Cutaneous Symptoms Research Articles

Multiple Myeloma-Associated Leukocytoclastic Vasculitis Mimicking Autoimmune Vasculitis.

A 52-year-old man was admitted because of complaints of swelling and skin rashes on the extremities, fever, and arthralgia. Physical examination revealed bilateral ankle and wrist joint tenderness and livedo racemosa and purpura on the lower extremities (Figure, A). Cutaneous lesion biopsy showed leukocytoclastic vasculitis (Figure, B). Laboratory testing revealed a hemoglobin level of 5.6 g/dL, mildly decreased glomerular filtration rate, and an immunoglobulin G level of 3,936 mg/dL. Serum and urine immunofixation electrophoresis showed monoclonal kappa light chains. Bone marrow biopsy revealed proliferation of monoclonal plasma cells (Figure, C). Finally, the patient was diagnosed with leukocytoclastic vasculitis as the initial presentation of multiple myeloma. Cutaneous symptoms resolved within 6 weeks following treatment with daratumumab, lenalidomide, and dexamethasone (Figure, D). Although leukocytoclastic vasculitis is commonly associated with immune-mediated systemic vasculitis, cutaneous leukocytoclastic vasculitis may be observed in case of malignancy. Monoclonal gammopathies should be considered in cases of leukocytoclastic vasculitis associated with renal and blood disorders.Differential diagnosis: immunoglobulin A vasculitis, microscopic polyarteritis, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis.

Dermatitis Herpetiformis: Clinical Exploration, Underlying Pathogenesis and Therapeutic Advances

Dermatitis herpetiformis (DH) emerges as a chronic, immune-mediated, systemic skin pathology closely linked to celiac disease (CD). Characterized by polymorphous and pruritic cutaneous manifestations, DH presents a unique clinical and diagnostic challenge. In this article, we seek to comprehensively delineate the clinical, histologic, and immunologic dimensions of DH, with particular emphasis on its etiopathogenic association with CD. The underlying autoimmune cascade involving the interaction between immunoglobulin A (IgA) and tissue transglutaminase is examined, with crucial implications in the pathogenesis of DH. As understanding of immunologic mechanisms expands, the diverse spectrum of clinical presentations of DH is explored, highlighting the variability in skin lesion morphology and its correlation with histologic findings. In the therapeutic arena, conventional and emerging approaches are reviewed, ranging from dapsone-based drug therapy to nutritional and pharmacologic interventions aimed at suppressing the abnormal immune response. As research advances, it is essential to further the early diagnosis and integrated management of DH, addressing both cutaneous symptoms and systemic implications, to improve patients' quality of life and provide comprehensive and insightful clinical care.

The etiology, risk factors, and clinical features of anaphylaxis: The single-center retrospective cohort study of the tertiary university hospital

