UNASSIGNED: Thrombotic thrombocytopenic purpura, particularly its immune-mediated variant (iTTP), necessitates accurate diagnostic approaches for effective management.
UNASSIGNED: To compare a chemiluminescence immunoassay (CLIA) and an enzyme-linked immunosorbent assay (ELISA) for testing ADAMTS-13 activity and detecting anti-ADAMTS-13 autoantibodies (AAbs) in patients with iTTP.
UNASSIGNED: This study involved 31 paired samples from 12 iTTP patients. ADAMTS-13 activity was measured using the HemosIL AcuStar (Instrumentation Laboratory, CLIA) and Technozym (Technoclone) activity assay (ELISA). The presence of AAbs was assessed using Technozym ADAMTS-13-INH assay (ELISA) and HemosIL AcuStar activity (CLIA) within a Bethesda assay following mixing with normal pool plasma. von Willebrand factor (VWF) multimers were analyzed using the HYDRASYS-2 SCAN system and the HYDRAGEL 5- or 11-VW Multimer kits (Sebia). VWF activity levels were measured with the HemosIL AcuStar VWF:GPIbR on the ACL AcuStar Analyzer (IL).
UNASSIGNED: For ADAMTS-13 activity, a strong linear relationship and no bias between CLIA and ELISA were confirmed (slope = 1.01 [0.91, 1.11], intercept = 0.00 [-0.47, 0]). However, significant discrepancies were found in AAb detection during remission phases with ADAMTS-13 activity between 10% and 50%, with CLIA and ELISA showing significant divergence (P < .001, Cohen\'s g = 0.34). Consistently, VWF multimers and activity levels exhibited significantly different values between remission samples with ADAMTS-13 activity below 50% and above 50%. In longitudinal analysis of patients with multiple iTTP relapses, positivity to CLIA appears to precede ELISA in predicting exacerbations.
UNASSIGNED: While CLIA and ELISA might be interchangeable for assessing ADAMTS-13 activity, they are not equivalent for detecting AAbs, particularly in patients in clinical remission with ADAMTS-13 activity between 10% and 50%.

Laser hair removal (LHR) has been established as a safe and efficient method for eliminating unwanted hair. This study aimed to investigate the frequency of LHR complications and assess the contributing factors. During one year, 16,900 patients undergoing LHR therapy were evaluated for complications. For each case, two external controls were selected (matched based on age, sex, Fitzpatrick skin type (FST) III-IV, and the treated anatomical region). To assess the impact of anatomical region on complication occurrence, each patient was used as their internal control if another area was treated during the same session. GEE analysis was used for statistical analysis.The incidence of LHR complications was calculated to be 0.69%. The most common complications were petechia, purpura, and ecchymosis (31.66%) followed by pigmentation changes (20.0%). LHR complications were most commonly observed in the lower limbs (32.0%), face and neck (23.3%), and genitalia and thighs (22.3%), respectively. Possible risk factors were younger age (OR = 0.74, P-value ≤ 0.001), operating LHR in the head and neck (OR = 5.8, P-value = 0.022), utilization of the alexandrite laser (OR = 2.32, P-value = 0.011), and fluence in the Alexandrite laser (OR = 3.47, P-value = 0.003).Overall, the results of this study indicate that LHR is generally a safe method for removing unwanted hair. However, factors such as younger age, treatment of the facial area, and use of the alexandrite laser especially with higher fluence levels in patients with FST III-IV were identified as potential risk factors.

