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Background: Purpura and glomerulonephritis are typical presentations in IgA vasculitis. Infective endocarditis mimicking IgA vasculitis by presenting with glomerulonephritis and purpura is rarely reported., Methods: We searched for cases with infective endocarditis-associated purpura and glomerulonephritis in a tertiary hospital in China and retrospectively reviewed their clinicopathological features. Differential diagnosis and treatment in patients with infective endocarditis-associated purpura and glomerulonephritis were discussed., Results: A total of 20 cases with infective endocarditis-associated purpura and glomerulonephritis were identified among 548 cases with infective endocarditis in our center during an 8-year period: 7 of the 20 cases (35%) were initially misdiagnosed as IgA vasculitis and 10 cases (50%) presented with left-sided endocarditis caused by Streptococcus viridans. Fever (100%, 20 out of 20), prior valvular deformities (80%, 16 out of 20), cardiac murmur (95%, 19 out of 20), splenomegaly (84%, 16 out of 19), embolism (55%, 11 out of 20), and hypocomplementemia (76%, 13 out of 17) were present in most patients. Crescents and mesangial hypercellularity with or without endothelial hypercellularity were the primary findings on light microscopy, with C3-dominant deposition on immunofluorescence. But IgA-dominant staining was also observed (40%, 2 out of 5). In patients with rapidly progressive glomerulonephritis, patients with complete recovery of renal function had shorter disease duration and higher ratio (67% vs 20%) of immunosuppressive therapy compared with patients with partial recovery., Conclusions: Infective endocarditis-associated glomerulonephritis and purpura can closely mimic IgA vasculitis. Differential diagnosis is challenging, particularly when typical presentations of infective endocarditis are absent. In adults with presentations like IgA vasculitis, infective endocarditis should be evaluated through comprehensive clinical and pathological investigations. Immunosuppressive therapy can be considered in patients with severe glomerulonephritis who do not improve after proper anti-infective therapy., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Ethylmalonic encephalopathy is a rare autosomal recessive mitochondrial disorder caused by pathogenic biallelic variants in the ETHE1 gene. The phenotype of this disease has been attributed to deficiency in the mitochondrial sulfur dioxygenase leading to many downstream effects. Ethylmalonic encephalopathy classically presents with developmental regression, petechiae, acrocyanosis, and chronic diarrhea. The neurologic phenotype includes hypotonia, spastic diplegia, ataxia, and developmental delay. As more patients with this condition are described, the neurologic phenotype continues to expand. Although strokelike episodes or metabolic strokes have been studied in other mitochondrial disorders, they have not been thoroughly reported in this disorder. Herein, we describe 3 patients with ethylmalonic encephalopathy who presented clinically with strokelike episodes and strokelike abnormalities on brain magnetic resonance imaging in the setting of acute illness, and the long-term sequelae with evolution into cystic changes in one of these subjects.

Ethylmalonic encephalopathy (EE) is a rare autosomal recessive inborn error of metabolism. To study the molecular effects of ETHE1 p. D165H mutation, we employed mass spectrometry-based mitochondrial proteome and phosphoproteome profiling in the human skeletal muscle. Eighty-six differentially altered proteins were identified, of which thirty-seven mitochondrial proteins were differentially expressed, and most of the proteins (37%) were down-regulated in the OXPHOS complex-IV. Also, nine phosphopeptides that correspond to eight mitochondrial proteins were significantly affected in EE patient. These altered proteins recognized are involved in several pathways and molecular functions, predominantly in oxidoreductase activity. This is the first study that has integrated proteome and phosphoproteome of skeletal muscle and identified multiple proteins associated in the pathogenesis of EE., (Copyright © 2021 Elsevier B.V. and Mitochondria Research Society. All rights reserved.)

Patients with coronavirus disease 2019 (COVID-19) present with multisystem signs and symptoms, including dermatologic manifestations. The recent literature has revealed that dermatologic manifestations of COVID-19 often are early onset and provide helpful cues to a timely diagnosis. We compiled the relevant emerging literature regarding the dermatologic manifestations of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) so that physicians can be aware of the various clinical cutaneous presentations in this time of high incidence of COVID-19.

