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Abstract: A femoral hernia is an uncommon and acquired hernia in the groin. Its incidence in adults is 2%-8% of all abdominal wall hernias, and it has a female-to-male ratio of 1.8:1. It is usually found in elderly patients and is associated with increased morbidity due to delays in diagnosis leading to a high incidence of incarceration and strangulation. Accurate preoperative diagnosis of femoral hernia is challenging, especially in obese patients in whom a small femoral hernia can be hidden under the groin fat. Unlike an inguinal hernia, it rarely reduces on its own and if asymptomatic and small, is often unnoticed by the elderly obese patient. Femoral hernia is often unsuspected and overlooked in males as it is predominant among females. Delay in diagnosis can lead to intestinal gangrene and high morbidity. We present a case of an incarcerated left femoral hernia in an elderly obese male who presented with acute intestinal obstruction. He was managed with resection of the gangrenous segment and double barrel ileostomy. Although uncommon in males, a femoral hernia has a high incidence of strangulation, and therefore should always be ruled out as a cause of acute intestinal obstruction in elderly patients. Therefore, never forget to examine the groin in case of intestinal obstruction., (Copyright © 2024 Copyright: © 2024 Nigerian Journal of Clinical Practice.)

Background: Peripheral T cell lymphoma (PTCL), not otherwise specified (NOS) is a heterogenous group of predominantly nodal T cell lymphomas that generally presents with lymphadenopathy with or without extra nodal involvement. Acral vascular syndrome clinically presents as digital ischemia with Raynaud's phenomenon and acral cyanosis. Although, this condition is commonly associated with connective tissue disorder, smoking and vasculitis, its association with lymphoid malignancy is very rare. Here, we present a case report of a patient with digital gangrene of all toes and fingers as a presenting symptom of PTCL-NOS., Case Description: A 62 year old male presented with digital ischemia associated with pain, low grade fever, loss of appetite and significant weight loss of 6 kilograms over a period of 3 months. On examination, he was found to have bilateral inguinal and axillary lymph nodes with gangrenous changes over toes and fingers but peripheral pulses were palpable. On evaluation he had anemia, elevated ESR and CRP. CT angiogram revealed thinned out digital arteries with multifocal areas of narrowing. Patient was screened for other causes of digital gangrene and was tested negative for ANCA, ANA, cryoglobulins and viral markers. Lymph node biopsy with IHC was suggestive of peripheral T-cell lymphoma-NOS and was started on CHOP regimen. Lymph nodes size decreased and gangrenous changes resolved., Conclusion: Though digital ischemia is a rare paraneoplastic presentation of lymphoma, it should be considered if there is a rapid progression of gangrene. Early initiation of chemotherapy may result in the reduction of further progression of digital gangrene and thus prevent permanent disability. In our patient, progression of gangrene was prevented even though it was an aggressive variant of T cell lymphoma., (© Journal of the Association of Physicians of India 2024.)

Competing Interests: The authors declare no conflict of interest.

Rest pain, ulceration and gangrene are hallmark features of chronic limb-threatening ischaemia (CLTI). Wound healing can be challenging, and this is compounded by an inability to measure lower limb perfusion via non-invasive tools such as toe pressure (TP). Novel perfusion tests, such as pedal acceleration time (PAT), may overcome some limitations. This study aimed to quantify the proportion of patients with CLTI that were unable to undergo TP measurement. Over a three-year duration, 344 consecutive patients with CLTI underwent PAT assessment (403 limbs). Overall, 32% of limbs were unable to undergo first toe TP, and 12.9% were unable to undergo first and second toe TP due to forefoot/digit amputation or tissue loss. Inability to measure first toe TP disproportionately impacted CLTI patients with diabetes compared to patients without diabetes (39.6% limbs (106/268); vs. 17% limbs (23/135); p < 0.001). Novel modalities may provide a useful tool for assessing perfusion in CLTI., (© 2023 The Authors. Wound Repair and Regeneration published by Wiley Periodicals LLC on behalf of The Wound Healing Society.)