BACKGROUND Pigmented purpuric dermatosis (PPD) is a rare disease that is poorly understood but thought to result from inflammation of the capillaries causing extravasation of erythrocytes into the soft tissue. There are a variety of potential causes, including medications, such as acetaminophen and aspirin, abnormal humoral immunity, and excessive exercise. Although benign, PPD can be bothersome to patients due to associated pruritus, weeping, and poor cosmetic results. Treatment of this lesion is difficult, with no standardized regimen and a tendency for relapse once treatment is discontinued. CASE REPORT This case reports on a 77-year-old man who presented to an outpatient dermatology clinic with bilateral lower extremity edema with associated weeping and erythema for 1 year. A biopsy was conducted and resulted as PPD. He began treatment with excimer laser therapy after conservative and topical treatment options failed, with resolution of symptoms without recurrence for approximately 1 year. CONCLUSIONS PPD is notoriously difficult to treat, and historic treatment options include topical corticosteroids, oral supplements, and immunomodulators, all of which come with a range of adverse effects. However, new literature supports the use of phototherapy to treat PPD, with varying results. Previously implemented options include but are not limited to phototherapy with psoralen plus ultraviolet A, narrow band ultraviolet B, advanced fluorescence technology pulsed light, and fractional non-ablative 1540-nm erbium: glass laser, each with varying degrees of success. This case discusses the successful treatment of recalcitrant PPD with excimer laser therapy and maintenance of remission for approximately 1 year.

BACKGROUND: Pulsed-dye lasers (PDL) are one of the standard therapies for rosacea, but alternatives are needed.
OBJECTIVE: To compare the efficacy and safety of the variable-sequenced, large-spot 532 nm KTP laser to the 595 nm PDL in treating rosacea.
METHODS: A prospective, controlled, evaluator-blinded study. Patients were treated with either a KTP or PDL with 1-3 sessions at intervals of 6-8 weeks. A follow-up visit was scheduled on Week 6 post-treatment. Clinical outcome was assessed by computer-assisted analysis and by patients and two blinded dermatologists. Pain intensity during treatment and adverse events were documented.
RESULTS: Forty-five patients (mean age 51 years) were allocated in a 2:1 ratio to either the KTP or PDL. Erythema in both treatment arms decreased significantly (p < 0.01). Clinical evaluation revealed high improvement. Mean pain intensity was significantly lower with the KTP (2.5/10) than with the PDL (4.1/10). Both lasers showed a good safety profile. Relevant purpura was only seen in the PDL group.
CONCLUSIONS: Both the variable-sequenced, large-spot KTP and the PDL demonstrated comparable efficacy in treatment of rosacea. Regarding safety, the KTP exhibited fewer post-treatment reactions. The KTP might serve as a potential alternative to PDL in the treatment of rosacea.

Facial and neckline telangiectasias have an underestimated yet important impact on quality of life of patients with systemic scleroderma (SSc). This monocentric, prospective, open-label, intra-patient comparative study was conducted in 21 consecutive patients with SSc. Patients underwent 4 sessions of PDL 8 weeks apart. A final quadruple assessment was performed by several raters 2 months after the last session, based on the following criteria: change in telangiectasia number; subjective improvement score (LINKERT scale); impact on the quality of life (QoL; SKINDEX score); visual analog pain scale; adverse effects (AEs), including treatment discontinuation for PDL-induced purpura and patient satisfaction. The mean telangiectasia number decreased by 5 (32%) at the end of the protocol. Eighteen patients (85.7%) reported an improvement or a strong improvement, versus 73.81% for the expert committee. Immediate session pain (mean = 3.4/10) was slightly less than overall pain (mean = 4.6/10). Ten patients (47%) experienced at least one AE (oozing/crusts, edema, epidermal blistering), including PDL-induced purpura in 3 patients (14%). AEs were mostly transient (<1 week) and mild (CTCAE grade 1). All QoL parameters improved after treatment, and 85% of patients were satisfied.

BACKGROUND: Primary immune thrombocytopenia (pITP) in dogs presents a diagnostic challenge, and clinical markers of severity are lacking.
OBJECTIVE: Identify clinicopathologic features that differentiate pITP from secondary ITP (sITP) and markers related to bleeding severity, transfusion, and survival of dogs with pITP.
METHODS: Ninety-eight thrombocytopenic dogs (58 pITP and 40 sITP).
METHODS: Client-owned dogs with platelet counts <50 000/μL were enrolled in a prospective, multi-institution cohort study. History and treatment information, through a maximum of 7 days, was recorded on standard data forms. Bleeding severity was scored daily using a bleeding assessment tool (DOGiBAT). At-admission blood samples were collected for CBC, biochemistry, C-reactive protein concentration, and coagulation panels, and to measure platelet surface-associated immunoglobulin G (PSAIg) and expression of platelet membrane proteins and phospholipids. Dogs with evidence of coincident disease were classified as sITP.
RESULTS: No definitive pITP diagnostic test was found. However, pITP cases were characterized by lower platelet counts, D dimer concentrations, and platelet membrane protein expression than sITP cases. Differentiation between pITP and sITP was further enhanced using logistic regression modeling combining patient sex, coagulation profile, platelet count, D dimer, and PSAIg. A second model of pITP severity indicated that low hematocrit and high BUN concentration were associated with non-survival. Low hematocrit at admission, but not platelet count or DOGiBAT score, was associated with transfusion.
CONCLUSIONS: Pending validation studies, models constructed from at-admission clinicopathologic findings may improve differentiation of pITP from sITP and identify the most severe pITP cases at the time of presentation.

