile cotton swabs were used to aseptically collect exudates or pus from lesions and anterior nares, which were used for culture, identification, and antibiotic susceptibility testing of the causative organisms. Results During the study period, a total of 182 patients were included, 121 (66.48%) of whom had primary pyoderma and 61 (33.52%) of whom had secondary pyoderma. Of the 182 patients, 161 showed bacterial growth on culture: 126 (78.26%) were Staphylococcus aureus, 18 (11.18%) were coagulase-negative staphylococci, 16 (9.94%) were Streptococcus pyogenes, and 1 (0.62%) was Pseudomonas aeruginosa. All staphylococci were susceptible to vancomycin and linezolid. Conclusions The most common cause of pyoderma in the pediatric age group is S. aureus, although the prevalence of methicillin-resistant S. aureus was low in this hospital. Proper identification and antibiogram are required for managing these cases.

Perioperative approach to precapillary pulmonary hypertension in non-cardiac non-obstetric surgery.

Pulmonary hypertension (PH) confers a significant challenge in perioperative care. It is associated with substantial morbidity and mortality. A considerable amount of information about management of patients with PH has emerged over the past decade. However, there is still a paucity of information to guide perioperative evaluation and management of these patients. Yet, a satisfactory outcome is feasible by focusing on elaborate disease-adapted anaesthetic management of this complex disease with a multidisciplinary approach. The cornerstone of the peri-anaesthetic management of patients with PH is preservation of right ventricular (RV) function with attention on maintaining RV preload, contractility and limiting increase in RV afterload at each stage of the patient's perioperative care. Pre-anaesthetic evaluation, choice of anaesthetic agents, proper fluid management, appropriate ventilation, correction of hypoxia, hypercarbia, acid-base balance and pain control are paramount in this regard. Essentially, the perioperative management of PH patients is intricate and multifaceted. Unfortunately, a comprehensive evidence-based guideline is lacking to navigate us through this complex process. We conducted a literature review on patients with PH with a focus on the perioperative evaluation and suggest management algorithms for these patients during non-cardiac, non-obstetric surgery.

Topical Antibacterials in Dermatology.

Topical antibacterials are commonly used for superficial pyodermas such as impetigo and treatment or prevention of infections following minor cuts, abrasions, burns, and surgical wounds. Several antibiotics and antiseptics are available for use in different indications. One of the major uses of topical antibacterials is acne in which benzoyl peroxide is the drug of the first choice either singly or in combination with antibiotics or retinoids. Mupirocin and fusidic acid are the two most commonly used antibiotics for the treatment of superficial pyodermas and eradication of staphylococcal carrier state. Bacterial resistance to topical antibiotics is a growing concern and topical antiseptics such as gentian violet are getting renewed interest as alternatives. Incidence of contact dermatitis is a limiting factor for the use of several topical antibacterials. Although many botanical products have demonstrated in vitro activities against skin pathogens, their clinical utilities remain to be established by good-quality clinical trials.

Dermatoses in the Elderly: Clinico-Demographic Profile of Patients Attending a Tertiary Care Centre.

BACKGROUND: Elderly population is vulnerable to develop a multitude of dermatological diseases, owing to comorbidities and polypharmacies. OBJECTIVE: To know the prevalence of dermatological conditions in elderly patients attending outpatient department, determine the pattern and relative frequency of skin diseases, and find the relation with associated comorbidities. MATERIALS AND METHODS: We performed a cross-sectional study on 250 patients, aged ≥60 years. Clinical diagnosis was done, followed by appropriate investigations when required. Descriptive data was analyzed on the parameters of range, mean ± S.D., frequencies, etc., Continuous variables were analyzed using unpaired t-test/Mann-Whitney U test and categorical data by Fisher's exact test/Chi-square test. Statistical software Medcalc version 10.2.0.0 for Windows vista was used. P value =0.05 was considered statistically significant. RESULTS: 250 patients were evaluated, 164 males (65.5%) and 86 females (34.4%). Mean age was 67.87 ± 7.29 years. Commonest disease category was infection (30%), followed by dermatitis (29.6%), papulo-squamous (18.4%), and immunobullous (6.4%). Difference in acute and chronic disease was significant (P = 0.0001). 30% had infections; fungal (50.66%), bacterial (32%), and viral (17.33%). 74 patients had dermatitis (29.6% of study population). Commonest systemic disease was hypertension (23.2%), followed by diabetes mellitus (19.6%). Association of diabetes mellitus was significant (P = 0.0014), more in infective dermatoses (P = 0.0007). All had signs of aging; idiopathic guttate hypomelanosis (51.2%), xerosis (45.2%), seborrheic keratosis (42.6%), cherry angioma (33.2%), senile acne (6.6%). Photoaging was noted as wrinkling (98.8%), freckles (35.6%), purpura (10.8%), telangiectasia (5.6%). People involved in outdoor activity had higher Glogau scale (3.01 ± 0.69) compared to those indoors (2.44 ± 0.74), statistically significant difference (P = 0.001). CONCLUSION: Our study is the first of its kind, in Eastern India, where we evaluated and explored the disease pattern and extent of geriatric dermatoses among patients attending dermatology OPD of a tertiary care hospital.

