Nail Changes Research Articles

Cutaneous manifestations of diabetes mellitus: Correlation with HbA1C level—A cross-sectional observational study from a tertiary care center in Eastern India

Abstract Background: Diabetes mellitus (DM) is a common and debilitating endocrinological disease that affects a variety of organs, including the skin. Between 30% and 70% of patients with DM, both type 1 and type 2, will present with a cutaneous complication at some point during their lifetime. A variety of dermatologic manifestations have been linked with DM; these conditions vary in severity and can be benign, deforming, and even life-threatening. Such skin changes can offer insight into patients’ glycemic control and, indirectly, their HbA1C levels. It may be the first sign of metabolic derangement in undiagnosed patients with diabetes. Objectives: Statistically correlate HbA1C value with glycemic control using cutaneous manifestations of DM. To classify the severity of DM by taking into account certain cutaneous manifestations. Materials and Methods: Consecutive 103 diabetic patients attending dermatology outpatient department of a tertiary care hospital in Kolkata were included in the study. History regarding duration and type of DM, control of diabetes, and drug history were taken. Patients underwent thorough dermatological examinations to evaluate the skin disorder. Appropriate routine and laboratory investigations were done. Relevant microbiological and histopathological examinations were carried out in atypical and doubtful cases to confirm the diagnosis. Data were recorded and analyzed. Results: Out of 103 diabetic patients included in the study, 59 (57.3%) were male, and 44 (42.7%) were female, of which 89 patients had cutaneous manifestations associated with DM. We have noticed a significant correlation between certain types of skin manifestations and HbA1c values. The fungal infections were seen among mid (8.0–9.5) and low (6.5–8.0) HbA1c ranges. Most of the cases of xerosis and associated pruritus had HbA1c levels in the mid range. Vitiligo, nail changes, and systemic complications were mostly seen among HbA1c values in the mid and high range (9.5–11). In our study, there was a strong association of HbA1c value with the duration of diabetes (P = 0.001), diabetic dermopathy (P = 0.012), and systemic complications of DM (P = 0.027). Acanthosis nigricans (P = 0.016), nail changes (P = 0.041), and fungal infection (P = 0.032) also showed a significant association with HbA1c values. A statistically weak association was observed with bacterial infection (P = 0.362), xerosis (P = 0.487), lichen planus (P = 0.066), and vitiligo (P = 0.778). Conclusion: DM is the commonest endocrine disorder that frequently accompanies skin manifestation. Recognition of clinical features of DM is important as delayed detection is associated with comorbidities like coronary artery disease, hypertension, and dyslipidemia; until recently, dermatologists were of the opinion that diabetic ulcers were the only skin manifestation that predated its development but studies have shown that there are many more cutaneous manifestations specific to DM. These manifestations appear earlier than the systemic manifestations and, if recognized, can help in the early diagnosis of DM. Cutaneous manifestations can serve as a predictor for the long-term glycemic control of patients and help endocrinologists in preventing the grave complications of a silent killer like diabetes. Limitations: A small sample size compromised the external validity of the study. The study was conducted in a tertiary care center and was thus not representative of the situation in the field.