Background: Cryoglobulinemic glomerulonephritis (CryoGn) caused by hepatitis B virus (HBV) infection was rarely reported. Our study aimed to investigate the clinical features, renal pathology findings, and prognosis in patients with HBV related CryoGn., Methods: This was a retrospective study including seven Chinese patients with HBV related CryoGn in a tertiary referral hospital from April 2016 to March 2019. The clinical and pathological data were collected and analyzed., Results: Age at renal biopsy was 47 ± 12 years, with female/male ratio 3/4. Urine protein was 5.6 (3.0, 6.6) g/d and five cases presented with nephrotic syndrome. The baseline eGFR was 23.5 (20.2, 46.3) ml/min per 1.73m 2 . The extrarenal manifestations included purpura (n = 6), arthralgia (n = 1), peripheral neuropathy (n = 1), and cardiomyopathy (n = 1). Six cases had type II cryoglobulinemia with IgMκ, the other one had type III. The median cryocrit was 4.0 (1.0, 15.0) %. Renal pathologic findings on light microscopy: endocapillary proliferative glomerulonephritis (Gn) (n = 3), membranoproliferative Gn (n = 3), and mesangial proliferative Gn (n = 1). On immunofluorescence microscopy, the predominant type of immunoglobulin deposits was IgM (n = 5). HBsAg and HBcAg deposits were found in one case. Ultrastructural studies showed granular subendothelial and mesangial electron-dense deposits in all patients and microtubules in one case. All patients received antiviral medications. They were given corticosteroid alone (n = 2) or combined with cyclophosphamide (n = 4) or mycophenolate mofetil (n = 1). Two patients received plasmapheresis. The median follow-up time was 18 (6, 37) months. Four patients got remission, two patients died of pneumonia, and one progressed to end-stage renal disease (ESRD). At endpoint of follow-up, 24hUP was 2.1 (0.8-5.2) g/d, and eGFR was 55.3 (20.7, 111.8) ml/min per 1.73m 2 . The median cryocrit decreased to 1.0 (0, 5.75) %., Conclusions: The etiology of mixed CryoGn should be screened for HBV infection. Endocapillary proliferative Gn and membranoproliferative Gn were the common pathologic patterns. Diagnosis and treatment in early stage benefit patients' renal outcomes. Immunosuppressive therapy should be considered for severe renal disease, based on efficient antiviral therapy.

In this article we focus on updates in select etiologies of retiform purpura. These causes of retiform purpura, in addition to bacterial or fungal sepsis, disseminated intravascular coagulation, purpura fulminans, and catastrophic antiphospholipid syndrome, are important diagnoses with potential for morbidity and mortality. Important aspects in the pathophysiology, patient demographics and risk factors, updates in the diagnostic workup, histopathology, and treatment of these specific conditions are discussed., (Copyright © 2019 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Negative pressure of cupping induces skin deformation such as ecchymosis and purpura in a circular shape. Thus, there is a desire to treat skin before depigmentation and scarring occurs. Therefore, we introduce laser therapy, a widely used technique treat pigmentation in dermatology. Various parameters of laser therapy can be applied, so to determine the optimal exposure parameters that do not damage the surrounding tissues, the subjects were divided into four groups: a non-stimulation group and three laser groups (4 J/cm2 group, 6 J/cm2 group, and 8 J/cm2 group). We selected the wavelength and output of laser as follows: 660nm and 50mW. The 40 were divided into four groups of 10. In the first experiment, we measured skin temperature using Digital infrared thermography in order to observe whether the laser could cause heat damage. In the second experiment, each group received the assigned laser therapy protocol every 24 hours for 72 hours. We obtained a skin image using a cross polarization technique. Previous studies have shown that a*and E.I (erythema index) represent the degree of skin erythema (hemoglobin content). M.I (melanin index) indicates the degree of skin pigmentation (melanin content). Hence, skin color information was analyzed with the a*, erythema index (E.I), and melanin index (M.I) for 72 hours. None of the laser exposure parameters led to skin damage by heating or energy dissipation. The results of a*, E.I, and M.I of all groups showed the different recovery rates towards the normal skin color information before cupping. As energy density increases, the result of a* and E.I showed the fast recovery rate. There was no significant different between M.I at non-stimulation group and M.I at 4 J/cm². Therefore, the least energy density as 6 J/cm² is need for the recovery of melanin content. The a*, E.I, and M.I at 8 J/cm² group rather than other groups were significantly recovered to normal skin color. In conclusion, the laser therapy (energy density: 8 J/cm²) has a significant recovery of the skin erythema and skin pigmentation except to skin damage.

Mitochondrial disorders are a group of highly invalidating human conditions for which effective treatment is currently unavailable and characterized by faulty energy supply due to defective oxidative phosphorylation (OXPHOS). Given the complexity of mitochondrial genetics and biochemistry, mitochondrial inherited diseases may present with a vast range of symptoms, organ involvement, severity, age of onset, and outcome. Despite the wide spectrum of clinical signs and biochemical underpinnings of this group of dis-orders, some common traits can be identified, based on both pathogenic mechanisms and potential therapeutic approaches. Here, we will review two peculiar mitochondrial disorders, ethylmalonic encephalopathy (EE) and mitochondrial neurogastrointestinal encephalomyopathy (MNGIE), caused by mutations in the ETHE1 and TYMP nuclear genes, respectively. ETHE1 encodes for a mitochondrial enzyme involved in sulfide detoxification and TYMP for a cytosolic enzyme involved in the thymidine/deoxyuridine catabolic pathway. We will discuss these two clinical entities as a paradigm of mitochondrial diseases caused by the accumulation of compounds normally present in traces, which exerts a toxic and inhibitory effect on the OXPHOS system., (© 2015 The Authors. Published under the terms of the CC BY 4.0 license.)

