[Is this iatrogenic pathology or rare disease?] / Yatrogennaya patologiya ili redkaya bolezn'?

The article is devoted to the problem of diagnosis and treatment strategy of Buerger's disease rarely found in the expert and clinical practice, that is inflammatory disease of unknown etiology, affecting mainly small and medium arteries and veins of limbs. Vascular surgeons around the world have been solving this problem for many years, both in terms of timely diagnosis of this disease and its proper pathogenic treatment. The authors of the article described an expert case of Buerger's disease larvated course in 15-years-old girl, which primarily was mistakenly assessed by specialists as iatrogenic pathology of right forearm vessels in injecting aminazin solution that, according to the clinicians' opinion, led to dry gangrene formation of right wrist and its subsequent amputation. The results of the forensic histological study and retrospective analysis of all child's medical documents allowed to correctly diagnose this rare pathology but only on the stage of commission forensic medical examination in the framework of the initiated criminal proceeding against several leading medical organizations in Saint-Petersburg with a pediatric profile. The authors noted the role of routine medical manipulation in manifestation of larvated pathologic process in a teenage girl in addition to full health and well-being. The objective of present article is devoted to understanding this problem.

Evaluation of radiological and temporal characteristics of acute appendicitis on the non-enhanced computed tomography images.

PURPOSES: To investigate the relationships and interactions between temporal and radiological features of gangrene and perforation of inflamed appendices. METHODS: A total of 402 patients were included who underwent laparoscopic appendectomies between January 1, 2016 and March 30, 2020 and had pathologically proved acute appendicitis and preoperative non-enhanced CT examinations. The radiological features (appendix diameter, appendicolith, appendiceal intraluminal gas, periappendiceal gas, periappendiceal fat stranding/fluid, and short axial diameter of the mesenteric lymph nodes) were obtained from the preoperative CT images of 382 patients with visible appendices. Clinical parameters and temporal variables (pre-CT delay, preoperative delay, estimated complication delay, symptom delay, and system delay) were recorded. RESULTS: Among simple/suppurative, gangrenous, and perforated appendicitis, the radiological characteristics except for short axial diameters of lymph nodes, and the temporal variables other than system delay were significantly different. The Cox regression analysis identified the appendicolith as the independent risk factor for both gangrene and perforation of inflamed appendices by using the preoperative delay or estimated complication delay. By the preoperative delay, the median time for gangrene and perforation was 76.23 (95%CI 73.89-78.58) h and 77.55 (95%CI 74.12-80.98) h, respectively, if appendicolith was present. If estimated complication delay was used as the elapsed time and the appendicolith was perceptible, the median time for gangrene and perforation and was 72.33 (95%CI 62.93-81.74) h and 75.07 (95%CI 69.48-80.65) h, respectively. CONCLUSION: There were interactions between the time evolution and radiological features of acute appendicitis. The evaluation of gangrene and perforation rate of acute appendicitis could be benefitted from combining the preoperative delay/estimated complication delay with CT characteristics in the preoperative urgent radiological analysis.

[Progress in diagnosis and treatment of gangrenous cholecystitis].

Gangrenous cholecystitis is a kind of acute cholecystitis, whose course of disease progresses rapidly, early diagnosis is difficult and mortality is high, and clinicians are prone to misdiagnosis and missed diagnosis in clinical work.However, gangrenous cholecystitis has been ignored in various guidelines.This paper systematically summarized the pathogenesis, pathological manifestations, epidemiology, clinical diagnosis and treatment of gangrenous cholecystitis, hoping to provide a complete and clear diagnosis and treatment process for clinicians.

Acral Gangrene: Ugly Cousin of "COVID Toes" in Multisystem Inflammatory Syndrome in Children Associated with SARS-CoV-2?

A spectrum of dermatologic manifestations has been reported in multisystem inflammatory syndrome in children associated with SARS-CoV-2 infection. We report 2 patients with multisystem inflammatory syndrome in children and severe cardiovascular dysfunction who developed acral gangrene. Both responded well to therapy and recovered in the follow-up.

Cardiovascular, hematological and neurosensory impact of COVID-19 and variants.