E25 The pattern of juvenile psoriatic arthritis in a tertiary centre in Libya

Abstract Background Juvenile psoriatic arthritis (JPsA) is a relatively rare condition in childhood as it represents ∼5% of the whole Juvenile Idiopathic Arthritis (JIA). There are fewer reports describing the characteristics and outcome of patients with JPsA. Aim To describe the characteristic features and the treatment modalities of JPsA among Libyan children in pediatric rheumatology clinic at the Tripoli Children Hospital which is the only referral clinic covering the western, northern and southern part of Libya and to compare our results with other populations worldwide. Methods All the medical records of the children who were diagnosed to have JPsA according to ILAR classification criteria or Vancouver criteria (definite or probable) from January 2001 to January 2020 were included and retrospectively reviewed. Data were collected about demographic, clinical, and laboratory features and treatment Results The study included a total of 30 cases of JPsA that represents a 12% of total JIA cases over the study period, the mean age at presentation was 5.8 ± 5.3 years with male to female ratio of 1:1, with the mean duration of symptoms prior to diagnosis of JIA of 9 ± 10 months. The foremost common type of arthritis was peripheral polyarthritis (83% & 58.3%), with the Small joints commonly affected, 30% of patients had hip joint involvement. 13% of the patient had psoriasis, half of them before presentation of arthritis. Nail changes secondary to psoriasis was seen in 1 of the cohort, and the percentage of patients with dactylitis among the group was 30%. Family history of psoriasis found in 83% of the cohort, 26% found to be ANA positive. Chronic uveitis occurred in 10%. Patients of the total cohort, ANA positive, Unilateral anterior uveitis being the most common presentation. The following table summarizes the Medications used during the course of the disease At the time of the first visit 100%of total patients fulfilled the Vancouver criteria for juvenile PsA, While 5 of the12 patients didn’t fulfilled the ILAR criteria for JPsA. Conclusions Juvenile psoriatic arthritis constituted 12% of our patients with juvenile idiopathic arthritis. Juvenile psoriatic arthritis constitutes 8% to20% of JIA patients worldwide with equal gender distribution and peak age at presentation of 6–13 years. More than four peripheral joints involvement were the most frequent presentation. Half of the patients had skin involvement before the development of arthritis. Prevalence of chronic anterior uveitis was higher in our cohort than worldwide (10% vs 5% respectively) as an extra articular manifestation. All patients were treated with NSAIDs at presentation and/or during follow-up. Methotrexate was the most commonly used second-line agent Ethics Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.

A clinicoepidemiological study of geriatric dermatoses

Skin diseases are a common and inevitable consequence of ageing. Moreover, the clinical presentation is not as classical as they do in the younger population. A lifetime of solar exposure, along with intrinsic changes in the dermal structures, predisposes to a variety of skin diseases.
 The aim: to study the spectrum of various geriatric dermatoses among our patient population at the Department of Dermatology, Venereology, and Leprosy at Kamineni Academy of Medical Sciences and Research Centre.
 Materials and methods: in this study, a total of 200 patients aged 60 years and above attending the DVL OPD of Kamineni Academy of Medical Sciences and Research Centre were included.
 Results: maximum number of patients in this study belonged to 60-65 years (60 %), Male to female ratio was 1.86:1. Most of the males had agriculture work, and most of the females were housewives. Diabetes mellitus was the commonest associated systemic disease seen in 68 cases (34 %), and generalised pruritus was the commonest symptom seen in 64 (32 %) cases, of which 42 cases (65.6 %) were associated with xerosis. Pathological skin disorders and eczematous conditions were seen in 56 out of 200 cases. Of this, asteatotic eczema was the common finding among the eczematous conditions seen in 14 cases (7 %). Psoriasis was seen in 32 (16 %) and lichen planus in 10 cases (5 %). Infectious diseases were seen in 78 cases (39 %). Of these, fungal infections were common, seen in 28 cases (14 %). The benign tumour was seborrheic keratosis in this study, seen in 61 cases (30.5 %); among the malignant tumours, 4 cases (2 %) of basal cell carcinoma and 2 cases (1 %) of squamous cell carcinoma were seen. Among 16 cases of bullous disorders, bullous pemphigoid was seen in 12 (6 %) cases. Among 22 cases of psychocutaneous disorders, delusional parasitosis was seen in 10 cases (5 %), and perforating folliculitis in 15 cases (7.5 %). Loss of luster was the commonest nail change seen in 182 cases (91 %), followed by nail plate thickening in 54 cases (27 %). Greying of the hair was seen in all cases. Out of 70 females, diffuse hair loss was seen in 58 cases (82.9 %), and out of 130 males, androgenetic alopecia was seen in 72 cases (55.4 %).
 Conclusion: skin diseases cause considerable morbidity in the elderly, particularly if associated with other comorbid conditions. Health education on proper skin care, avoidance of irritants and self-medication etc., would help to reduce the incidence of common dermatoses.

Evaluation of the Knowledge of Primary Care Physicians About Important Nail Diseases Before and After a Short Online Training.

Nail diseases are often diagnosed late with a potential prognostic and functional impact. This could be partly due to knowledge gaps among primary care physicians (PCPs). To evaluate the knowledge about diagnosis and management of ten common/important nail conditions in a population of French PCPs and its improvement after a 31-minute online training session. We submitted 10 pre-test and post-test clinical cases and an educative online course on the diagnosis and the management of nail diseases to 138 volunteer PCPs; 73 completed the whole training path. Compared to pre-test, more PCPs in the post-test required an urgent second opinion to dermatologist for pigmented melanoma (100% versus 80.3%; P <0.05) and use of inappropriate/dangerous systemic treatment for trauma-induced nail changes was reduced after the training program (0% versus 6.8%; P <0.05). A lack of knowledge remained after training for amelanotic melanoma with an increase of mycological/bacteriological tests (9.6% versus 0%; P <0.05). Management of nail diseases by our panel of PCPs was suboptimal and was improved after a short online training.