Background/Aim: Anaphylaxis presents in multiple ways, making its diagnosis challenging. Delayed diagnosis can lead to a postponement in administering crucial adrenaline treatment. The prevalence of anaphylaxis varies by geographical region and gender. However, there has been no comprehensive regional analysis of anaphylaxis data within our country. Despite an increasing incidence, our understanding of anaphylaxis etiology, risk factors, and clinical features remains limited, particularly within our nation. This study aims to assess the frequency, etiology, risk factors, and clinical findings of anaphylaxis among patients seen at the allergy clinic of a tertiary university hospital. Additionally, it seeks to compare regional data with existing literature. Methods: This retrospective cohort study reviewed the medical records of 8,295 patients who visited the allergy outpatient clinic at Sivas Cumhuriyet University Hospital between July 2, 2018, and December 10, 2019. The hospital’s data system retrospectively analyzed records using the ICD code T78.2 (anaphylaxis). Only cases where patients were prescribed an adrenaline auto-injector were included. The study evaluated anaphylaxis frequency, etiologies, demographics, and clinical features. Results: The study identified 77 patients (n=77) with a mean age of 40.29 (3.77) years, consisting of 47 females and 30 males. The frequency of anaphylaxis among allergy outpatient admissions was less than 1% (0.009%). Single-type atopic diseases included venom allergy (23%), drug allergy (14%), inhalant allergens (n=6), food allergens (n=4), and skin allergic diseases (n=3). Multiple allergic diseases were present in 40% (n=31) of cases. Prick tests were performed on 56 (72%) patients, with 25 (44%) yielding negative results. Among positive prick test cases, venom was the main cause of anaphylaxis (82%), while drug allergy was more prevalent (68.2%) among negative test results (P=0.016). Inhalant allergen sensitivity and allergen polisensitivity did not significantly influence the anaphylaxis cause (P<0.001). Causes of anaphylaxis included drug allergy (47%), venom allergy (31%), food allergens (16%), food-dependent exercise-induced reactions (n=2), idiopathic cases (n=2), and cold urticaria (n=1). Non-steroidal anti-inflammatory drugs (NSAIDs) (44%) and beta-lactams (10%) were the primary culprits. In cases where neither drugs nor venom were involved, food allergies were the cause (P<0.001). With venom allergy, the cause was venom, and without venom, drug allergy was the cause (P<0.001). Female patients showed significantly higher drug- and food-related anaphylaxis rates than males (P=0.032 and P=0.042, respectively). History of Apis mellifera-related anaphylaxis was significantly more common than Vespula vulgaris-related cases (P=0.028). Anaphylaxis severity included grade 2 (30%), grade 3 (48%), and grade 4 (12%) reactions. Recurrent anaphylaxis episodes occurred in 55% (n=42) of patients. Initial hospital administrations involved epinephrine injections in only 25% (n=19) of cases. Cutaneous symptoms were present in 94%, respiratory symptoms in 88%, cardiovascular symptoms in 63%, neurological symptoms in 57%, and gastrointestinal symptoms in 12% of patients. Conclusion: This study identified drug allergy as the leading cause of anaphylaxis in the examined cases. Preventable factors contributing to drug-induced anaphylaxis included insufficient patient and physician knowledge and widespread over-the-counter drug use without medical consultation. Despite 55% of patients experiencing recurrent attacks, only a quarter received epinephrine administration. These findings emphasize the need to educate patients with recurrent anaphylaxis about avoidance strategies and to enhance healthcare providers’ understanding of anaphylaxis treatment.

O03 Langerhans cell histiocytosis: case series of presentations to dermatology in the BRAF era

Abstract Langerhans cell histiocytosis (LCH) is a rare disease driven by MAPK pathway mutations. This condition is on a spectrum, from single-system to life-threatening multisystem disease. Identification of the BRAF mutation is leading to improved understanding and targeted therapies. However, there is limited information on genetic status, phenotype and dermatological features. Fourteen patients were seen in the Paediatric Dermatology department in period 2015–2022: 11 males and 3 females; 9 patients had multisystem LCH; 8 patients had cutaneous manifestations of LCH; and 4 had limited cutaneous LCH. Six patients were diagnosed in the department and typically had multiple incorrect diagnoses and treatments prior to review. Clinical features varied from widespread skin involvement, scalp/groin intertrigo, flexural ulcerations and telangiectatic papules. Eight had LCH diagnosed in other tissues by oncology and referral advice on cutaneous symptoms were sought. Most patients have required input from dermatology throughout treatment. Biopsies were taken when unclear diagnosis and/or pathology would influence treatment. Several consultations revealed benign pathologies including keratosis pilaris and dermatitis requiring topical treatment advice only. All five patients with BRAF v600 mutation had multisystem disease including diabetes insipidus, adrenal insufficiency, gut and bone lesions. Two patients with severe LCH and BRAF mutations are being treated successfully with dabrafenib. Our series demonstrates the important role of dermatology in the diagnosis and management of LCH. This series confirms clinical heterogeneity and an association of BRAF V600E mutation with multiorgan disease. Ongoing work should expand our knowledge of the relationship of genetic alterations with clinical phenotypes and comorbidities.

Assessment of YKL-40 as a Potential Biomarker of the Disease Severity in Psoriatic Patients with or without Psoriatic Arthritis Treated with Methotrexate

Abstract Introduction Psoriasis is an inflammatory disease of a complex pathogenesis with its typical cutaneous symptoms are papules& plaques covered with silvery scales and arthritis is one of its most common complications. The biological role of chitinase-3-like protein 1 (YKL-40) remains unknown. It is suggested that this protein takes part in processes such as proliferation, inflammation, angiogenesis and tissue remodelling. Aim To investigate the serum level of YKL-40 in psoriatic patients with or without psoriatic arthritis and to study the effect of treatment with methotrexate on the serum levels of YKL-40. Material and methods The study was performed on 40 patients with psoriasis vulgaris with or without psoriatic arthritis. All patients showed the clinical signs of psoriasis vulgaris with or without psoriatic arthritis. The diagnosis of cutaneous psoriasis was made clinically based on the characteristic lesions and scored according to Psoriasis Area and Severity Index (PASI) and, additionally, the Body Surface Area (BSA). Psoriatic arthritis patients had fulfilled the diagnostic criteria defined by the classification of Psoriatic Arthritis (CASPAR) criteria. The severity of psoriatic arthritis was assessed using DAPSA Score. Blood samples were taken to measure the serum concentration of YKL-40. Results YKL-40 serum levels were significantly higher in patients with psoriatic arthritis, compared to patients with psoriasis only & a significant reduction inYKL-40 levels in both groups, psoriasis patients with and without psoriatic arthritis, after receiving methotrexate treatment. There was a positive correlation between serum YKL-40 and PASI. Conclusions YKL-40 can be a useful biomarker for assessment of the disease severity in psoriatic patients with or without psoriatic arthritis. It also detects the efficacy of treatment with methotrexate.