Early-Onset Versus Late-Onset Psoriasis: A Comparative Study of Clinical Variables, Comorbidities, and Association with HLA CW6 in a Tertiary Care Center.

Background: Psoriasis is a chronic inflammatory diseaseresulting from a complex interplay of genetic and environmental factors affecting the skin, nail and joints. Two distinct types of psoriasis are said to exist (i) early onset psoriasis (EOP), beginning before the age of 40 years and (ii) late onset psoriasis (LOP), beginning ≥40 years; with the presence of Human lymphocyte antigen (HLA) Cw6, present in majority of patients with early onset. Several studies demonstrated clinical and genetic differences between EOP and LOPamong European and East Asian populations. Lack of similar study in the Indian population has prompted us to undertake the present work. Aims and Objectives: (i) To compare the clinical patterns of early onset and late onset psoriasisin patients attending the Dermatology outpatientdepartment (OPD) and admitted in the in-patient department (IPD). (ii) To analyze the association age of onset with presence of HLA Cw6. Materials and Methods: It was an institution-based, descriptive, cross-sectional study. Consecutive patients with psoriasis at the OPD and IPD of the department of Dermatology during the study period, were recruited in the study after obtaining informed consents. Detailed history was obtained regarding the disease, co-morbidities and complications. Through physical examination was carried out, PASI was calculated and blood samples were drawn fromconsenting adult patients (age>/=18 years) to study the presence of Cw6. Results: The study population (n=250) wasbroadly divided into "Early onset psoriasis(EOP)" (n=138) and Late onset psoriasis (LOP)" (n=112).Significant higher occurrence of positive family history, nail involvement and koebnerization were found in EOP, but such differences were absent considering the types, patterns, joint involvement, severity and HLACW6 positivity. Conclusion: This study supports the concept of two subtypes of psoriasis based on age of onset showing different clinical and evolutionary features.

Dysbiotic Lesional Microbiome With Filaggrin Missense Variants Associate With Atopic Dermatitis in India.