Antiphospholipid syndrome is an autoimmune disorder characterized by the occurrence of venous and arterial thrombosis, as well as morbidity in pregnancy, in the presence of anti-phospholipid antibodies. The diagnosis of antiphospholipid syndrome is usually established based on clinical and laboratory findings by strictly following the 2006 Sapporo classification. However, the diagnosis remains challenging owing to the ongoing debates on the serological criteria. We report a case we describe as forme fruste antiphospholipid syndrome in which these criteria were not fulfilled. Purpura appeared repeatedly in a female infant starting from the age of 6 months and following episodes of upper respiratory infections and vaccinations. The levels of anti-cardiolipin IgG antibodies and anti-phosphatidylserine/prothrombin complex antibodies were elevated in accordance with these events. Histopathological evaluation revealed multiple small vessel thrombi in the dermis and adipose tissue. After 2 weeks of treatment with aspirin and heparin, the cutaneous symptoms subsided. Infection has long been associated with antiphospholipid syndrome, and anti-phosphatidylserine/prothrombin antibodies are considered a new marker for the diagnosis of antiphospholipid syndrome. Forme fruste antiphospholipid syndrome should be considered even if the antiphospholipid syndrome diagnostic criteria are not completely fulfilled, especially in the presence of elevated levels of anti-phosphatidylserine/prothrombin antibodies and known preceding infections.

Granulomatous pigmented purpuric dermatosis (PPD) is a rare and poorly recognized histological variant of PPD, which commonly affects the distal extremities of mainly Far East Asian patients. Many of the reported cases are associated with hyperlipidemia or other associated systemic derangements. The authors hereby describe an additional case of granulomatous PPD affecting a 56-year-old Caucasian woman presenting unusually as a solitary lesion confined to the lower back. The report also describes dermoscopic findings, summarizes clinicopathological features of all the cases published till date, and discusses the histopathological differential diagnosis.

The aim of the study was to investigate the association between capillary fragility and some hemostatic parameters, lipid profile in patients with rosacea. 50 patients (30 women and 20 men) aged 35 to 65 years were under observation. Control group consisted of 50 healthy persons, adequate to comparison group by sex and age. To determine the resistance of the capillary, Rumpel-Leede cuff (tourniquet test) was used which consists in determining the formation of petechial hemorrhages on the skin in the area of ​​short-term increase in venous pressure. The hemostatic system was evaluated in terms of prothrombin and thrombin time. Content of fibrinogen and fibrinolytic activity of blood were determined also. The serum lipid profile was studied by means of the following parameters: total cholesterol, triglycerides, HDL (high density lipoprotein), LDL (low density lipoproteins). The survey revealed that in 25 patients the arm cuff test was positive, whereas in the control group, only 2 cases it was weakly positive. Manifestations of hypercoagulation were found in half of patients with a positive cuff test, almost in half of the patients an increased level of fibrinogen and the reduced fibrinolytic activity in blood serum has been revealed. Significant correlation with lipid metabolism have not been identified. Phenomenon of hypercoagulation in rosacea patients on the one hand suggests the existence of processes of microcoagulation, on the other hand the connection with the results of a cuff test can be used to predict the severity of the dermatosis and the possible risk for developing of cardiovascular disease.

Cupping is one of the various treatment methods used in traditional oriental medicine. Cupping is also used as a diagnostic method and it may cause skin hyperpigmentation. Quantitative measurements and analysis of changes in skin color due to cupping are critical. The purpose of this study is to suggest an optical technique to visualize and identify changes in skin color due to cupping. We suggest the following analysis methods: digital color spaces [red, green, and blue (RGB) and L∗a∗b], the Erythema Index (E.I.), and the Melanin Index (M.I.). For experiments, we selected and stimulated 10 acupoints at 80 kilopascals (kPa) per minute. The RGB and L∗a∗b color spaces were observed to be decreased (p < 0.05) after cupping. The E.I. and M.I. were observed to be increased significantly (p < 0.05) after cupping. To assess various changes in skin color, we observed the changes for 72 hours. We also obtained the color changes by using the recovery pattern during the recovery period (p < 0.01). We propose that this method can be useful for visual identification and as a way to improve the identification of skin color changes., (Copyright © 2014. Published by Elsevier B.V.)

Human immunodeficiency virus (HIV) infection is growing medical concern worldwide. There are many types of glomerulonephritis which are associated with HIV infection. We report a case of a 53-year-old Korean man with an HIV infection, who was developed nephritic range proteinuria and purpura with elevated IgA level rasing a possibility of Henoch-Schölein Purpura (H-S purpura). However, renal biopsy showed "lupus-like feature" glomerulonephritis without clinical or serologic evidence of systemic lupus erythematosus. Although baseline renal function was maintained without further need for maintenance dialysis following anti-retroviral therapy (ART) and steroid, patient died from uncontrolled gastrointestinal bleeding.