OBJECTIVE: The purpose of this article was to review our clinical experience with COVID-19 patients observed in the Cardiovascular Division of Pompidou Hospital (University of Paris, France) and the Department of Neurology of the Eastern Piedmont University (Novara, Italy), related to the impact on the cardiovascular, hematological, and neurologic systems and sense organs. PATIENTS AND METHODS: We sought to characterize cardiovascular, hematological, and neurosensory manifestations in patients with COVID-19 and variants. Special attention was given to initial signs and symptoms to facilitate early diagnosis and therapy. Indications of ECMO (extracorporeal membrane oxygenation) for cardiorespiratory support were evaluated. RESULTS: Preliminary neurosensorial symptoms, such as anosmia and dysgeusia, are useful for diagnosis, patient isolation, and treatment. Early angiohematological acro-ischemic syndrome includes hand and foot cyanosis, Raynaud digital ischemia phenomenon, skin bullae, and dry gangrene. This was associated with neoangiogenesis, vasculitis, and vessel thrombosis related to immune dysregulation, resulting from "cytokine storm syndrome". The most dangerous complication is disseminated intravascular coagulation, with mortality risks for both children and adults. CONCLUSIONS: COVID-19 is a prothrombotic disease with unique global lethality. A strong inflammatory response to viral infection severely affects cardiovascular and neurological systems, as well as respiratory, immune, and hematological systems. Rapid identification of acro-ischemic syndrome permits the treatment of disseminated intravascular coagulation complications. Early sensorial symptoms, such as gustatory and olfactory loss, are useful for COVID-19 diagnosis. New variants of SARS-CoV-2 are emerging, principally from United Kingdom, South Africa, and Brazil. These variants seem to spread more easily and quickly, which may lead to more cases of COVID.

Cutaneous calciphylaxis of the glans penis presenting as a gangrenous ulceration.

Report _Case Presentation X Photo Vignette _Letter Authors declare that the contents of this article are their own original unpublished findings. Title: Cutaneous calciphylaxis of the glans penis presenting as a gangrenous ulceration Authors: Marie Danset, Cécile Lesort, Denis Jullien, Jean Kanitakis Affiliations: Dermatology Department, Edouard Herriot Hospital, Hospices Civils de Lyon, Claude Bernard Lyon I University, Lyon, France Corresponding Author: Jean Kanitakis, Department of Dermatology, Edouard Herriot Hospital Group, 69437 Lyon Cedex 03, France, Tel: 33-472110301, Email: jean.kanitakis@univ-lyon1.fr Abstract: Calciphylaxis is a rare microvascular disorder causing necrotic skin ulcers. It is characterized by deposits of calcium within vascular walls but its precise pathogenesis remains poorly understood. A major risk factor is end-stage renal disease on dialysis. We report a 67-year-old man with calciphylaxis revealed by an unusual necrotic ulcer of the glans penis. The patient also presented with bilateral panniculitis of the thighs and a calf ulcer. All those lesions were painful, highlighting the value of pain as a diagnostic clue. Penile involvement of calciphylaxis is rare and biopsy is often avoided in this area. However, rapid diagnosis of calciphylaxis is important because early treatment has a better chance of being successful. Our patient's condition deteriorated rapidly with development of bilateral retinal artery occlusion and he died shortly thereafter. This case further highlights the fact that calciphylaxis is a systemic vascular disease with an ominous prognosis.

Cutaneous manifestations of antiphospholipid syndrome.

Antiphospholipid syndrome (APS) is an acquired thrombophilic disorder in which autoantibodies are produced against a variety of phospholipids and phospholipid-binding proteins. The purpose of this article is to review cutaneous findings in patients with APS diagnosis. An overview regarding prevalence, description, pathogenesis and histopathology, are described for cutaneous manifestations of APS.

Symmetrical peripheral gangrene in critical illness.

Symmetrical peripheral gangrene (SPG) is a disabling complication that affects a small proportion of patients who survive critical illness. Its pathogenesis reflects profoundly disturbed procoagulant-anticoagulant balance in susceptible tissue beds secondary to circulatory shock (cardiogenic, septic). There is a characteristic SPG triad: (a) shock (hypotension, lactic acidemia, normoblastemia, multiple organ dysfunction), (b) disseminated intravascular coagulation (DIC), and (c) natural anticoagulant depletion (protein C, antithrombin). In recent years, risk factors for natural anticoagulant depletion have been identified, most notably acute ischemic hepatitis ("shock liver"), which is seen in at least 90% of patients who develop SPG. Moreover, there is a characteristic time interval (2-5 days, median 3 days) between the onset of shock/shock liver and the beginning of ischemic injury secondary to peripheral microthrombosis ("limb ischemia with pulses"), reflecting the time required to develop severe depletion in hepatically-synthesized natural anticoagulants. Other risk factors for natural anticoagulant depletion include chronic liver disease (e.g., cirrhosis) and, possibly, transfusion of colloids (albumin, high-dose immunoglobulin) lacking coagulation factors. A causal role for vasopressor therapy is unproven and is unlikely; this is because critically ill patients who develop SPG do so usually after at least 36-48 hours of vasopressor therapy, implicating a time-dependent pathophysiological mechanism. The most plausible explanation is a progressive time-dependent decline in key natural anticoagulant factors, reflecting ongoing DIC ("consumption"), proximate liver disease whether acute or chronic ("impaired production"), and colloid administration ("hemodilution"). Given these evolving concepts of pathogenesis, a rationale approach to prevention/treatment of SPG can be developed.