Purpose: Distinguishing between perforated/gangrenous from uncomplicated appendicitis in children helps management. We evaluated hyponatremia as a new diagnostic marker for perforated/gangrenous appendicitis in children., Methods: A prospective study including all children with acute appendicitis who underwent appendectomy at our institution from May 2021 to May 2023. Medical history and clinical criteria were analyzed. All blood samples were taken upon admission including serum inflammatory markers and electrolytes. Patients were divided into two groups (Group I: uncomplicated and Group II: perforated/gangrenous), and data between both groups were compared., Results: The study included 153 patients [Group I: 111 (73%), Group II: 42 (27%)]. Mean serum sodium concentration in children with perforated/gangrenous appendicitis was significantly lower compared to children with uncomplicated appendicitis (131.8 mmol/L vs. 138.7 mmol/L; p < 0.001). The ROC curve of preoperative sodium level to differentiate between perforated/gangrenous and uncomplicated appendicitis revealed an AUC of 0.981. The cut-off-value of sodium level < 135 mmol/L identified perforated/gangrenous appendicitis with a sensitivity of 94% and a specificity of 91% (p < 0.001). Predictive factors of perforated/gangrenous appendicitis were: age less than 5 years (12% vs. 3%; p = 0.02), experiencing symptoms for more than 24 h (100% vs. 58%; p < 0.001), body temperature more than 38.5 °C (52% vs. 13%; p < 0.001), a serum sodium level less than 135 mmol/L (90% vs. 6%; p < 0.001), and a CRP serum level more than 50 mg/L (71% vs. 17%; p < 0.001)., Conclusions: Hyponatremia, upon admission, is a novel, objective biochemical marker that can identify perforated/gangrenous appendicitis in children. We advocate that the assessment of serum sodium level should be added to the diagnostic algorithm in children with suspected acute appendicitis. Surgical intervention in patients with hyponatremia should not be delayed, and non-operative management should be avoided., (© 2023. The Author(s).)

Digital gangrene is frequently encountered in patients who have diabetes with peripheral vascular compromise, with or without superimposed infection. Preoperative laboratory values and radiographic images are important to determine a proper course of action. Equally important is a thorough history taking to confirm or rule out systemic entities and preexisting conditions that can aggravate or predispose one to the development of digital gangrene. A patient with diabetes presented with a rare and unusual case of digital gangrene, as he clinically had strong pedal pulses. Preoperative workup revealed a suspicion of polycythemia, which was subsequently confirmed. The patient underwent several days of phlebotomy until his hemoglobin and hematocrit levels were brought down to optimized levels before a digital amputation was performed. He went on to heal uneventfully, and he is currently being closely followed by oncology/hematology colleagues with periodic phlebotomy.

Rickettsial infections are an important cause of acute febrile illness in developing countries. They can rarely present with cutaneous manifestations such as gangrene. Here we report a 12-year-old boy who presented with acute undifferentiated fever, multiorgan dysfunction, and gangrene of bilateral toes and ear lobes. The diagnosis was made by serologic testing of paired sera and clinical recovery occurred with doxycycline., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

The pandemic of a new coronavirus infection has made certain adjustments to modern emergency medicine. Systemic endothelial dysfunction following COVID-19 largely determines hemostatic disorders. Numerous studies revealed that intense platelet adhesion followed by platelet aggregates in COVID-19 patients and functional disorders of fibrinolysis system are combined with activation and severe endothelial dysfunction. The last one inevitably leads to thrombosis and adverse vascular events. There are a lot of studies devoted to deep vein thrombosis and fatal massive pulmonary embolism under COVID-19 infection. However, there are no descriptions of mesenteric thrombosis followed by intestinal wall necrosis. Our experience is based on the treatment and follow-up of 14 patients with venous gangrene of small intestine under COVID-19-induced severe endothelial dysfunction. We present a 40-year-old man with coronavirus infection complicated by ileum gangrene and subsequent favorable outcome.