We present an unusual case of congestion bleeding of the head and neck following myocardial infarction. A 51-year-old man presented with widespread facial petechiae and subconjunctival haemorrhages following a collapse associated with evolving electrocardiographic changes. Emergency coronary artery stent placement was undertaken. No cardiopulmonary resuscitation (CPR) was performed. We hypothesise that the presence of facial petechiae in our case following transient loss of consciousness due to a presumed ventricular arrhythmia in the setting of acute myocardial ischaemia, may have been precipitated by a Valsalva manoeuvre on regaining consciousness with sudden acute increase in venous pressure and consequent venous congestion of the head and neck, and that congestion bleeding of the face may occur in acute cardiac events without a history of CPR.

Introduction: Red Bull Stratos was a commercial program that brought a test parachutist protected by a full pressure suit to 127,852 ft (38,964 m), via a stratospheric balloon with a pressurized capsule, from which he free fell and subsequently parachuted to the ground. In light of the uniqueness of the operation and the medical threats faced, medical protocols specific to distinctive injury patterns were developed. One unique threat was that of a flat spin during free fall with resultant exposure to -Gz (toe-to-head) acceleration. In preparation for stratospheric free fall, the medical team conducted a review of the literature on the spectrum of human and animal injury patterns attributable to -Gz exposures. Based on the findings, an emergency medical field response protocol was developed for the rapid assessment, diagnosis, and treatment of individuals suspected of -Gz injury., Methods: A systematic review was conducted on available literature on human and animal studies involving significant -Gz exposure, with subsequent development of an applicable field treatment protocol., Results: The literature review identified pathophysiologic processes and mitigation strategies that were used to develop a prevention and treatment protocol, outlining appropriate interventions using current best medical practices. A medical field treatment protocol was successfully established for the high-altitude balloon program., Discussion: Available literature provided insight into best medical practices for the prevention and treatment of significant -Gz exposures during high-altitude parachute activity. Using the protocol developed for the field medical response, injuries from sustained -Gz exposure can be effectively managed in similar high-altitude and space operations.

Exercise induced vasculitis (or purpura) is usually misdiagnosed and ignored in the literature, although it is not uncommon. We report two female patients who developed a rash on the lower legs after walking all day long. The rash was red, itchy with a burning sensation. The lesions resolved after a few days with and without steroid treatment. Allergic etiology was suspected, therefore they were sent to the allergy clinic for evaluation. Exercise-induced vasculitis tends to occur in healthy people, especially in hot weather. Extensive investigation with blood or allergy tests is not needed.

Background: Children with petechial rash are more likely to undergo invasive diagnostics, to be treated with antibiotics for potential bacterial infection and to be hospitalized. However, viruses have also been associated with petechial rash. Nonetheless, a systematic analysis of viral infections with modern available techniques as quantitative real-time polymerase chain reaction in the context of petechial rash is lacking. The purpose of this pediatric study was to prospectively uncover viral pathogens that may promote the emergence of petechiae and to analyze the correlation with the clinical characteristics and course., Methods: We conducted a prospective study in children (0 to 18 years) presenting with petechiae and signs or symptoms of infection at the emergency department between November 2009 and March 2012. In nasopharyngeal aspirates the following viruses were analyzed by quantitative real-time polymerase chain reaction: cytomegalovirus, Epstein-Barr virus, parvovirus B19, influenza A and B, parainfluenza viruses, human respiratory syncytial virus A and B, human metapneumovirus, rhinovirus, enterovirus, adenovirus, human coronavirus OC43, 229E, NL63 and human bocavirus., Results: A viral pathogen was identified in 67% of the analyzed 58 cases with petechial rash. Virus positive patients showed a significantly higher incidence of lower respiratory tract infections. Forty-one percent were viral coinfections, which were significantly younger than virus negative patients, had a higher leukocyte count and were hospitalized for a longer time., Conclusions: A petechial rash is frequently associated viral single- and coinfections and can rapidly be identified via quantitative real-time polymerase chain reaction.