Cell Surface B2m-Free Human Leukocyte Antigen (HLA) Monomers and Dimers: Are They Neo-HLA Class and Proto-HLA?

Cell surface HLA-I molecules (Face-1) consist of a polypeptide heavy chain (HC) with two groove domains (G domain) and one constant domain (C-domain) as well as a light chain, B2-microglobulin (B2m). However, HCs can also independently emerge unfolded on the cell surface without peptides as B2m-free HC monomers (Face-2), B2m-free HC homodimers (Face 3), and B2m-free HC heterodimers (Face-4). The transport of these HLA variants from ER to the cell surface was confirmed by antiviral antibiotics that arrest the release of newly synthesized proteins from the ER. Face-2 occurs at low levels on the normal cell surface of the lung, bronchi, epidermis, esophagus, breast, stomach, ilium, colorectum, gall bladder, urinary bladder, seminal vesicles ovarian epithelia, endometrium, thymus, spleen, and lymphocytes. They are upregulated on immune cells upon activation by proinflammatory cytokines, anti-CD3 antibodies, antibiotics (e.g., ionomycin), phytohemagglutinin, retinoic acid, and phorbol myristate acetate. Their density on the cell surface remains high as long as the cells remain in an activated state. After activation-induced upregulation, the Face-2 molecules undergo homo- and hetero-dimerization (Face-3 and Face-4). Alterations in the redox environment promote dimerization. Heterodimerization can occur among and between the alleles of different haplotypes. The glycosylation of these variants differ from that of Face-1, and they may occur with bound exogenous peptides. Spontaneous arthritis occurs in HLA-B27+ mice lacking B2m (HLA-B27+ B2m-/-) but not in HLA-B27+ B2m+/- mice. The mice with HLA-B27 in Face-2 spontaneous configuration develop symptoms such as changes in nails and joints, hair loss, and swelling in paws, leading to ankyloses. Anti-HC-specific mAbs delay disease development. Some HLA-I polyreactive mAbs (MEM series) used for immunostaining confirm the existence of B2m-free variants in several cancer cells. The upregulation of Face-2 in human cancers occurs concomitantly with the downregulation of intact HLAs (Face-1). The HLA monomeric and dimeric variants interact with inhibitory and activating ligands (e.g., KIR), growth factors, cytokines, and neurotransmitters. Similarities in the amino acid sequences of the HLA-I variants and HLA-II β-chain suggest that Face-2 could be the progenitor of both HLA classes. These findings may support the recognition of these variants as a neo-HLA class and proto-HLA.

Cutaneous adverse effects of chemotherapy in cancer patients: A clinico epidemiological study

With chemotherapy being the major modality of treatment, cutaneous and systemic side effects have been a concern for not only the patients, but also the treating clinicians. Because chemotherapy target not only the cancer cells, but also the other rapidly proliferating cells, skin and skin appendages like hair and nails are most commonly affected.: To study the cutaneous adverse events associated with the commonly used cancer chemotherapy drugs.: 120 patients of clinically diagnosed cancer who underwent chemotherapy and satisfied the inclusion and exclusion criteria were included in this observational study. Photographic images of skin, hair and nails were taken before and after every cycle of chemotherapy were collected. Data were analysed using SPSS version 16.0.: 120 patients including 72 females and 48 males were included in this study with majority (56%) belonging to age group of 55-70 years. The common indications for chemotherapy were carcinoma breast (34%), carcinoma oropharynx (22%) and carcinoma stomach (12%).Hair changes were the most common presentation, reported in 38 patients, skin changes in 35 cases, nail changes in 15 cases. Xerosis (46%) and hyperpigmentation (22%) were the most commonly observed adverse event affecting skin.: Since all cancer chemotherapy agents are not target based, adverse effects of skin and appendages become inevitable. However, early detection of such adverse effects can help them managed effectively and thereby reducing the morbidity and increasing the quality of life of cancer patients.