BACKGROUND: The use of a tourniquet in combination with tranexamic acid (TXA) not only ensures clear vision, reduces intraoperative blood loss and shortens operative time but also improves cement-bone inter-digitation in total knee arthroplasty (TKA). However, there is no proof whether the blood flow blocking effect of tourniquet affects the antifibrinolytic effect of TXA, and the optimal timing of TXA administration is still unclear. Therefore, this study aims to investigate the effect of the first dose of TXA administered intravenously before tourniquet compression and release in TKA on perioperative blood loss and therapeutic efficacy in patients.
METHODS: In this double-blind trial, 90 patients undergoing primary TKA were randomised into 2 groups: Group A, patients received intravenous TXA 10 min before tourniquet compression (20 mg/kg) and 3, 6 and 24 h later (10 mg/kg), and Group B, patients were treated the same as those in Group A but received intravenous TXA before tourniquet release. The primary outcomes were changes in blood loss, haemoglobin and haematocrit. Secondary outcomes included operation and tourniquet times, blood transfusion rate, subcutaneous petechiae and circumferential changes in the operated limb, visual analogue scale (VAS) score, hospital for special surgery (HSS) score, length of stay (LOS) postoperatively, complications and patient satisfaction.
RESULTS: No statistically significant difference was found between the 2 groups with regard to age, sex, weight, body mass index (BMI), Kellgren-Lawrence class, preoperative blood volume, preoperative laboratory values, operation and tourniquet times, transfusion rate, knee circumference, preoperative HSS, or VAS score (P:n.s.). There was no significant difference in intraoperative blood loss (IBL) (52.7 ml vs. 63.4 ml, P = 0.07), hidden blood loss (HBL) (91.4 ml vs. 119.9, P = 0.4) or total blood loss (TBL) (144.1 ml vs. 183.3 ml, P = 0.72) between Groups A and B. Haemoglobin, haematocrit and red blood cell count (RBC) dropped to a low point on postoperative day 3 and then rebounded, returning to normal levels on day 21, and the trend of change between the 2 groups was not statistically significant (P:n.s.). There was no significant difference in subcutaneous ecchymosis incidence, knee swelling rate, HSS score, VAS score, LOS postoperatively, complication rate or patient satisfaction (P:n.s.).
CONCLUSIONS: TXA was administered intravenously prior to tourniquet compression could effectively reduce blood loss in patients who had undergone total knee arthroplasty. However, there was no significant difference in knee swelling rate, subcutaneous bruising and petechiae incidence, knee function, complication rate or satisfaction between patients who TXA was given intravenously before tourniquet compression and release in primary TKA.