Recreational or regular physical and sport activities may be responsible for a wide range of cutaneous complications. Among them, "sports purpura" is a peculiar symptom that can occur during a large number of sports. "Effort purpura" defines any purpura occurring within the context of physical exercise irrespective of its cause. Therefore this clinical diagnosis includes various aetiologies. Diagnosis of traumatic purpura is often easy if the sport is mentioned in the anamnesis; cutaneous exercise - induced vasculitis must be also noted. Purpura can reveal systemic diseases or internal haemorrhage, such as spleen rupture, thrombopathies or systemic vasculitis, and other effort purpuras must be taken into account, including those related to the environment (cold, sun exposure...). Knowledge of a physical activity before the occurrence of purpura should be known by practitioner to avoid unnecessary and costly explorations in most of the cases., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Organic acidurias or organic acidemias constitute a group of inherited disorders caused by deficient activity of specific enzymes of amino acids, carbohydrates or lipids catabolism, leading to large accumulation and excretion of one or more carboxylic (organic) acids. Affected patients usually present neurologic symptoms and abnormalities, sometimes accompanied by cardiac and skeletal muscle alterations, whose pathogenesis is poorly known. However, in recent years growing evidence has emerged indicating that mitochondrial dysfunction is directly or indirectly involved in the pathology of various organic acidemias. Mitochondrial impairment in some of these diseases are generally due to mutations in nuclear genes of the tricarboxylic acid cycle or oxidative phosphorylation, while in others it seems to result from toxic influences of the endogenous organic acids to the mitochondrion. In this minireview, we will briefly summarize the present knowledge obtained from human and animal studies showing that disruption of mitochondrial homeostasis may represent a relevant pathomechanism of tissue damage in selective organic acidemias. The discussion will focus on mitochondrial alterations found in patients affected by organic acidemias and by the deleterious effects of the accumulating organic acids on mitochondrial pathways that are crucial for ATP formation and transfer. The elucidation of the mechanisms of toxicity of these acidic compounds offers new perspectives for potential novel adjuvant therapeutic strategies in selected disorders of this group.

Increased ethylmalonic acid (EMA) in urine is a non-specific finding, and is observed in a number of inborn errors of metabolism, as well as in individuals who carry one of two common polymorphisms identified in the SCAD coding region. The authors present an 8-month-old girl with a suspicion of neuroinfection, although the clinical presentation led to diagnosis of ethylmalonic aciduria. From the neuropathological point of view the most remarkable changes were observed in the brain cortex, which was diffusely damaged practically in all regions of the brain. Of note, the most severe destruction was observed in the deepest regions of the sulci. The cortex of the affected regions showed no normal stratification and its structure was almost totally replaced by a form of "granulation tissue" with a markedly increased number of capillaries. To the authors' knowledge this is the first clinical report of ethylmalonic aciduria with brain autopsy findings.

Unlabelled: Post-transfusion purpura (PTP), a delayed post-transfusion event, is underdiagnosed, not only in Poland. Thrombocytopenia results from the destruction of patients' and transfused platelets by platelet specific antibodies (anti-HPA). The incidence of PTP is not estabilished., The Aim: Analysis of 10 cases with PTP diagnosed in Poland during last 25 years., Material and Methods: 9 women and 1 man with normal platelet count who developed thrombocytopenia with diathesis haemorrhagica after transfusion of red cells or/and platelets. Platelet specific antibodies (anti-HPA) were examined by the platelet immunofluorescence test and by the monoclonal immobilization of platelet antibodies (MAIPA) assay; leucocyte antibodies by standard lymophocytotoxicity test (LCT)., Results: In all 10 patients the PTP was diagnosed because: 1) thrombocytopenia with diathesis haemorrhagia occured 3-10 days after the transfusion (in 5 cases blood was transfused during the operation), 2) in all the patients anti-HPA were detected (in 9- anti-HPA-1a, in 1 case anti-HPA-3a), 3) a primary HPA alloimmunization was very probable. A recovery of platelets count occured in 6 patients within 8-34 days after corticosteroids and/or IVIG, however, therapeutic effect of them was difficult to assess due to a great probability of spontaneous remission up to 1 month. Four patients died due to their basic disease although the impact of the PTP cannot be excluded., Conclusion: The diagnosis of PTP was possible because of the detection of anti-HPA antibodies in patients who developed thrombocytopoenia after blood transfusion.

The eutectic mixture of local anesthetic cream, a 1 : 1 mixture of prilocaine and lidocaine, 2.5% each, is frequently used in pediatric and dermatologic practice to obtain local anesthesia. Side effects include transient skin blanching, erythema, urticaria, allergic contact dermatitis, irritant contact dermatitis, hyperpigmentation, and purpura. We report two children with a purpuric reaction after application of this mixture cream. Purpura after application of this anesthetic cream is a rare nonallergic reaction and only 17 occurrences have been reported, to our knowledge, in the literature. Patch tests could not be performed in our two patients because of lack of parental consent but we suggest that the purpuric reactions were most probably of toxic origin. The pathogenesis of purpura after application of eutectic mixture of local anesthetics cream, which resolves within 2 weeks without dermatologic sequelae and without any specific therapy, is complex. The lesions are probably caused by the direct effect of the cream components on the vessels but many other factors, such as atopic dermatitis, prematurity, subjective predisposition to purpura, trauma, and thrombocytopenia may play important pathogenetic roles.