Cutaneous Manifestations in Celiac Disease: An Observational Study

Abstract Background: Cutaneous manifestations associated with celiac disease (CD), other than dermatitis herpetiformis, are poorly known. The aim of the present study was to study the spectrum of dermatological disorders in children with CD. Methodology: A total of 100 cases of biopsy-confirmed CD (both new and old) were recruited after taking written informed consent/assent from the child/guardians. The patient’s demographic details, history, vitals, general physical examination, systemic examination, and cutaneous examination findings were recorded. Relevant investigations were carried out, depending on the clinical findings of the cutaneous examination. Results: A total of 100 patients comprising 62 girls and 38 boys were studied, with the average age being 8.08 ± 3.67 years. Seventy patients showed the presence of dermatological findings. Cutaneous, mucosal, nail, and hair findings were seen in 35%, 7%, 14%, and 14% of patients, respectively. A higher incidence of dermatological findings was found in females (39%) compared to males (18%). The most common cutaneous conditions seen were atopic dermatitis (17%) and xerosis (10%). The most common mucosal finding was oral aphthae (4%). The most common nail changes were leukonychia and ragged cuticles (4% each). There was no statistical correlation between the presence of dermatological findings with the duration of the disease or with the duration of a gluten-free diet. Conclusion: Mucocutaneous manifestations are present in a significant proportion of patients with CD. Further studies with a larger sample size are needed to determine whether these findings are true associations or coincidental.

Clinical and onychoscopic evaluation of nail changes in psoriasis at a tertiary-care hospital: A cross-sectional study

Background: Up to 30–50% of cases of psoriasis experience nail involvement, while 5–10% may have only isolated nail disease. Recently, the use of onychoscopy, a non-invasive technology, has emerged as a promising tool that may eliminate the necessity of a nail biopsy in most cases. Objective: The objective of this study was to assess the onychoscopic characteristics of the nail unit in individuals with nail psoriasis. Materials and Methods: The study recruited fifty patients with a clinical diagnosis of nail psoriasis. Each nail underwent onychoscopic assessment. The clinical degree of cutaneous and nail involvement was evaluated with the Psoriasis Area and Severity Index (PASI) and the Nail Psoriasis Severity Index (NAPSI), respectively. Results: The following findings were observed significantly more often on onychoscopy than with the naked eye: pitting (p = 0.03), leukonychia (p = 0.04), onycholysis (p = 0.04), and splinted hemorrhage (p = 0.05). The other novel findings included fuzzy lunula, which was only onychoscopically (8%), We also encountered 4% of cases of triangular onychomadesis and 6% of non-traumatic Median canaliform dystrophy of Heller. Conclusion: Our findings suggest that, even before clinical symptoms become obvious, onychoscopy may help with nail lesion diagnosis. Key words: Onychoscopy; Nail Psoriasis; Psoriasis; Nail Pitting; Fuzzy Lunula; Triangular Onychomadesis

A Cross-Sectional Study of Dermatologic Manifestations in Patients with Chronic Renal Failure on Hemodialysis

Background: Chronic renal failure patients undergoing dialysis suffer from various side effects and complications where skin ailments are one of them. Skin is the largest and one of the most dynamic organs of the body and can be used as a window since it is easily accessible for observation and reflects changes seen in multiple organs including kidneys. Objective: The objective of this study is to describe various cutaneous manifestations in chronic kidney disease patients undergoing hemodialysis. Materials and Methods: A hospital-based, cross-sectional, observational study was conducted in the department of dermatology, venereology, and leprosy at a tertiary care center from November 2019 to May 2021. The demographic details and clinical manifestations were recorded. Data were recorded as numbers and percentages. For categorical data, the number and percentage were used in the data summaries, and data were analyzed by the Mann‒Whitney U test for the comparison of two data. Results: A total of 94 patients were included in the study. The most common manifestation observed was pruritus (85.1%), followed by xerosis (84%). Other findings seen in the study were mainly nonspecific changes such as pallor (59.57%), diffuse hyperpigmentation (3.2%), and ecchymosis (16%). Nail changes (56.38%) observed were half-and-half nails (22.6%), onycholysis (11%), and Beau’s lines (9%). Mucosal changes (36.17%) included depapillated tongue (12.8%) and macroglossia (8.5%). Other findings such as skin infections and keloid were also noted. New finding such as angina bullosa hemorrhagica was seen in one patient. Specific change like Kyrle’s disease was seen in 6.4% of the study group and 50% of these patients had concomitant diabetes mellitus. Conclusion: Patients on hemodialysis present with an array of cutaneous manifestations. The most common include pruritus and xerosis. Prompt diagnosis and early treatment help alleviate many of the cutaneous manifestations posed due to hemodialysis.