With the onset of the SARS-CoV-2 pandemic, Kawasaki Disease (KD) has come to the fore with its many atypical manifestations. Atypical clinical neurological, ophthalmological, musculoskeletal, gastrointestinal and pulmonary manifestations in a febrile child with raised markers should prompt the clinician to think of Kawasaki disease. Peripheral gangrene is a rare atypical manifestation of KD reported in infancy. We present a three-and-a-half-year-old boy with extensive gangrene all four limbs and face along with purpura fulminans. He was successfully treated with two doses of intravenous immunoglobulin (IVIG) and infliximab, with no residual gangrene. This case highlights that very severe forms of Kawasaki disease require IVIG, pulse steroids as well as infliximab for adequate control and complete resolution of the disease.

Takayasu arteritis (TA) is a large-vessel vasculitis most commonly affecting women of childbearing age. The disease process is usually slow and smoldering, presenting over months to years. Digital gangrene is an uncommon manifestation of TA because of the formation of good collateral circulation. Similarly, although pulmonary artery involvement is well described, pulmonary parenchymal involvement is very rare. We are reporting a case of a young girl with TA presenting with digital gangrene and pulmonary consolidation, which was treated successfully with a combination of aggressive systemic immunosuppression and anti-coagulants. The possible mechanism for gangrene along with the confounding diagnostic possibility of co-existing tuberculosis have been discussed., (© Japan College of Rheumatology 2021. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

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.

Background and Aims: The novel coronavirus disease (COVID-19) caused by SARS-CoV-2 has turned the world topsy-turvy since its onset in 2019. The thromboinflammatory complications of this disease are common in critically ill patients and associated with poor prognosis. Symmetrical peripheral gangrene (SPG) is characterized by symmetrical distal gangrene in absence of any large vessel occlusion or vasculitis and it is usually associated with critical illness. Our aim was to report the clinical profile and outcome of patients diagnosed with SPG associated with COVID-19. To the best of our knowledge, no such similar cases have been reported till date., Methods: In this case series, we have discussed the clinical presentation, laboratory parameters and outcome in a series of two patients of SPG associated with COVID-19 and also compared those findings. Due to paucity of data, we also reviewed the literature on this under-diagnosed and rarely reported condition and association., Results: Two consecutive patients (both males, age range: 37-42 years, mean: 39.5 years) were admitted with the diagnosis of COVID-19 associated SPG. Both patients had clinical and laboratory evidence of disseminated intravascular coagulation (DIC). Leucopenia was noted in both patients. Despite vigorous therapy, both patients succumbed to their illness within a fortnight of admission., Conclusion: SPG in the background of COVID-19 portends a fatal outcome. Physicians should be aware of its grim prognosis., Competing Interests: Declaration of competing interest No, there are no competing interests for any author., (Copyright © 2021 Diabetes India. Published by Elsevier Ltd. All rights reserved.)

Aims: Identify the prevalence, risk factors and outcomes of lower extremity ischemic complications., Methods: A systematic review was conducted by searching PubMed and SCOPUS databases for SARS-CoV-2, COVID-19 and peripheral arterial complications., Results: Overall 476 articles were retrieved and 31 articles describing 133 patients were included. The mean age was 65.4 years. Pain and gangrene were the most common presentation. Hypertension (51.3%), diabetes (31.9%) and hypercholesterolemia (17.6%) were associated co-morbidities. Overall, 30.1% of patients died and amputation was required in 11.8% patients., Conclusions: COVID-19 patients with diabetes or hypertension are susceptible for lower limb complications and require therapeutic anti-coagulation., (Copyright © 2021 Diabetes India. Published by Elsevier Ltd. All rights reserved.)

At the onset of the COVID-19 crisis, a 63-year-old woman with multiple life-limiting comorbidities was referred with a necrotic infected left breast mass on a background of breast cancer treated with conservation surgery and radiotherapy 22 years previously. The clinical diagnosis was locally advanced breast cancer, but four separate biopsies were non-diagnostic. Deteriorating renal function and incipient sepsis and endocarditis resulted in urgent salvage mastectomy during the peak of the COVID19 pandemic. The final diagnosis was infected ischaemic/infarcted breast (wet gangrene) secondary to vascular insufficiency related to diabetes, cardiac revascularisation surgery and breast radiotherapy.