Cutaneous microvascular occlusion syndrome as the first manifestation of catastrophic lupus-associated antiphospholipid antibody syndrome: a case report

BackgroundAntiphospholipid syndrome (APS), defined by thrombotic events or obstetric complications in the presence of persistently high antiphospholipid antibodies, is characterized by a wide variety of clinical presentations and the effects of vascular occlusion can impact almost any organ system or tissue. Since adult-onset APS classification criteria are not well verified in pediatrics (where pregnancy-related problems are rare), estimating childhood prevalence is challenging. Stroke and pulmonary embolism are thromboembolic events occurring in children that can cause considerable long-term morbidity. Children with APS are more prone to recurrent thromboembolism than adults. Cutaneous symptoms are prominent and typically represent the first clue of APS. Although dermatologic findings are exceedingly heterogeneous, it is essential to consider which dermatological symptoms justify the investigation of antiphospholipid syndrome and the required further management.Case presentationWe describe a seven-year-old Iranian boy with retiform purpura and acral cutaneous ischemic lesions as the first clinical presentation of antiphospholipid syndrome in the setting of systemic lupus erythematous.ConclusionAPS in pediatrics, is associated with a variety of neurologic, dermatologic, and hematologic symptoms. Therefore, it is essential for pediatricians to be aware of the rare appearance of Catastrophic APS as an initial indication of APS.

Treatment of pediatric ocular rosacea: A systematic review

BackgroundOcular rosacea is an underdiagnosed form of rosacea that may occur without typical cutaneous signs of rosacea. Manifestations include blepharitis, lid margin telangiectasias, and scleritis. A systematic comparison of treatment options for ocular rosacea in children is lacking. MethodsA systematic review was conducted according to the PRISMA guidelines on treatment for pediatric ocular rosacea. ResultsEleven articles were included, representing 135 patients with a mean age of 5 years, of whom 69% (n = 75/108) were female. 55% (n = 55/99) exhibited ocular symptoms prior to cutaneous symptoms. Most patients (83%, n = 34/41) experienced a delay in diagnosis (mean 27 months, range 2–120 months). Doxycycline was the most frequently reported treatment (25%, n = 33/135). A complete response was achieved in 33% of patients treated with doxycycline (n = 10/30), while 53% (n = 16/30) achieved a partial response. Erythromycin was used in 20% of cases (n = 26/135), with a complete response in 58% (n = 15/26) and partial response in 42% (n = 11/26). Metronidazole was used in 14% of patients (n = 19/135), with a complete response being reported in 79% (n = 15/19) and partial response in 21% (n = 4/19). ConclusionSystemic antibiotics, led by doxycycline, were the most commonly reported treatment modalities for pediatric ocular rosacea. Increased awareness of ocular rosacea in this population is crucial for earlier diagnosis.

Clinical characteristics of children with IgE-mediated cow's milk protein allergy