Clinico-epidemiological profile and onychoscopic pattern of nail disorders in geriatric population

Background: Adequate knowledge about nail changes in the geriatric population can help in the early identification and timely treatment of underlying disease adding to the quality of life of the elderly population with nail concerns. The present study is aimed to study the clinico-epidemiological profile and onychoscopic pattern of nail disorders in geriatric populations. Methods: This observational study included 100 subjects aged 60 years or more. Clinical examination and onychoscopic examination by Dermlite DL4 dermoscope with 10x magnification of the nail bed, nail plate, nail fold, and hyponychium in all the 20 nails were done. Even single positive findings in a single nail are considered in the study. If clinically suspected of onychomycosis, 20% potassium hydroxide mount was performed to identify fungal elements. Results: Mean age of the subjects was 66.27±5.546 years. Male: female ratio was 2.1:1. Mean duration of complaints pertaining to nail changes was 4.7±3.864 years. The damaged cuticles were the universal finding in all the subjects (100%) in nail fold examination, nail dystrophy and longitudinal ridges were seen in all [100.0%)] subjects of nail plate examination. The most frequently observed change in nail bed has been splinter haemorrhage [26% versus. 32%] and in hyponychium was subungual hyperkeratosis [36% versus 37.0%] clinically and dermoscopically respectively. Onychomycosis was diagnosed in 25% of total subjects. Conclusions: Onychoscopy is an important, non-invasive modality to identify and diagnose the nail changes earlier which helps in managing the disease earlier.

Assessment of prognostic markers of alopecia areata in pediatric patients

Alopecia areata (AA) is associated with impairment of psychological health being more pronounced in pediatric population. Hence, it becomes imperative to study the AA in pediatric population so that early diagnosis and adequate treatment can be initiated at early stage to prevent further morbidity associated with the condition. This observational study was conducted in the department of dermatology venereology and leprosy, SAIMS and PG institute, Indore, India after obtaining approval from the IEC. The study included 14 children with AA who visited the hospital over the period of 1 year. Results showed that Mean age of the patients was 8.8±3.423 years with M:F ratio of 3:4. AA subtotalis was found to be most frequent (57.1%). The mean duration of alopecia was reported to be 9.3±7.965 months. The most common finding was the presence of a single patch (35.7%) and 50% of subjects have had a single episode of the disease. 64.3% of children had involvement of multiple sites with scalp being the most common (85.7%). There is high prevalence of markers of disease severity and activity amongst pediatric patients with AA. High prevalence of nail changes, mark hair (64.3%), coudability hair (50.0%), yellow dots (42.8.0%), black dots (78.6%), S. vellus hair (28.6%) shows that children have high chance of AA progressing to severe forms. There is high prevalence of markers of disease severity and activity amongst pediatric patients with AA with AA subtotalis more common among children as compared to the patchy type.

Onychomadesis and Staphylococcus aureus Infection of the Nail after HandFood-Mouth Disease

Hand-foot-mouth disease is a viral disease that occurs mostly in children, but sometimes affects adults as well. It is most often caused by Coxsackie virus A16 or Enterovirus 71. Other strains of these viruses can also be an etiological factor for the development of the disease. It usually starts with an increase in body temperature and fatigue. Subsequently, papulo-vesicular rashes appear on the oral mucosa, palms and soles. Over time, some of them form small erosions or ulcers. Although rare, few weeks after an infection has passed, nail changes can be seen. Some are associated with falling off the nail plate, others with onychomаdеsis or the development of a bacterial and/or fungal infection. In this report, we present a clinical case of a 22-year-old woman who in the month of September 2022 had hand-foot-mouth disease and two months later developed nail changes associated with a change in the integrity and color of the nail plate.

Alopecia Universalis Treated with Ayurveda Protocol and Assessed by SALT (Severity of Alopecia Tool) - A Case Report

Alopecia Universalis is a condition that causes generalized hair loss in the body. It is associated with nail changes in 10-15 % of Autoimmune origin conditions. Several medicines normalize hair growth, but this condition requires a treatment modality that concentrates on the root cause of hair falling all over the body. Here is a case report about the Ayurvedic approach in a 48-year-old male patient with Alopecia Universalis presented with hair loss all over the body associated with itching. We administered the Ayurveda protocol and Severity of Alopecia Tool (SALT) criteria assumed before and after assessing clinical output.