Digital Ulcers in Systemic Sclerosis.

Digital ulcers (DU) are one of the most common complication of Systemic Sclerosis (SSc)-related vasculopathy and represent an important burden for the patients as well as for the society. Still today there is no agreement on the definition, classification and cathegorization of DU even if they are of pivotal importance in clinical practice, for treatment choice and prognostic outcomes, as well as for clinical trials. DU management requires a dedicated multidisciplinary team, that must remain ever vigilant for the development of infective complications and gangrene throughout their disease course, as well as patient education that is crucial to obtain the best compliance to assure the success of the treatment. Currently several drugs are available for DU treatment but in the future, more investigations will be needed to ameliorate the approach and the systemic and local therapies.

Axillary artery thrombosis resulting in upper limb amputation as a COVID-19 sequela.

Novel COVID-19 continues to intrigue medical professionals with its varied presentations. Though it affects the respiratory tract primarily, thrombogenesis has been the Achilles' heel. A 44-year-old man diagnosed with COVID-19 presented with upper limb pain at a local hospital and was found to have thrombosis of the right axillary artery. Despite a successful embolectomy at the local hospital, there was re-occlusion of the axillary artery and the limb became ischaemic. He was referred to our institution by which time the limb became gangrenous above the elbow and had to be amputated. Extensive sloughing of the nerves was also seen in the local area. Hypercoagulability presenting with various manifestations is common in COVID-19 and needs early anticoagulation. We present this asymptomatic patient who lost a limb to this COVID-19 sequelae.

Severe ischaemic gangrene of scalp in an extreme preterm: a fatal case of combined aetiology.

A 24+5-week preterm neonate with a severe scalp lesion was admitted to the neonatal intensive care unit (NICU) after caesarean section due to maternal chorioamnionitis (MC). An Arabin pessary had been inserted in addition to a previous cervical cerclage due to cervix insufficiency at 21+5 weeks of pregnancy (wp). At 23+5 wp, preterm rupture of membranes was evidenced. Both devices were kept to provide fetal viability. On 24+4 wp, she developed MC. Urgent caesarean section was performed. Transvaginal manual manipulation was required during the procedure. On NICU, she presented severe shock which required high-dose vasopressors and blood products. Following surgical repair, a bilateral grade IV intracranial haemorrhage was evidenced. Subsequently, it was agreed to withdraw life support. We hypothesise that MC and local infection could have acted as predisposing factors, with the presence of a pessary in the setting causing uterine contractions and its manipulation acting as a precipitating factor.

Echtyma gangrenosum caused by coinfection with group A Streptococcus and Staphylococcus aureus: an emerging etiology? Case reports and literature review.

Ecthyma gangrenosum (EG) is a potentially lethal skin infection, most commonly due to Pseudomonas aeruginosa with bacteremic dissemination and affecting mostly immunocompromised patients. We present two cases of EG in two men in Belgium recently admitted to our hospital, caused by a suspected coinfection by group A Streptococcus and Staphylococcus aureus, with a cutaneous dissemination, in which multiple impetigo lesions were the portal of entry. The first patient had no risk factors nor immunodeficiency, but the second was a homeless man with drug and alcohol abuse and advanced HIV infection. Early management of the condition is crucial, with initial broad spectrum antibiotherapy, rapidly narrowed down to the germs identified and skin lesion debridement if necessary. Any immunocompromising condition must be ruled out in any patient suffering from EG.

Synchronous rectal prolapse and midgut volvulus presenting as complete small bowel gangrene.

<b>Introduction:</b> Adult midgut malrotation is very rare. The presentation in adults is mostly subtle; the diagnosis is often made on imaging. <br><b>Case report:</b> A 32-year-old man presented with a 3-day history of an irreducible painful mass protruding per rectum, followed by abdominal pain and constipation. The patient was febrile and toxic, with marked signs of peritonitis and complete fullthickness irreducible rectal prolapse. Abdominal radiographs showed multiple air-fluid levels. A diagnosis of irreducible rectal prolapse with intestinal obstruction was made. Laparotomy revealed complete small bowel gangrene, abnormal rotation of the small bowel 180° around the mesenteric root, hypermobile duodenojejunal flexure and ileocaecal junction and an abnormally mobile caecum lying in the central abdomen. Abnormal mobility of the sigmoid colon with a large mesocolon, and a large irreducible rectosigmoid intussusception, was also noted. Our patient is probably the first case in literature, which may prompt awareness of simultaneously-occurring disorders of fixation, and preventive steps.

Histopathological findings in a COVID-19 patient affected by ischemic gangrenous cholecystitis.