Objective: To analyze the clinical characteristics of children with IgE-mediated cow's milk protein allergy (CMPA) and provide a basis for disease management and prevention. Methods: A cross-sectional study was conducted to analyze 142 children aged 0-12 years who were diagnosed with IgE-mediated CMPA in Capital Institute of Pediatrics Affiliated Children's Hospital from 2020 to 2022. There were 79 males (55.6%) and 63 females (44.4%), with an average age of 14 (8, 27) months. 61 cases (43.0%) were in the <1-year-old group, 54 cases (38.0%) in the 1-3-year-old group, and 27 cases (19.0%) in the >3-year-old group. Data on demographic data, clinical manifestations, mean wheel diameter of skin prick test and serum specific IgE level were collected. The serum cow's milk protein sIgE and component sIgE were measured by ImmunoCAP fully automated system of fluorescence enzyme-linked immunosorbent assay, and statistically analyzed using chi-square test, nonparametric tests, correlation. Results: Cutaneous symptoms were the first and most frequent in 142 children (97.9%, 139/142 cases), followed by digestive (29.6%, 42/142 cases) and respiratory symptoms (27.5%, 39/142 cases).The proportion of children with respiratory symptoms after consuming cow's milk was significantly higher in the>3 years age group than those in the infant and toddler groups(66.7% vs 19.7%,χ2=18.396,P<0.01;66.7% vs 16.7%,χ2=20.250,P<0.01), and the symptoms involving ≥3 systems were also significantly higher than those in the other two groups(37.0% vs 13.1%,χ2=6.597,P<0.05;37.0% vs 7.4%,χ2=12.120,P<0.01). The average cow's milk SPT diameter and serum sIgE levels in the>3 years age group were significantly higher than those in the infant and toddler groups (Z=-4.682, P<0.01; Z=-3.498, P<0.01); (Z=-4.463, P<0.01; Z=-6.463, P<0.01). The most common cow's milk component protein were β-lactoglobulin(65.1%,56/86 cases) and casein (57.0%, 49/86 cases). Multiple-sensitization rate of the patients were 54.9%. Egg white (43.7%, 62/142 cases) was the most common co-sensitization food allergen while mold (12.7%, 18/142 cases) and weed pollen (12.7%, 18/142 cases) were the main co-sensitization aeroallergens. The proportion of multiple-sensitization to aeroallergens in the children group was the highest (51.9%, 14/27 cases), followed by the toddler group (29.6%, 16/54 cases), and the infant group was the least (3.3%, 2/61 cases). There was a significant difference among these three groups (χ2=7.476, P<0.05). Conclusion: Skin and mucosal symptoms are the most common in CMPA patients. The proportion of respiratory symptoms and multisystem involvement increased with age as well as the wheal diameter in skin test and serum sIgE level elevated. CMPA patients older than 3 years had the highest proportion of aeroallergen sensitization and airway allergic diseases.

A nerve-to-implant distance as a novel predictor for lateral femoral cutaneous nerve injuries after anterior subcutaneous pelvic internal fixation.

Although anterior subcutaneous pelvic internal fixation is a valuable tool for the reduction and fixation of unstable pelvic ring injuries, lateral femoral cutaneous nerve irritation by the implant is the most common complication. This study aimed to investigate the association between the nerve-to-implant distance and the postoperative lateral femoral cutaneous nerve symptom. Patients who underwent anterior subcutaneous pelvic internal fixation between 2016 and 2019 were retrospectively analyzed. Lateral femoral cutaneous nerve status was defined as follows: not identified, nerve-to-implant distance <13 mm, and ≥13 mm. The proportion of patients who experienced postoperative nerve disorders was compared using the nerve status. Nerve-to-implant distances were compared using the presence or absence of postoperative lateral femoral cutaneous nerve disorders. The predictive value of a nerve-to-implant distance of 13 mm for postoperative nerve disorders was assessed. Overall, 26 lateral femoral cutaneous nerves were included. Ten patients had postoperative nerve disorders, of which seven had an nerve-to-implant distance <13 mm, while the other three occurred in patients whose nerves were not identified. A nerve-to-implant distance ≥13 mm was significantly associated with a decreased risk of postoperative nerve disorder compared to a nerve-to-implant distance <13 mm (p = 0.017). A nerve-to-implant distance ≥13 mm had a perfect sensitivity (100%) and modest specificity (58.3%). Nerve-to-implant distance was ≥13 mm. Nerve disorders were frequently observed when the nerve-to-implant distance was <13 mm or the nerve was not identified intraoperatively. Efforts to identify the lateral femoral cutaneous nerve may be useful to avoid internal fixation-related nerve disorders.

A Clinical Qualification Protocol Highlights Overlapping Genomic Influences and Neuro-Autonomic Mechanisms in Ehlers-Danlos and Long COVID-19 Syndromes.