TRLS-08. EXPERIENCE WITH THE SELECTIVE FIBROBLAST GROWTH FACTOR INHIBITOR ERDAFITINIB IN THREE PEDIATRIC PATIENTS WITH CNS TUMORS

Abstract Activation of FGF signaling has been described for at least 3% of pediatric solid tumors including pediatric gliomas. Therapeutic actionability of FGF signaling activations, however, remains challenging. Here, we report on three patients with FGFR activation treated with erdafitinib, originally approved for the treatment of FGFR3 or FGFR2 mutated urothelial carcinoma. Our patients were treated for posterior fossa group A ependymoma (patient (Pat) 1&amp;2) and glioma NOS °II-III (Pat3). Dose of erdafitinib at initiation of therapy was 5,2mg/kg/d; 3,8mg/kg/d and 2,8mg/kg/d for Pat1-3 respectively. Rationale for treatment with erdafitinib was increased FGFR3 expression in tumor tissue (TT) of Pat1, increased FGFR1 expression in TT of Pat2, and in Pat3 an activating FGFR1 internal tandem duplication (ITD). Both ependymoma patients received erdafitinib as a fourth line treatment and progressed rapidly under therapy. Treatment was discontinued after 2 weeks (Pat2) and 3 months (Pat1) due to progressive disease. Pat3, with the FGFR1 ITD, responded very well to therapy with a decrease in tumor size and reduced contrast-enhancement after 3&amp;6 months. Therapy was discontinued after six months due to dystrophic nail changes. During treatment, our patients reported on diarrhea, abdominal pain, pain in the lower extremities, dysgeusia, hypersensitivity of the oral mucosa, dry skin and dystrophic nail changes. Additionally, we observed a distinct increase in longitudinal growth (Pat1&amp;3), increased serum phosphate levels, LFPs, alpha-amylase, cholinesterase, alkaline phosphatase and LDH. Summing up, treatment with erdafitinib has high potential for a selected cohort of pediatric CNS tumor patients, as demonstrated by the treatment response of Pat3 with the FGFR1 IDT. However, further defining the exact molecular alterations that predict the individual patient’s response as well as side effects in the pediatric population are imperative to elucidate the further role of erdafitinib in treatment of pediatric CNS tumors.

Erdafitinb in patients with FGFR-altered tumors: Results from the NCI-COG Pediatric MATCH trial arm B (APEC1621B).

10007 Background: The NCI-Children’s Oncology Group (COG) Pediatric Molecular Analysis for Therapy Choice (MATCH) trial assigns patients aged 1-21 years with relapsed or refractory solid tumors, CNS tumors, lymphomas, and histiocytic disorders to phase 2 treatment arms of molecularly-targeted therapies based on genetic alterations in their tumor. Arm B (APEC1621B) evaluated the orally bioavailable FGFR inhibitor erdafitinib in patients whose tumors harbored activating alterations in FGFR 1/2/3/4. Methods: Patients received oral erdafitinib 4.7 mg/m2/dose (Max dose: 8 mg) daily for 28-day cycles until disease progression or drug related dose limiting toxicity (Max 26 cycles). Disease assessments were performed every 2-3 cycles. The primary endpoint was objective response rate; secondary endpoints included progression free survival (PFS). Results: A total of 20 evaluable patients (median age: 15 yrs) were enrolled from June 2018 to July 2022 in this first study of erdafitinib in pediatric patients. The most common diagnosis (n = 17) was glioma or glioneuronal tumor (11 low grade and 6 high grade). Three patients had non-CNS solid tumors (1 each with neuroblastoma, osteosarcoma, and rhabdomyosarcoma). FGFR alterations detected consisted of activating hotspot mutations in FGFR1 (n = 16), FGFR2 (n = 1), FGFR4 (n = 1), and FGFR1 fusions (n = 2; FGFR1: TACC1, FGFR1: TACC3). Partial responses were confirmed by central review in 2/20 patients (10%, 90%CI: 3.4%, 26.2%), both with low grade gliomas or glioneuronal tumors (LGG, LGGNT) and FGFR1 hotspot mutations (K687E and N577K). A best response of stable disease (SD) was observed in 6 additional patients with gliomas with a median duration of SD of 6.5 cycles. Six-month PFS was 45% (95% CI: 23.1%, 64.7%) and six-month OS was 89.7% (95%CI: 64.8%, 97.3%). Treatment related adverse events included those previously reported with FGFR inhibitors including hyperphosphatemia and nail changes or infections. Grade 1 vision changes were also reported. There was one Grade 3 spinal cord compression and one Grade 4 intracranial hemorrhage. Conclusions: Erdafitinib was generally well tolerated in this heavily pre-treated cohort of children with relapsed tumors harboring FGFR alterations. Partial responses or stable disease were observed in the majority (6/11, 54%) of patients with FGFR1-mutant low grade gliomas or glioneuronal tumors, suggesting the possible benefit of FGFR inhibitor therapy for these patients. Further studies would be necessary to more thoroughly evaluate the activity of erdafitinib for patients with other histologies and FGFR alteration types or combination approaches that include other targeted and/or cytotoxic agents. Clinical trial information: NCT03210714 .