Objectives/hypothesis: Intrathoracic petechiae are a prominent diagnostic finding in sudden infant death syndrome (SIDS) victims. In this study, the laryngeal chemoreflex (LCR) was elicited experimentally to discover whether intrathoracic petechiae would be produced by way of the LCR. The hypothesis was that water stimulation of the larynx in piglets, leading to death by prolonged apnea, would produce postmortem findings similar to those found in SIDS victims., Study Design: Using the piglet as an animal model, the LCR was initiated by way of water stimulation of the larynx, resulting in death. Normoxic and hypoxic conditions were established before the stimulation. The piglets were studied postmortem to determine the relationship between the physiologic mechanisms of the LCR and characteristic pathologic findings in SIDS., Methods: Using protocols approved by animal care, 14 mixed-breed piglets aged 7 to 14 days were sedated with a ketamine/xylazine mixture. Respiratory and pressure-monitoring devices were affixed and light anesthesia maintained with Surital infusion. In 10 of the piglets, a small catheter was placed between the arytenoid cartilages, and 5 mL of tap water was introduced over 1 second. The LCR ensued, producing periods of central apnea bordered by gasping efforts and resulting in hypoxemia and death in all cases. Four piglets underwent this manipulation in normoxic conditions. Six breathed a hypoxic gas mixture for 1 hour to bring their Po2 down to below 50 torr before water was introduced into the larynx. Four control piglets breathed the hypoxic gas mixture for 1 hour (without water stimulation or LCR) before Surital overdose. Within 24 hours of death, all piglets underwent thoracoabdominal autopsy by a blinded evaluator experienced in SIDS pathology., Results: The autopsies revealed nothing remarkable in the abdominal viscera of any of the experimental animals. Thymus, heart, and lungs were graded 0 to 4 to indicate the degree of petechiae on external surfaces. Average cumulative scores (ACS) were applied to each animal. The control (hypoxic) piglets had no petechiae (ACS 0.0). The normoxic experimental piglets had moderate petechiae (ACS 3.5). The hypoxic experimental piglets had more prominent petechiae (ACS 6.3)., Conclusions: Stimulation of the LCR, leading to death by prolonged apnea, produces postmortem findings in piglets similar to those found in SIDS victims. Petechiae were more severe among piglets pretreated with a hypoxic mixture of gases. This study supports the hypothesis that initiation of the LCR may produce pathologic features often prominent in SIDS.

Physiological reactions of four cosmonauts to +Gx during the descent after short-term (less than 30 d) space flights were correlated with the hemodynamic shifts developed previously during the LBNP test and occlusive plethysmography in microgravity. The cosmonauts who had exhibited a sharp decrease in cerebral circulation during the in-flight LBNP tests showed signs of deteriorated cerebral blood flow (vertigo, weakness, hyperhydration) at the attempt to assume the vertical posture and to climb out of the landing module. Also, the cosmonauts found to have the arterial and venous tone significantly dropped in microgravity, suffered petechial hemorrhages in the back integument during the exposure to +Gx. These data suggest a correlation between the physiological reactions of cosmonauts to +Gx at the end of short-term space flight and the shifts in hemodynamics earlier in microgravity.

Background and Objectives: Since the development of laser-induced photothermolysis for the therapy of port wine stain (PWS) birthmarks, clinical results have shown that dark purple lesions usually respond well to the first three to five treatments. However, for most PWS, complete blanching is never achieved, and the lesion stabilizes at a red-pink color. The aim of this feasibility study is to demonstrate that with the aid of a local vacuum applied to the lesion site prior to laser exposure, photocoagulation of the smaller PWS blood vessels may be successfully achieved., Study Design/materials and Methods: Suction cups were designed to fit onto the hand pieces of commercial laser devices used for PWS laser therapy. One subject with normal skin and another with PWS skin were recruited for this study. Laser pulses of various fluences were applied at atmospheric pressure or shortly after (5-15 seconds) hypobaric pressures (17-51 kPa) were placed as test sites on the forearm of both subjects. The laser-induced purpura at the test sites was documented over the course of 1 week on both subjects and the resulting PWS blanching was optically quantified by visible reflectance spectrometry 7 months after therapy., Results: For the subject with normal skin, the laser fluence needed with hypobaric pressure (51 kPa) to induce similar purpura intensity to that observed with atmospheric pressure was approximately 35% lower. For PWS skin, all suction application times (5-15 seconds) and hypobaric pressures (17-51 kPa) resulted in more intense purpura and the PWS blanching 7 months after treatment was clinically significant for test sites treated with hypobaric pressures ranging from 17 to 34 kPa., Conclusions: The temporary and controlled dilation of the targeted blood vessels achieved with a local vacuum can significantly reduce the "small-vessel-limitation" in the treatment of PWS without increasing the risk of epidermal damage., ((c) 2005 Wiley-Liss, Inc.)