A substantial fraction of the 15% with double-jointedness or hypermobility have the traditionally ascertained joint-skeletal, cutaneous, and cardiovascular symptoms of connective tissue dysplasia and its particular manifestation as Ehlers-Danlos syndrome (EDS). The holistic ascertainment of 120 findings in 1261 EDS patients added neuro-autonomic symptoms like headaches, muscle weakness, brain fog, chronic fatigue, dyspnea, and bowel irregularity to those of arthralgia and skin laxity, 15 of these symptoms shared with those of post-infectious SARS-CoV-2 (long COVID-19). Underlying articulo-autonomic mechanisms guided a clinical qualification protocol that qualified DNA variants in 317 genes as having diagnostic utility for EDS, six of them identical (F2-LIFR-NLRP3-STAT1-T1CAM1-TNFRSF13B) and eighteen similar to those modifying COVID-19 severity/EDS, including ADAMTS13/ADAMTS2-C3/C1R-IKBKG/IKBKAP-PIK3C3/PIK3R1-POLD4/POLG-TMPRSS2/TMPRSS6-WNT3/WNT10A. Also, contributing to EDS and COVID-19 severity were forty and three genes, respectively, impacting mitochondrial functions as well as parts of an overlapping gene network, or entome, that are hypothesized to mediate the cognitive-behavioral, neuro-autonomic, and immune-inflammatory alterations of connective tissue in these conditions. The further characterization of long COVID-19 natural history and genetic predisposition will be necessary before these parallels to EDS can be carefully delineated and translated into therapies.

Diagnostic Evaluation of Hypersensitivity Reactions to Antibiotics in a Large Cohort of Mastocytosis Patients.

Anaphylactic reactions are a well-known feature of mastocytosis, particularly in relation to hymenoptera venom stings. Although data on the frequency of drug hypersensitivity reactions is limited in mastocytosis, it is hypothesized that these patients may be predisposed to hypersensitivity reactions to certain drugs, including antibiotics. Nevertheless, this issue has not been systematically investigated. Thus, we investigate the prevalence and clinical features of hypersensitivity reactions to antibiotics (HRA) in mastocytosis. A 15-year retrospective study was conducted among 239 (≥18 years old) consecutive mastocytosis patients who were investigated in our center. All patients underwent a thorough allergy work-up, where self-reported reactions were individually evaluated by an allergist. Overall, 34 patients (14.2%) were deemed to have HRA. Most patients reacted with cutaneous symptoms (74%), and anaphylaxis was rare, confirmed only in two of 34 patients (0.8%). Beta-lactams were the most common elicitors (63%). There were no differences in age, gender, atopic status and tryptase levels between mastocytosis patients with and without antibiotic hypersensitivity. The present study indicates a similar prevalence of HRA in mastocytosis compared to those of the general population. Moreover, antibiotics appear to be rare elicitors of anaphylaxis in these patients. Hence, our results suggest that mastocytosis patients without a history of HRA may be treated with these drugs without special precautions.

BG07 VEXAS syndrome without the V: a New Zealand case report

Abstract VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is an autoinflammatory condition first described in 2020, often with cutaneous features. Effective treatments are yet to be established. We describe the case of a 60-year-old man who presented with rapidly enlarging cutaneous lesions to the face and neck, polychondritis, myalgia, arthralgia and fevers over a 2-month period. Skin biopsy showed a neutrophilic dermatosis with a differential diagnosis of seronegative rheumatoid neutrophilic dermatosis and atypical Sweet syndrome. His cutaneous complaints improved following treatment with prednisone 30 mg. His cutaneous symptoms relapsed on attempts to wean prednisone to &amp;lt; 20 mg. He then developed macrocytic anaemia and transient thrombocytopenia. He was then hospitalized and treated for community-acquired pneumonia. Bone marrow biopsy was normal and without evidence of vacuoles. The patient developed progressive fatigue and shortness of breath, with transient stenosing tenosynovitis and Raynaud symptoms over 3 years. He was most burdened by soft tissue and joint swelling, with muscular stiffness and pain particularly affecting his hands and feet. Methotrexate was partially effective in managing myalgia. Dapsone, hydroxy­chloroquine, mycophenolate, cyclophosphamide and colchicine were tried without benefit. Following a regional dermatology multidisciplinary team meeting, the patient was screened for VEXAS syndrome. Blood and bone marrow genetic testing found a UBA1 mutation load which is 50% of total DNA, confirming a diagnosis of VEXAS syndrome and the highest found in a New Zealand laboratory to date. Bone marrow images were re-examined, with no vacuolization noted. Our patient had a heterozygous UBA1C (Met41Leu) variant present in 50% of his blood and bone marrow DNA—to our knowledge the highest VEXAS genetic mutation seen in a New Zealand laboratory so far. Our case illustrates that, in patients with clinical evidence of VEXAS syndrome, the absence of vacuolization on bone marrow biopsy should not be a criterion for exclusion.