Cold agglutinin disease (CAD) is extremely rare in children. We report an 8-year-old boy who presented with gangrene of right foot with hypertension and absent lower limb pulses. Blood peripheral smear evidence of autoagglutination and falsely elevated red blood cell indices were suggestive of CAD and on subsequent investigations he was found to have high titres of cold agglutinin antibodies. He also had evidence of pneumonia on chest X-ray and serology for mycoplasma was positive. Computed tomography angiography showed multifocal thrombotic occlusion in bilateral popliteal arteries. He was effectively managed using antimicrobials, warm clothing, aspirin, anticoagulation and corticosteroids. He remains clinically well on follow-up and had no recurrence. CAD presenting with peripheral gangrene is extremely unusual. A careful look at peripheral blood smear gives an initial diagnostic clue. CAD triggered by infection is often self-limiting and requires supportive care., (Copyright © 2021. Published by Elsevier GmbH.)

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., (Copyright © 2021. Published by Elsevier Ltd.)

Background: Acral vascular syndrome clinically presents as digital ischemia with Raynaud's phenomenon and erythromelalgia but can be rarely seen in the malignant condition. Patients may present pain, permanent digital blanching or cyanosis, and desquamation or ulceration of the fingers. Acral vascular syndrome is rarely associated with lymphoid neoplasm and is associated with smoking, autoimmune connective tissue diseases, and vasculitis. Here, we describe a 79-year-old female who was diagnosed with vitiligo and peripheral T cell lymphoma Lennert type stage 4 with anemia of chronic disease with digital acral vascular syndrome., Case Presentation: A 79-year-old female with vitiligo presented with gangrene of the distal extremities associated with pain and intermittent fever for 2 months. On evaluation, she was found to have anemia of chronic disease and generalized lymphadenopathy and diagnosed as peripheral T cell lymphoma Lennert type with bone marrow involvement and digital acral vascular syndrome., Conclusion: Acral vascular syndrome can be a presentation of lymphoma; if intervened earlier, the patient can be saved from the amputation of fingers or affected limb. Though it is a rare presentation of lymphoma, it should be considered if there is a rapid progression of gangrene. Early initiation of chemotherapy may result in the reduction of further progression of digital gangrene and thus prevents functional dependence on caregivers. In our patient, gangrene of other fingers was prevented even though it is an aggressive variant of T cell lymphoma.

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.

Neonates are at highest risk of thrombosis among paediatric patients. The relative prothrombotic state in a well neonate is compensated by other factors preventing spontaneous thrombosis; however, in a neonate with genetic predisposition, the balance is tilted predisposing them to a life-threatening thrombotic episode. We describe a rare case of methylenetetrahydrofolate reductase A1298C (homozygous) mutation along with plasminogen activator inhibitor (4G) mutation in a neonate who developed bilateral lower limb gangrene following thrombosis of the iliac vessels without any triggering factor. The neonate underwent thrombectomy as debulking measure along with thrombolytic therapy followed by unfractionated heparin and low-molecular-weight heparin which is still being continued along with oral aspirin. The neonate had to undergo amputation of both the involved lower limbs in view of dry gangrene. This case highlights that the dual mutations causing the prothrombotic state predispose the individual to the spontaneous life-threatening thrombotic episode as compared with the single mutation., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

The diagnosis of gangrenous acute cholecystitis represents a diagnostic challenge for the physician and is rarely identified preoperatively. We report a longitudinal prospective study in 180 patients who underwent cholecystectomy for acute cholecystitis. A ROC curve was obtained to determine the preoperative cut-off for various biomarkers (neutrophil to lymphocyte ratio [NLR], C-reactive protein [CRP], platelet to lymphocyte ratio [PLR], lactate and procalcitonin) and their association with both preoperative and postoperative findings. The area under the curve (AUC) for NLR, CRP, PLR, lactate and procalcitonin was 0.75, 0.8, 0.65 and 0.6, respectively. NLR > 5 and CRP > 100 are still independent factors for gangrene (adjusted odds ratio [OR], 2 and 2.1, respectively).