Aims: (1) To determine the causes of meningitis in children immunized with Hib vaccine, presenting without a non-blanching rash; (2) to review the use of dexamethasone in this group., Method: Retrospective review of all children with more then 10 white cells/mm(3) in their cerebrospinal fluid (CSF), admitted between January 1998 and August 2002. Children were excluded if they had a non-blanching rash on admission or if their discharge diagnosis was not meningitis. Local guidelines recommended dexamethasone to be given before antibiotics for children with meningitis and no rash., Results: One hundred and eight children were identified. Causes of proven meningitis were: viral 41 (enterovirus 40), bacterial 22. CSF culture or PCR was the only diagnostic test in 31 children. Dexamethasone was given to 16 children. Length of admission was shorter in children with viral compared with bacterial meningitis (4 vs 8 days; P < 0.0001)., Summary: Viral meningitis is the commonest cause of meningitis without rash. Enteroviral PCR was the most useful test and needs to be widely available. Confirmation of enteroviral meningitis allowed early discharge. Few children were given dexamethasone, but only 5/108 may have benefited., Conclusions: The most common cause of meningitis without a rash in British children is enterovirus. The use of dexamethasone in children with meningitis without a rash should be reconsidered or, at least, individualised.

Background: Among patients with ethylmalonic aciduria, a subgroup with encephalopathy, petechial skin lesions, and often death in infancy is distinct from those with short-chain acyl-coenzyme A dehydrogenase deficiency or multiple acyl-coenzyme A dehydrogenase deficiency. The nature of the molecular defect in this subgroup is unknown, and the source of the ethylmalonic acid has been unclear., Objective: To determine whether the administration of candidate amino acids increased the excretion of ethylmalonic acid., Design: Examination of patterns of organic acids excreted in the urine before and following loading doses of isoleucine and methionine., Setting: General clinical research center., Patient: An infant with ethylmalonic aciduria, global developmental delay, acrocyanosis, and intermittent showers of petechiae., Main Outcome Measure: Excretion of ethylmalonic acid in the urine., Results: Loading with methionine increased the excretion of ethylmalonic acid, whereas loading with isoleucine did not. Restriction of the dietary intake of methionine decreased ethylmalonic acid excretion., Conclusion: In ethylmalonic acid encephalopathy with petechiae, methionine is a precursor of ethylmalonic acid.

Background and Objectives: The average success rate in achieving total blanching of port wine stain (PWS) lesions treated with laser-induced selective photothermolysis is below 25%, even after multiple treatments. This is because smaller diameter (5-20 microm) PWS blood vessels are difficult to destroy with selective photothermolysis since the volumetric heat generated by absorption of laser light is insufficient to adequately heat the entire vessel wall. The aim of this study was to investigate a potential technique for more efficient photocoagulation of small diameter PWS blood vessels in PWS that respond poorly to selective photothermolysis., Study Design/materials and Methods: The blood volume fraction (BVF) in the upper dermis of the forearm of human volunteers was increased by placing an inflated blood pressure cuff on the upper arm. Applied pressures were in the range of 80-100 mm Hg for up to 5 minutes. The increased BVF was determined by matching reflectance spectra measured with that computed using a diffusion model. The impact of increased BVF on purpura formation induced by a 0.45 milliseconds pulsed dye laser (PDL) at 585 nm wavelength was investigated in normal and in PWS skin., Results: In the presence of a 100 mm Hg pressure cuff, the BVF, as determined from the diffusion model, increased by a factor of 3 in the forearm and by 6 in the hand. Increasing BVF by a factor of 3 corresponds to an increase in blood vessel diameters by a factor of radical 3 approximately 1.7. BVF increased at 1-3 minutes after application of the pressure cuff, remained constant at 3-5 minutes, and returned to baseline values at 3 minutes after removal of the pressure cuff. Approximately 40% less radiant exposure was needed to induce the same amount of purpura after PDL irradiation when the blood pressure cuff was used. Applying an 80 mm Hg pressure cuff reduced the required radiant exposure for purpura formation by 30%. Heating of blood vessels was calculated as a function of vessel diameter and of radiant exposure (at 585 nm and at 0.5 and 1.5 milliseconds pulse duration)., Conclusions: Enlarging the vessel lumen, for example, by obstructing venous return, can significantly reduce the "small-vessel-limitation" in PDL treatment of PWS. Dilation of PWS blood vessels enables a more efficient destruction of smaller vessels without increasing the probability of epidermal damage., (Copyright 2004 Wiley-Liss, Inc.)

Aim: To evaluate clinical peculiarities and prognostic value of abdominal syndrome in adult patients with systemic vascular purpura (SVP)., Material and Methods: Incidence, clinical picture, complications of abdominal syndrome, its relations with principal extraintestinal manifestations of SVP were studied in 125 SVP patients. Absorption and barrier functions of the small intestine, their correlations with clinical activity of the disease were investigated in 32 SVP patients with no clinical signs of intestinal affection. Computer-assisted morphometry of duodenal mucosa biopsies was conducted and the results were compared to those of intestinal permiability and clinical activity of SVP., Results: Negative correlation was found between the occurrence of abdominal syndrome and the patients' age. The gastrointestinal tract suffered more frequently in men. 21% patients had abdominal complications, association of abdominal syndrome with higher frequency of glomerulonephritis. In the absence of abdominal symptoms, the barrier function of the small intestine for protein macromolecules was impaired. This correlated with exacerbations of skin purpura. Increased intestinal permeability and activity of skin purpura correlated with severity of inflammation in the duodenal mucosa., Conclusion: Abdominal syndrome in SVP is an unfavourable prognostic factor indicating possible severe complications and high risk of glomerulonephritis. Subclinical intestinal involvement presenting as barrier dysfunction may contribute to aggravation of skin vasculitis.