Background: Atopic Dermatitis (AD) has been associated with the loss of function (LoF) mutations in Filaggrin (FLG) gene and increase in relative abundance of specific microbes in the lesional skin, predominantly in Caucasians. Our study aims to determine, in Indian AD patients, (a) the prevalence of FLG LoF and missense mutations, and (b) the nature and extent of dysbiosis and altered microbial pathways with and without mutations in FLG. AD patients (n = 34) and healthy controls (n = 54) were recruited from India in this study and shotgun sequencing was carried out in a subset of samples with adequate microbiome DNA concentration. Host DNA from the same subset of samples was subjected to FLG coding region sequencing and host-microbiome association was estimated. Results: The prevalence of FLG LoFs that are associated with AD globally were significantly lesser in our cases and controls (8.6%, 0%) than those reported in Europeans (27%, 2.6%). Staphylococcus aureus was present only on AD skin [abundance in Pediatric AD: 32.86%; Adult AD: 22.17%], but not on healthy skin on which Staphylococcus hominis (Adult controls: 16.43%, Adult AD: 0.20%; p = 0.002), Cutibacterium acnes (Adult controls:10.84%, Adult AD: 0.90%; p = 0.02), and Malassezia globosa (Adult controls: 8.89%, Adult AD: 0.005%; p = 0.001) were significantly more abundant. Microbial pathways mostly associated with skin barrier permeability, ammonia production and inflammation (Arginine and Proline metabolism, Histidine Metabolism and Staphylococcus aureus infection) were significantly enriched on AD skin metagenome. These pathways are also reported to impair antimicrobial peptide activity. Among AD patients with missense single nucleotide polymorphisms harboring "potentially damaging" alleles in FLG gene, damaging allele dosage was significantly (p < 0.02) positively correlated with relative abundance of phylum_Proteobacteria up to order_Pseudomonadales and negatively correlated with phylum_Firmicutes up to species_Staphylococcus aureus. Conclusion: Our study has provided evidence that host DNA profile is significantly associated with microbiome composition in the development of AD. Species and strain level analysis showed that the microbial pathways enriched in AD cases were mostly found in MRSA strains. These evidences can be harnessed to control AD by modulating the microbiome using a personalized strategy. Our findings on the association of FLG genotypes with the microbiome dysbiosis may pave the way for a personalized strategy to provide a more effective control of AD.

A Profile of 23 Indian Patients with Purpura Fulminans: A Retrospective, Descriptive Study.

BACKGROUND: Purpura fulminans (PF) is a potentially fatal uncommon disorder of intravascular thrombosis and is clinically characterized by rapidly progressive hemorrhagic infarction of the skin. OBJECTIVE: To describe the clinical feature and outcome of a series of patients with PF. MATERIALS AND METHODS: A descriptive study based on review of case records was carried out at a tertiary care hospital in Kolkata. RESULTS: Twenty three consecutive cases seen over a period of 8 years were studied. The age range was 4 days to 78 years (mean 35.6 years) with a male to female ratio of 1:2.8. Hemorrhagic rash was the universal presenting symptom. Other major presenting features included pneumonia (26.1%), sudden-onset shock syndrome (21.7%), and urinary tract infection (17.4%). All patients presented with retiform purpura and lesional necrosis and 8 (34.8%) patients had associated peripheral gangrene. Nineteen (82.6%) patients had sepsis and 60.9% patients had vesiculo-bullous lesion. Pneumococcus was the most common (26.1%) pathogenic organism detected. The precise cause of PF could not be detected in two (8.7%) patients. One patient (4.3%) with neonatal PF had protein C deficiency. All patients had evidence of disseminated intravascular coagulation (DIC). One patient had to undergo a below knee surgical amputation and one patient had autoamputation of the digits. Ten (43.5%) patients succumbed to their illness. Seven of the 8 patients who had peripheral gangrene had a fatal outcome. LIMITATIONS: Relatively small sample size and a referral bias were a few limitations of the present study. CONCLUSION: The present study emphasizes that PF is a cutaneous marker of DIC. Association of peripheral gangrene, leukopenia and neutropenia may be the reason for the high mortality rate.

Clinico-Demographic Profile of Patients with Foot Dermatitis: A Cross-Sectional Study with Special Reference to Patch Test Results.

BACKGROUND: Foot dermatitis is a common debilitating dermatological disorder where the eczematous process predominantly involves the feet. AIMS AND OBJECTIVES: To analyze the clinico-demographic profile, type, clinical pattern, and evaluate the role of patch testing to determine the causative factors of foot dermatitis. MATERIALS AND METHODS: Fifty-eight new patients clinically diagnosed with foot dermatitis were subjected to detailed history taking and physical examination. The patients were subjected to patch testing using the Indian standard battery and Indian footwear series (Contact and Occupational Dermatoses Forum of India [CODFI]). RESULTS: Among the 58 patients (mean age 31.48 ± 16.4 years, M:F 1:1.2), the majority (39.7%) presented with allergic contact dermatitis (ACD) followed by irritant contact dermatitis (ICD) (19%), while atopic dermatitis was the least (3.45%). However, 48% of our patients reported a history of atopy. About 43.5% of ACD patients showed a positive patch test reactions to at least one allergen of Indian standard battery and footwear series. Mercaptobenzothiazole (MBT) was the commonest allergen (50%), followed by potassium dichromate (40%), thiuram mix (20%) while paraphenylenediamine was the least common (10%). Dorsum of the foot was affected most commonly (55.17%), followed by toe (46.55%) and sole (41.38%). Scaling was observed in almost 80% of patients followed by crusting. Housewives were affected most commonly followed by students and cement workers. CONCLUSION: Rubber and rubber chemicals were found to be the commonest allergen inciting foot dermatitis. Atopy might be a predisposing factor in these patients. Thus, patch testing is recommended to determine the cause of foot dermatitis and provide suitable treatment.