Systemic lupus erythematosus presenting as lupus erythematosus tumidus and lupus nephritis: a case report

BackgroundLupus nephritis and lupus erythematosus tumidus (LET) are uncommon manifestations of systemic lupus erythematosus (SLE), and their coexistence as the initial presentation of SLE is exceedingly rare. Here, we report such a case, emphasizing the diagnostic challenges and therapeutic implications of this unusual association.Case reportA 38-year-old North African woman presented in Nephrology department with a history of lower extremity edema, fatigue, and weight loss of 3 kg in 4 weeks. Physical examination revealed LET lesions on the chest and the Neck. Laboratory investigations showed lymphopenia, low C3 and C4 complement levels, positive antinuclear antibodies, anti-dsDNA antibodies, and anti-SSA/Ro antibodies. Renal function tests showed normal serum creatinine and nephrotic proteinuria. Renal biopsy revealed Class V lupus nephritis. Skin biopsy confirmed the diagnosis of LET, with the presence of lymphohistiocytic infiltrates and dermal mucin. The patient was diagnosed with SLE based on the 2019 EULAR/ACR criteria and treated with prednisone (1 mg/kg/day) and hydroxychloroquine. She showed significant improvement in her cutaneous and renal symptoms at 6 and 12 months follow-up.ConclusionThe rarity of the coexistence of LET and lupus nephritis as the initial manifestation of SLE, especially in the North African population, underscores the need for further research to elucidate the immunopathogenic mechanisms and prognostic factors associated with this association.

Adverse events in children and adolescents undergoing allergen immunotherapy for respiratory allergies-Report from the Allergen Immunotherapy Adverse Events Registry (ADER), a European Academy of Allergy and Clinical Immunology taskforce.

Although it has been shown that allergen immunotherapy (AIT) is well-tolerated in children, systematic and prospective surveillance of AIT safety in real life settings is needed. The multinational Allergen Immunotherapy Adverse Events Registry (ADER) was designed to address AIT safety in real life clinical practice. Data on children ≤18years old with respiratory allergies undergoing AIT were retrieved. Patient- and AIT-related features were collected and analyzed. The characteristics of adverse events (AE) and risk factors were evaluated. A total of 851 patients, 11.3±3.4years old, with rhinitis only (47.6%); asthma and rhinitis (44.5%); asthma (7.9%), receiving 998 AIT courses were analyzed. Sublingual immunotherapy (SLIT) accounted for 51% of the courses. In 84.5% of patients only one AIT treatment was prescribed. Pollen was the most frequent sensitizer (57.1%), followed by mites (53.4%), molds (18.2%) and epithelia (16.7%). Local and systemic AEs were reported in 85 patients (9.9%). Most AEs (83.1%) were mild and occurred in <30min (87%). Respiratory and cutaneous symptoms were more frequent. Only 4 patients (0.47%) had severe AE (none after 6weeks of maintenance). The risk of AE was higher in patients undergoing SCIT. AIT is safe and well tolerated in children and adolescents with respiratory allergies in real-life clinical practice. Though SCIT is more prone to AE compared to SLIT, overall severe reactions are rare and occur during build-up and early maintenance.

Machine learning prediction of patient-reported outcomes associated with systemic therapy from medical records.