The hallmarks of severe meningococcal sepsis include the rapid onset of shock, purpuric rash, and metabolic derangement, in particular, hypocalcemia. The severe ecchymoses and purpura associated with meningococcal sepsis are usually attributed to acute thrombotic episodes, attributable to the associated procoagulation disorder. An alternative explanation for the rash is a sudden extravasation of calcium from the intravascular space into the tissues. We will argue that in meningococcal sepsis, cleavage of albumin into fragments by protease(s) occurs and these fragments, along with calcium, cross the endothelium into the interstitium. The fragmentation of albumin and its loss through the endothelium would also provide a more rational explanation for the rapidity of the shock and the hypocalcemia that is so characteristic of the disease.

The causes of purpura can be classified into intravascular, vascular, and extravascular mechanisms. We describe a case of cervicofacial purpura in a powerlifter attributed to the accompanying Valsalva-associated increased arterial pressure. Powerlifting should be added to the list of activities that may cause purpura.

Cryoglobulins are immunoglobulins that persist in the serum, precipitate with cold temperature and resolubilize when rewarmed. Types II and III are mixed cryoglobulins (MC), composed of different immunoglobulins, with a monoclonal component in type II and only polyclonal immunoglobulins in type III. Mixed cryoglobulins are associated with connective-tissue disease, malignant hematological disorder (type B lymphoproliferation) or obvious infectious process. Mixed cryoglobulinemia syndrome is characterized by the clinical triad of purpura, arthralgia and asthenia associated with type II or type III MC. The disorder is the consequence of an immune-complex-type vasculitis as supported by clinical features, analysis of the cryoglobulins, the usually depressed level of complement during the active phase of the disease, and the deposition of immunoglobulins and complement in the lesions. Such cryoglobulinemia vasculitis may involve numerous organs, particularly the peripheral nervous system and the kidneys. MC is frequently associated with clinical and biological evidence of liver disease. There has been some controversy about which comes first, MC or chronic liver disease, but it seems fairly clear that MC is often a manifestation of underlying chronic active or persistent hepatitis. In MC patients, the hepatotropic antigen(s) capable of triggering production of antibodies which can later form immune complexes has been sought for many years. In the last ten years, numerous studies have demonstrated that infection with hepatitis C virus is involved in the pathogenesis of most mixed cryoglobulinemia. This review analyzes the main published data on hepatitis C virus-mixed cryoglobulinemia, the role of liver alterations, the predictive factors associated with MC production in HCV patients and its symptomatic nature or not, and the different types of vasculitis associated with HCV chronic infection.

Non-traumatic stereotyped postictal purpura is rare. A 25-year-old woman presented with right facial, cheek and periorbital purpuric eruptions that occurred after secondarily generalized tonic-clonic seizures. The stereotyped, invariably right-sided facial skin eruption, which resolved in 48 hours, falsely raised concerns of spousal abuse. Possible pathophysiological mechanisms include: (a) valsalva-induced capillary hypertension with secondary purpura, (b) ictal corticolimbic stimulation of the autonomic nervous innervation of facial vasomotor structures, and (c) trigeminal-mediated local release of vasoactive substances. Although rare, such stereotyped patterns of purpura should be recognized to avoid incorrect attribution of spousal abuse.

Psychogenic purpura, also known as recurrent painful bruising or autoerythrocyte sensitization syndrome (Gardner-Diamond syndrome) is usually associated with emotionally disturbed patients. It is a troublesome entity for both patient and physician since extensive work-ups yield no diagnosis. We describe two females in their early twenties with recurrent painful bruising and diverse accompanying symptoms which appeared after physical trauma. One of the patients developed a bruise after intradermal injection of her own blood (with no reaction to saline injection). One patient had a personality (borderline) disorder, the other a factitious disorder. Punch biopsies revealed a perivascular inflammatory infiltrate, erythrocyte extravasation and no vasculitis. Psychogenic hemorrhagic disorders are uncommon yet must be considered in the differential diagnosis of purpura. Patients are usually young emotionally troubled females who present painful recurrent bruises on extremities frequently after trauma or surgery. Autoimmune mechanisms and increased cutaneous fibrinolytic activity have been implicated, although further studies are needed. Correct diagnosis is important to avoid aggressive and even mutilating treatments, and an adequate comprehension of these purpuras is important for the attending physician.