Presented in this article is a clinical case report regarding treatment of a patient with deep vein thrombosis complicated by venous gangrene having developed 10 days after the onset of the disease. Conservative therapy (infusion of colloids and crystalloids, anticoagulants, agents improving microcirculation, venotonics, nonsteroidal anti-inflammatory drugs, elevated position of the limb) made it possible to stabilize the patient's condition, but not improving haemodynamics of the affected limb. A decision was made to use endovascular techniques. Treatment was carried out in three stages. The first stage during 48 hours consisted in regional catheter thrombolysis with urokinase, exerting a minimal clinical effect. The second stage was percutaneous mechanical thrombectomy after which the diameter of thrombosed veins became free by half, with the beginning of disease regression. The third stage consisted in venous stenting of residual stenosis of the iliac vein, resulting in normalization of the venous outflow from the affected limb. A conclusion was drawn on feasibility of combined use of regional thrombolysis, percutaneous mechanical thrombectomy, and venous stenting in treatment of venous gangrene.

Competing Interests: Competing interests: None declared.

We describe an autopsy case of a 75-year-old female with limited cutaneous systemic sclerosis (lcSSc) and gangrene due to macrovascular involvement. She was diagnosed with lcSSc complicated with pulmonary arterial hypertension and digital ulcers 9 years before admission. She had recurrent and refractory lower limb ulcers (LLUs), and died because of sepsis caused by gangrene infection. Autopsy findings revealed severely thickened arterial walls of the visceral organs, consistent with vascular involvement of SSc. Systemic vascular involvement in lcSSc may progress in patients with LLUs who harbour several risk factors for vascular involvement.

Background: Ileo-sigmoid knotting is a rare cause of intestinal obstruction with a rapidly progressive course, for which expedient surgical intervention is required to prevent mortality. The aim of this study was to determine the characteristics, presentation, morbidity and mortality associated with ileo-sigmoid knotting at Parirenyatwa Group of Hospitals (PGH). To determine the preoperative diagnostic precision and management patterns of ileo-sigmoid knotting cases at PGH., Methods: A retrospective analysis was performed on patients operated on at Parirenyatwa Hospital with a diagnosis of ileo-sigmoid knotting between April 2011 and April 2018. Data inclusive of demographics, time to presentation and surgery, preoperative diagnosis, complications and in-hospital mortality was collected. The relationship between the duration of symptoms prior to surgery and incidence of both septic shock and transfusion were analysed., Results: Twenty-one cases of ileo-sigmoid knotting were identified for analysis. The median age was 37 years (range 18-65 years) with a 6:1 male to female ratio. Two of the three females included were pregnant. Twenty patients (95.2%) described an acute onset abdominal pain, with 83.3% experiencing the pain nocturnally, while asleep. The median duration of symptoms at presentation was 12.5 hours (range 2-39 hours). At admission, leucocytosis (WCC > 11x10³/dl) was noted in eleven patients (52.4%). Seventy-three per cent of patients were noted to have electrolyte derangements at presentation. Seven patients (33.3%) had recorded episodes of severe hypotension (SBP < 90) prior to surgery. The most common preoperative diagnosis, based on both clinical assessment and plain x-ray evaluation, was sigmoid volvulus (52.4%), with no preoperative diagnosis of ileo-sigmoid knotting being made. All patients had gangrenous small bowel, with 81% having a gangrenous sigmoid colon. All cases underwent small bowel resection and primary anastomosis plus Hartmann's procedure. Postoperatively, eleven patients (52.4%) developed septic shock, while 62% required blood transfusion. There was one (4.8%) early postoperative mortality., Conclusion: To avoid mortality, the diagnosis of ileo-sigmoid knotting should be entertained and the imperative of emergency surgery recognised in the young male or pregnant female patient with acute nocturnal onset abdominal pain, a rapidly deteriorating small bowel obstruction clinical picture and with radiological features suggestive of both small and large bowel obstruction., (Copyright© Authors.)