BACKGROUND: In South Korea, COVID-19 vaccination has been recommended to adolescents aged 12 - 17 since October, 2021. We aimed to assess the rate of adverse events following COVID-19 vaccination in adolescents with chronic kidney disease (CKD) in South Korea, using national cohort data.
METHODS: We retrieved the clinical information of adolescents 12 - 17 years old from the Korea Disease Control and Prevention Agency-COVID19-National Health Insurance Service (K-COV-N) database, to calculate incidence rates of purpura and other hemorrhagic conditions, Guillain-Barré syndrome (GBS), Kawasaki disease/multisystem inflammatory syndrome in children (MIS-C), myocarditis and/or pericarditis, and anaphylaxis in adolescents with CKD, after BNT162b2 vaccination.
RESULTS: Among the 2306 adolescents with CKD, 62.7% (n = 1446) had received the BNT-162b2 vaccine. GBS, Kawasaki disease/MIS-C, and anaphylaxis or anaphylactic shock did not occur during the observation period. Purpura and hemorrhagic conditions were more frequent in the unvaccinated group (7/860 vs. 1/1446), while myocarditis/pericarditis was observed only in the vaccinated group (0/860 vs. 1/1446). Adjusted odds ratio for any of the two adverse events was lower in vaccinated adolescents than in the unvaccinated group which did not reach statistical significance (adjusted odds ratio = 0.14, 95% confidence interval: 0.02, 1.16, P = 0.068).
CONCLUSIONS: In this national cohort study of adolescents with CKD in South Korea, we observed no evidence of increased risk of adverse events following BNT162b2 vaccination. Our finding offers insights into the safety of COVID-19 vaccines, empowering adolescent patients with CKD and their caregivers to make informed decisions.

BACKGROUND: Dengue is an arbovirosis affecting nearly 4 billion people worldwide. Since 2018, dengue has been re-emerging in Reunion Island. The incidence of mucocutaneous manifestations varies according to the studies and is generally called \'rash\'.
OBJECTIVE: To assess the prevalence of different mucocutaneous symptoms and describe the characteristics of patients developing these symptoms and the clinical signs associated with severe dengue.
METHODS: A prospective study was conducted in 2019 at the University Hospital of La Réunion, in patients presenting a positive PCR for dengue. Descriptive analyses were performed. All cases in the prospective study were examined by a dermatologist.
RESULTS: A total of 163 cases were included. The prevalence of mucocutaneous signs was 80.4%. A pruritus was reported in 33.7% cases, an erythematous rash in 29.4% and a mouth involvement including lip, tongue, cheek, angular cheilitis, pharyngitis, mouth ulcer and gingivitis in 31.3%. Most of symptoms appeared in the first days, but some of them could disappear only after the 3rd week. Mucocutaneous signs were not associated with a severe dengue fever (p = 0.54), but ecchymotic purpura was (p = 0.037). In multivariate analysis, skin involvement was associated with flu-like syndrome (headache, pharyngitis, rachis pain) and patient required rehydration but not invasive reanimation.
CONCLUSIONS: This work confirms the high prevalence of skin symptoms in dengue disease, but also their wide diversity. The mucocutaneous involvement of dengue fever appears to be accompanied by a pronounced flu-like syndrome in people without severity, but careful examination to identify ecchymotic purpura or sign of dehydration in the mucous membranes would better identify cases that may worsen.

BACKGROUND: Pigmented purpuric dermatosis (PPD) is characterized by grouped petechiae, purpuric macules, and pigmentation in the bilateral lower extremities. It runs a chronic and relapsing course. Pathophysiology is poorly understood, but it has been proposed to be an immune-complex disease or capillaritis. This study aimed to determine the incidence and patterns of positive direct immunofluorescence (DIF) findings in patients with clinically and histopathologically confirmed PPD. The association between DIF deposition type and clinical profile was also analyzed.
METHODS: Patients with a clinical and histopathologic PPD diagnosis who had undergone DIF studies at a tertiary medical center with attached dermatopathology and immunofluorescence diagnostic centers between January 2002 and December 2021 were included in this study. Data on age, sex, disease duration, comorbidities, and drug intake were collected from medical records.
RESULTS: There were 65 patients who satisfied the inclusion criteria. Among them, 58 (89%) had at least one positive finding and 53 (82%) were vascular deposition of immunoglobulin (Ig), complement, or fibrinogen. The most common vascular deposition was fibrinogen (71%) followed by C3 (62%), IgM (18%), IgA (6%), and IgG (3%). Fibrinogen deposition was associated with hypertension (p < 0.03). There was no association between vascular DIF deposition of IgG, IgA, and C3, with age, sex, comorbidities, disease duration, and drug history.
CONCLUSIONS: The most common DIF findings in PPD were vascular deposition of fibrinogen and C3, with or without Ig presence. DIF findings supported a vascular origin in PPD but not an immune complex-mediated disease. Hypertension was associated with fibrinogen deposition and may play a role in its pathophysiology.