Mucocutaneous presentations of consultant critical and non-critical cases of admitted COVID-19 patients, outpatients, and vaccine-associated dermatoses: a clinical atlas and a large original study of two general COVID-19 centers from Iran.

Rapid and proper diagnosis of mucocutaneous presentations of COVID-19 which in many cases are representing internal organ damage is a key way to better approach these patients, and it could be even lifesaving. In this original study, we reported consultant critical and non-critical cases of admitted COVID-19 patients and some interesting outpatient cases for 14 months, and some newly encountered vaccine-associated dermatoses. We presented 121 cases divided into 12 categories; all had full multi-aspects photographs attached as an atlas to a Supplementary File. These categories were:1- Generalized papulopustular eruptions (3 patients), 2- Erythroderma (4 patients), 3- Maculopapular lesions(16 patients), 4- Mucosal lesions (8 patients), 5- Urticarial lesions and angioedema (16 patients), 6- Vascular injuries (22 patients), 7- Vesiculobullous lesions (12 patients), 8- The specific new onset of mucocutaneous presentations or aggravation of any especial previous dermatoses (9 patients), 9- Nail changes (3 patients), 10- Hair loss (2 patients), 11- Non-specific mucocutaneous problems (16 patients) and 12-Vaccine-associated dermatoses (10 patients).In the pandemic, if we countered with extensive mucocutaneous lesions with vascular components or vesiculobullous erosive lesions in association with any cutaneous rash that could be an alarming sign of a probable life-threatening systemic event, we would need to approach them as soon as possible.

Dermoscopic Features of Psoriatic Nails and Their Correlation to Disease Severity.

Nail psoriasis is a challenging chronic condition affecting patients functionally and psychologically. Nail involvement is observed in 15-80% of psoriatic patients with occasional presence of isolated nail psoriasis. To evaluate dermoscopic features of nail psoriasis and correlate them clinically. The study included fifty subjects with nail psoriasis. Psoriasis skin and nail severity was evaluated using psoriasis area severity index (PASI) and nail psoriasis severity index (NAPSI). Dermoscopy of the nails (onychoscopy) was performed, and features were recorded and analyzed. The most common clinical and dermoscopic findings were pitting (86%) and onycholysis (82%). Among all dermoscopic features of nail psoriasis, only longitudinal striations and subungual hyperkeratosis were significantly higher in patients with moderate to severe psoriasis than in patients with mild psoriasis (P=0.028; P=0.042, respectively). PASI scores correlated positively but none significantly with NAPSI scores (r = 0.132, P=0.360), and similarly, no significant correlation was observed between the duration of psoriasis and the dermoscopic NAPSI (r = 0.022, P=0.879). Dermoscopy can serve as a useful tool for early diagnosis of psoriatic nail changes not always visible to the naked eye and is a non-invasive easy-to-use confirmatory tool for nail changes in psoriatic disease or in isolated nail involvement.