BACKGROUND: Since its first documentation, a novel coronavirus (SARS-CoV-2) infection has emerged worldwide, with the consequent declaration of a pandemic disease (COVID-19). Severe forms of acute respiratory failure can develop. In addition, SARS-CoV-2 may affect organs other than the lung, such as the liver, with frequent onset of late cholestasis. We here report the histological findings of a COVID-19 patient, affected by a tardive complication of acute ischemic and gangrenous cholecystitis with a perforated and relaxed gallbladder needing urgent surgery. CASE PRESENTATION: A 59-year-old Caucasian male, affected by acute respiratory failure secondary to SARS-CoV-2 infection was admitted to our intensive care unit (ICU). Due to the severity of the disease, invasive mechanical ventilation was instituted and SARS-CoV-2 treatment (azithromycin 250 mg once-daily and hydroxychloroquine 200 mg trice-daily) started. Enoxaparin 8000 IU twice-daily was also administered subcutaneously. At day 8 of ICU admission, the clinical condition improved and patient was extubated. At day 32, patient revealed abdominal pain without signs of peritonism at examination, with increased inflammatory and cholestasis indexes at blood tests. At a first abdominal CT scan, perihepatic effusion and a relaxed gallbladder with dense content were detected. The surgeon decided to wait and see the evolution of clinical conditions. The day after, conditions further worsened and a laparotomic cholecystectomy was performed. A relaxed and perforated ischemic gangrenous gallbladder, with a local tissue inflammation and perihepatic fluid, was intraoperatively met. The gallbladder and a sample of omentum, adherent to the gallbladder, were also sent for histological examination. Hematoxylin-eosin-stained slides display inflammatory infiltration and endoluminal obliteration of vessels, with wall breakthrough, hemorrhagic infarction, and nerve hypertrophy of the gallbladder. The mucosa of the gallbladder appears also atrophic. Omentum vessels also appear largely thrombosed. Immunohistochemistry demonstrates an endothelial overexpression of medium-size vessels (anti-CD31), while not in micro-vessels, with a remarkable activity of macrophages (anti-CD68) and T helper lymphocytes (anti-CD4) against gallbladder vessels. All these findings define a histological diagnosis of vasculitis of the gallbladder. CONCLUSIONS: Ischemic gangrenous cholecystitis can be a tardive complication of COVID-19, and it is characterized by a dysregulated host inflammatory response and thrombosis of medium-size vessels.

Severe acute dried gangrene in COVID-19 infection: a case report.

OBJECTIVE: Coronavirus disease 2019 (COVID-19) related coagulopathy may be the first clinical manifestation even in non-vasculopathic patients and is often associated with worse clinical outcomes. CASE PRESENTATION: A 78 years old woman was admitted to the Emergency Unit with respiratory symptoms, confusion and cyanosis at the extremity, in particular at the nose area, hands and feet fingers. A nasal swab for COVID-19 was performed, which resulted positive, and so therapy with doxycycline, hydroxychloroquine and antiviral agents was started. At admission, the patient was hemodynamically unstable requiring circulatory support with liquids and norepinephrine; laboratory tests showed disseminated intravascular coagulation (DIC). During hospitalization, the clinical condition worsened and the cyanosis of the nose, fingers, and toes rapidly increased and became dried gangrene in three days. Subsequently, the neurological state deteriorated into a coma and the patient died. DISCUSSION: In severe cases, COVID-19 could be complicated by acute respiratory disease syndrome, septic shock, and multi-organ failure. This case report shows the quick development of dried gangrene in a non-vasculopathic patient, as a consequence of COVID-19's coagulopathy and DIC. CONCLUSIONS: In our patient, COVID-19 related coagulopathy was associated with poor prognosis.

Acral vascular syndrome during an immune checkpoint inhibitor.

Immune checkpoint inhibitors, including antiprogrammed death cell protein 1 (anti-PD-1) and anti cytotoxic T-lymphocyte-associated protein 4 (anti-CTLA-4), have been associated with a range of autoimmune-related side effects since their introduction in cancer treatment. Small vessel digital necrosis, referred to as the acral vascular syndrome, is a rare but serious complication that can result in loss of digits. Here we present a case report of acral vascular syndrome and review possible aetiologies. A 45- year-old woman with invasive ductal carcinoma of the left breast presented to the emergency department during neoadjuvant treatment with carboplatin, docetaxel and pembrolizumab with complaints of severe pain in her right third digit. She had physical findings consistent with ischaemic necrosis and gangrene of the distal phalanx. Angiography demonstrated Raynaud's phenomenon in the distal portion of the digits. Laboratory testing showed a weakly positive RNA polymerase III antibody level. Her case resulted in surgical amputation of her affected digit after partial resolution of symptoms with prednisone, vasodilators and antibiotics.