e13564 Background: Measures of patient-reported outcome (PRO) are important metrics in cancer care. However, PROs are poorly captured in routine practice, and data collection may incur undue burden on patients. It remains unclear if electronic medical records (EMR) contain sufficient information to dynamically monitor PROs in patients undergoing intensive systemic therapy. Methods: Over a 6 week period, consecutive patients attending an outpatient chemotherapy unit completed two questionnaires (EORTC QLQ-C30 and NCI PRO-CTCAE) at each visit. The corresponding EMR, authored by oncologists and allied health providers, were extracted up to 4 weeks prior to the questionnaire date. Random forest algorithm was used to predict each dichotomized PRO class using bag-of- n-grams extracted from the text. The classification performance was assessed by 8-fold cross-validation, using the area under receiver operating characteristic curve (AUC) as the metric. This study is part of the ePERSIST (electronic phenotyping, event retrieval, and case summarisation in solid tumours) project, exploring methods for extracting structured variables from EMR to support oncology research. Results: 115 patients (157 visits to the chemotherapy unit) completed up to 6 sets of questionnaires each. Overall, the median quality-of-life (QOL) score of QLQ-C30, QL2, was 67 (IQR 50 to 83). Machine learning predicted low QL2 score with mean AUC of 0.710 (95% CI 0.630 to 0.791). At the Bonferroni threshold of 0.00025, machine learning significantly predicted several components of QLQ-C30: PF2 (mean AUC 0.722, 0.633 to 0.812), AP (0.725, 0.630 to 0.819), CO (0.686, 0.607 to 0.764), FI (0.677, 0.598 to 0.755), PA (0.696, 0.603 to 0.787), FA (0.686, 0.584 to 0.787), and RF2 (0.717, 0.595 to 0.839). Conversely, EMR could only be used to significantly predict 4 of 13 PRO-CTCAE categories: cutaneous (0.745, 0.659 to 0.832), oral (0.734, 0.630 to 0.838), cardiovascular (0.675, 0.593 to 0.758), and gastrointestinal (GI, 0.742, 0.625 to 0.860), or 15 of 79 (19%) of PRO-CTCAE items related to adverse events including cutaneous (radiation burns, hypohidrosis, rash), oral (anorexia, dysphagia, dysgeusia), and GI symptoms (constipation, bloating), and others (anxiety, hoarseness, pain, erectile dysfunction). An exploratory analysis revealed that specific keywords linked to the PRO items were present in only one (11%) QLQ-C30 and 4 (5%) PRO-CTCAE items. Conclusions: The use of machine learning on clinical text predicts QOL and certain patient-reported adverse events with moderate accuracy in individuals undergoing intensive systemic treatment, suggesting its potential utility in syndromic monitoring where PRO is not formally assessed. Given many items cannot be accurately extracted, however, obtaining information directly from patients through questionnaires may still be required because of limited documentation by clinicians.

Refractory alopecia in lupus treated with tofacitinib - a case-based review.

One of the common cutaneous symptoms of systemic lupus erythematosus (SLE) that may have major psychosocial effects in a female is diffuse alopecia. Although Janus kinase inhibitors have shown encouraging results in the treatment of SLE and of alopecia areata in recent studies, tofacitinib in treating refractory alopecia caused by SLE has been rarely documented. The Janus kinases (JAKs) are intracellular tyrosine kinases that play a significant role in the pathophysiology of SLE by participating in a wide range of inflammatory cascades. Here, we reported a 33-year-old SLE patient with long standing (3years) refractory alopecia who took tofacitinib and observed a substantial increase in hair growth. This was sustained at 2-years follow-up even after tapering off glucocorticoids completely. In addition, we reviewed the literature to look for further evidence to support the use of JAK inhibitors for alopecia in SLE.

Dermatoscopic and Dermatopathologic Features of a 3-Year-Old Buried Polypropylene Suture

Cutaneous foreign bodies are a well-known cause of delayed wound healing and complications such as abscesses, fistula formation, and secondary infections. Polypropylene sutures are widely utilized in cutaneous surgery because they easily travel through tissues while eliciting minimal tissue reactions. Despite these advantages, retained polypropylene sutures can cause complications. The authors report a case of a retained polypropylene suture that remained buried after a total excision 3 years prior. It started to cause cutaneous symptoms when the patient began exercising 1 week prior to presentation. The authors also examine the dermatoscopic and dermatopathologic features and other complications related to retained polypropylene sutures that have been reported in the literature.

Therapeutic effects and mechanisms of Ku-Gan formula on atopic dermatitis: A pilot clinical study and modular pharmacology analysis with animal validation.

Atopic dermatitis (AD) is a persistent, recurrent inflammatory skin disorder with a rapid upward trend worldwide. The first-line treatment for AD consists of topical medicines such as topical corticosteroids (TCSs). However, long-term use of conventional topical medicine results in side effects and recurrence, presenting therapeutic challenges for the management of AD. Ku-Gan formula (KG) has been extensively used to treat skin diseases since the Song dynasty. In particular, topical administration of the KG alleviates the cutaneous symptoms of AD and reduces recurrence rates with a good safety profile; however, the mechanisms of the KG's action remain unknown. The current study aimed to evaluate the efficacy and safety of KG in AD patients and to investigate the molecular mechanisms that underlie the efficacy of KG in the treatment of AD. A single-arm prospective pilot study with historical controls was conducted. This study evaluated 11 patients with mild to moderate AD, who underwent topical KG treatment. The primary outcome was the change in local eczema area and severity index (EASI) scores. The secondary outcomes included the recurrence rate and safety. The recurrence rate were compared to those of a matched historical control group. Secondly, modular pharmacology analysis was used to elucidate the therapeutic mechanism of KG in AD treatment by identifying the hub genes and kernel pathways. Moreover, we evaluated treatment effects and verified modular pharmacology-based findings using the calcipotriol (MC903)-induced mouse model and bioinformatics analysis. Our clinical pilot study demonstrated that the KG wet wrapping could effectively ameliorate skin lesions in AD patients with a significant drop from 4.18 to 1.63 in local EASI. Compared to the historical controls, KG had a reduced recurrence rate (36%) and a longer median time to relapse (>12 weeks). Modular pharmacology analysis identified the hub genes including IL6, IL1B, VEGFA, STAT3, JUN, TIMP1 and ARG1, and kernel pathway including IL-17 signaling pathway of KG. Pharmacodynamic results suggested that KG ameliorated skin symptoms and demonstrated no less efficacy than halcinonide (HC) in MC903-induced AD-like mice. In addition, KG regulated the mRNA expression of hub genes as well as the related genes involved in IL-17 signaling pathway including Il25, Il17a,Traf3ip2, and Traf6, in skin lesions of AD-like mice. These results showed that KG is a safe and effective topical treatment for AD with low recurrence. In addition, our study identified potential molecular pathways and therapeutic candidate targets of the KG formula, providing evidence for its clinical applicability in AD.