A Study of Nailfold Capillaroscopy in Different Morphological Variants of Psoriasis in a Tertiary Care Hospital

Abstract Background: Psoriasis is a chronic skin disease. Nailfold capillaroscopy (NFC) is a method to analyze nail changes in autoimmune disorders and is gaining popularity as a bedside tool for studying the capillaroscopic changes of psoriasis. Aim: The aim was to assess the nail fold capillaroscopic parameters in controls versus psoriasis patients and to assess the nail fold capillaroscopic parameters in various morphological types of psoriasis patients. Materials and Methods: A cross-sectional observational study of NFC was conducted on 100 patients and 100 controls who attended the outpatient department of dermatology of a tertiary care hospital in Central India from June 2022 to October 2022. Nail fold of little fingers of both hands was studied by the Universal Serial Bus (USB) Digital Microscope and photographed. Results: This study was conducted on 100 patients of psoriasis and 100 controls. The mean age of patients was 50.77 ± 11.33 years with a male-to-female ratio of 1.27. The most common type of psoriasis was chronic plaque psoriasis, and erythrodermic psoriasis was the least common. Majority of 51 patients were having a Psoriasis Area and Severity Index score of &gt;3–15 suggestive of moderate psoriasis. Capillary morphology in 89% of psoriasis cases was inverse U-shape, while remaining 11% had bizarre morphology. There was a significant difference (P &lt; 0.011) in capillary density between psoriasis patients and controls. Only 6% of patients had irregular and haphazard capillary distribution, compared to 94% of patients who had regular and parallel capillary distribution. Other parameters of NFC such as subpapillary plexus, ramified/bushy capillaries, and hemorrhage were also studied. Conclusion: Psoriasis is characterized by microvascular abnormalities which can be studied by a novel technique of nailfold capillaroscopy.

Frequency and Effect of Cutaneous Manifestations on Quality of Life in Patients with End-Stage Renal Disease Undergoing Hemodialysis.

To determine the frequency of skin manifestations found in end-stage renal disease (ESRD) patients undergoing dialysis, while assessing their effect on the quality of lives of the same patients. Descriptive cross-sectional study. Place and Duration of the Study: Benazir Bhutto Hospital, Holy Family Hospital, and Hussain Lakhani Hospital, from 12th December 2021 to 13th October 2022. Seventy-three Patients undergoing hemodialysis were enrolled in the study. Skin manifestations were defined as "cutaneous signs and symptoms related to ESRD unrelated to the symptoms resulting from any primary dermatological disorder or other systemic diseases". Data on the skin manifestations of their disease and their effect on patients' quality of life were collected by using a 2-part questionnaire. The first part consisted of demographic details along with the type of skin disorders faced by the patient and the second part of the questionnaire comprised of the dermatology life quality index (DLQI). The data were entered and analysed using the statistical package for social sciences (SPSS) version 23.0. Xerosis and pruritus were most commonly reported (83.7%), followed by nail changes (18.6%) and skin discolouration (16.3%). The median duration of dialysis was 36 (1-180) months and there was no significant increase in skin symptoms with the increase in the duration of dialysis (p=0.082). The median DLQI score was 3 (range:0-10) A significantly higher number of females (n=14) reported associated mental discomfort with their skin symptoms of pruritis as compared to males (n=5, p=0.008). Cutaneous manifestations have variable effects on the quality of life of ESRD patients. Adopting a multidisciplinary approach early in the management may help to minimise the mental discomfort of these patients and bring an improvement in their quality of life. End-stage renal disease (ESRD), Hemodialysis, Skin manifestations, Pruritus, Quality of life.

Evaluation of Nail Psoriasis: Delving into Some Useful Scores

Introduction: Several scoring systems have been established for nail psoriasis severity assessment. However, there is a lack of consensus on these tools. Objectives: To evaluate the nail changes in psoriasis patients and to correlate the severity of nail findings by Nail Psoriasis Severity Index (NAPSI) and modified NAPSI (m-NAPSI) scores. Material and Methods: Three hundred and seventy consecutive adult psoriatic patients were studied to describe the pattern of nail changes with its frequency and the findings were compared with data collected on age-and gender-matched 150 control subjects to determine which of the observed features are psoriasis disease-specific nail changes. The nail severity was assessed by NAPSI and m-NAPSI scores and correlated. Results: Nail signs included in NAPSI were more commonly seen in psoriatic patients as compared to healthy controls and it was statistically significant. Beau’s line was also found more in patients as compared to controls. None of the cases and controls had red spots in the lunula, and leukonychia was comparatively common in the controls. There was a strong and significant correlation found between NAPSI and m-NAPSI (r=0.916). Fingernails had a stronger correlation as compared to toenails between NAPSI and m-NAPSI (r=0.920 vs. r=0.877; p&lt;0.001). Conclusions: m-NAPSI may be considered to measure the degree of nail changes however leukonychia and a red spot in the lunula could be removed and Beau’s line could be added as further modification of m-NAPSI.