An update on sarcoidosis-associated pulmonary hypertension.

PURPOSE OF REVIEW: Pulmonary hypertension in sarcoidosis is a well known entity. Sarcoidosis-associated pulmonary hypertension (SAPH) incurs substantial morbidity and mortality. This review examines recent literatures published on epidemiology, prognosis and therapeutic management in SAPH. RECENT FINDINGS: Several registries have been published between 2017 and 2020. The consensus conclusion - SAPH is a harbinger for poor prognosis. Several factors were noted for predicting adverse outcome in SAPH like reduced 6-min walk distance and diffusing capacity for carbon monoxide. Given its adverse outcome, experts have now focused on methods for early screening of SAPH in sarcoid patients. The exploration of pulmonary vasodilator drugs in SAPH is ongoing. In recent times, trials have been published utilizing Macitentan and parenteral prostacyclin in severe SAPH. Although these trials show encouraging results, the evidence from these studies are limited to approve these agents as preferred drugs for treating SAPH. A large multicentric trial of drugs used for pulmonary arterial hypertension with meaningful, yet feasible, event driven endpoint is still lacking. Lately, interventional treatment by pulmonary artery balloon pulmonary angioplasty and stenting has gained traction for treating pulmonary artery stenosis and chronic thromboembolic pulmonary hypertension. However, the conclusion is still based on small cohorts or case series. SUMMARY: Several registries have highlighted SAPH portends an unfavorable consequence. On the contrary, no published guideline exists to treat SAPH. The precise role of immunosuppressive agents is unclear. The limited evidence favoring use of pulmonary vasodilators arise from small retrospective case series and/or single-center nonrandomized observational studies. Further multicenter randomized research is warranted to better define patient population to treat and how best to treat them.

Phakomatosis Pigmentovascularis: A Clinical Profile of 11 Indian Patients.

INTRODUCTION: Phakomatosis pigmentovascularis (PPV) is a rare congenital syndrome characterized by the simultaneous presence of capillary malformation and pigmentary nevi. The objective of our study was to describe the clinical characteristics of a series of Indian patients presenting with this rare entity. MATERIALS AND METHODS: It was a record-based descriptive case series. RESULTS: A total of 11 patients with PPV (9 females, 2 males, age range: 7 days to 45 years; mean 11.6 years) were studied. Port wine stain was present in 10 (91%) patients and one patient (9%) had cutis marmorata telangiectatica congenita. Isolated nevi of Ota and Mongolian spots were seen in 4 (36%) patients each. Simultaneous presence of both Mongolian spots and nevus of Ota was present in 1 (9%) patient. The combination of Mongolian spots and bilateral palatal hyper-melanosis was noticed in 2 (18%) patients. Café au lait macule was present in one patient. Bilateral ocular melanosis was found in 3 (27%) patients. Unilateral ocular melanosis was noticed in 4 (36%) patients. Two patients (18%) had history of seizure disorder and intracranial vascular anomalies on MRI imaging. Two patients (18%) had features of Klippel-Trenaunay syndrome. According to the traditional classification, three patients had PPV type 2b, one patient had PPV type 5b, and seven patients had PPV type 2a. According to the Happle's classification, 10 patients had PPV of cesio flammea type, and one patient had PPV of cesio marmorata type. LIMITATIONS: We could not perform genetic study of the patients. CONCLUSION: Our findings emphasize the importance of detailed systemic evaluation including ocular examination and brain imaging in every patient of PPV.