Focal vascular occlusion: A link between livedoid vasculopathy and cutaneous arteritis?

AbstractBackgroundLivedoid vasculopathy (LV) and cutaneous arteritis (CA) are rare cutaneous diseases. A consideration of these two conditions as different entities is debatable, because thrombosis and inflammation are closely related in their pathogenesis mechanisms. Varying reports on the effectiveness of anticoagulant and immunosuppressant therapies in LV and CA also point to diagnostic challenge and overlap in these conditions.ObjectivesWe sought to assess the therapeutic effect of anticoagulation therapy in adult patients with CA, who were recalcitrant to immunosuppressive modalities.MethodsA monocentric retrospective analysis of adult patients who were diagnosed with CA between 2010 and 2018, was carried out.ResultsWe describe 8 cases of long‐term recalcitrant CA, in whom anticoagulation therapy significantly reduced the pain and cutaneous symptoms. All patients had cutaneous symptoms compatible with CA along with clinical features also characteristic of LV, including underlying procoagulative disorders in the majority (87.5%), as confirmed by screening for thrombophilia. We also review current concepts regarding LV and CA, and suggest vascular occlusion is a common link between these two entities.ConclusionsAlthough current knowledge advocates segregating vasculopathy from vasculitis by means of histopathological examination in LV and CA, clinical experience suggests some overlap and common complex underlying pathomechanisms.

Dermatomyositis: A Case Report and Review of a Rare Autoimmune Inflammatory Disease

Dermatomyositis is a chronic progressive autoimmune disease of unknown etiology and rare occurrence worldwide. It is an immunologically mediated idiopathic inflammatory disease in which damage to small blood vessels contributes to injury and inflammation of muscle and skin. Dermatomyositis belongs to a heterogeneous group of three autoimmune rheumatological diseases termed idiopathic inflammatory non-suppurative myopathies, with polymyositis and inclusion body myositis as other component diseases.&#x0D; Dermatomyositis and polymyositis typically show characteristic clinical features of autoimmune inflammatory diseases and association with autoantibodies, HLA-DR genotype, and other autoimmune diseases. While it is known that inflammation plays a significant role in the pathogenesis of these diseases, its role in inclusion body myositis remains unclear. Overlapping forms of inflammatory myopathies that are poorly differentiated and defy a precise classification have also been identified. However, the clinical response to steroids and other immunosuppressive agents may help distinguish the subtypes of inflammatory myopathies from one another.&#x0D; Dermatomyositis is further described as a multisystemic rheumatologic disease presenting with muscular and cutaneous symptoms. It is associated with malignancy in 20 – 25% of patients. Dermatomyositis occurs in children and adults, with a higher incidence observed in women. Diagnosis is usually made from clinical manifestations, elevated blood enzymes, autoantibody testing, electromyography (EMG), and muscle biopsy. The diagnosis, however, remains challenging in third-world countries. There is no known cure for dermatomyositis. However, corticosteroids and other immunosuppressive agents, and intravenous immunoglobins are routinely used for treatment.&#x0D; The index patient in this review is a 22-year-old Afro-Caribbean woman whose presentation met the probable-to-definite criteria for diagnosis based on the Bohan and Peter Classification Criteria. This article is a case report and a detailed review of the autoimmune inflammatory disease known as dermatomyositis that can be used to teach about the disease condition.