Catastrophic antiphospholipid antibody syndrome (CAPS) is a severe form of antiphospholipid antibody syndrome (APS) that sometimes represents the first manifestation of the later syndrome. The clinical manifestations of CAPS are relatively non-specific. Hence, the diagnosis may be delayed, resulting in high mortality. We herein present a case of a 40-year-old male who presented with rapid-onset renal failure, gangrene of finger and toe tips and hematological abnormalities with no underlying secondary cause for this complication. The symptoms were precipitated by febrile illness of short duration. A provisional diagnosis of CAPS was made and treatment instituted. With timely diagnosis and intervention, both the life of the patient and kidney function were salvaged. A high index of suspicion for CAPS is important as early treatment can be lifesaving.

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.

Competing Interests: None

Acute lupus myocarditis and pulmonary arterial hypertension (PAH) are rare complications associated with systemic lupus erythematosus (SLE). No previous reports have shown the coexistence of these disorders. Here we present a 41-year-old patient with SLE who concurrently developed severe acute lupus myocarditis and PAH with digital gangrene as an initial manifestation. Acute lupus myocarditis and PAH were successfully treated with prednisolone and intravenous cyclophosphamide pulse therapy (600-700 mg × 6) along with anticoagulant therapy. Catheter-directed thrombolysis was required for digital gangrene caused by vasculitis. Concurrent development of these rare disorders may represent a common mechanism such vasculitis as an underlining cause of SLE.

Diagnosis of calciphylaxis is crucial, yet its distinction from other vascular diseases can be challenging. Although vascular calcification and thrombosis are hallmarks of calciphylaxis, the incidence and patterns of these features in other vascular diseases have not been well characterized. The specificity of fine calcium deposits in vessel walls (identifiable on von Kossa [VK] stain only) and other extravascular calcifications is not entirely clear. We retrospectively examined the clinicopathologic features in calciphylaxis (n=27), gangrene and viable skin at amputation margin (n=20 each), chronic stasis (n=22), and thrombotic vasculopathy (n=19) to identify useful discriminators. Calcification of subcutaneous small vessels appreciable on hematoxylin and eosin stain was relatively specific for calciphylaxis, although sensitivity was low (56%). VK detected fine calcium deposits in vessel walls not appreciable on hematoxylin and eosin, however, specificity was limited by frequent finding of similar deposits in peripheral artery disease. Combining calcium deposits detected by VK and thrombosis of subcutaneous small vessels resulted in optimal sensitivity (85%) and specificity (88%) for calciphylaxis. Similar observations applied to medium-sized vessel calcification. Calcification of eccrine gland basement membranes, elastic fibers, and perineurium did not effectively distinguish calciphylaxis from other groups. Diffuse dermal angiomatosis was exclusively found in calciphylaxis in this study. In conclusion, VK is useful in enhancing the detection of vascular calcification and avoiding the false-negative diagnosis, but this finding requires concomitant subcutaneous small vessel thrombosis to support a diagnosis of calciphylaxis. Diffuse dermal angiomatosis should increase suspicion for underlying calciphylaxis and prompt deeper sampling in the appropriate clinical setting.

Diabetic foot gangrene with lower extremity ischemia can preclude amputation. However, wound treatment principles based on the Wagner classification system are lacking. We proposed the STAGE principle for the surgical management of diabetic foot wounds. The STAGE principle guides surgical intervention during the wound treatment of diabetic foot ulcers and emphasizes that "based on anatomical layers, the management focuses on blood supply and includes layer-by-layer incision to the infected area, maintenance of effective wound drainage, and step-by-step treatment of the wound." We applied the STAGE principle for the treatment of 7 patients with an ankle brachial index <0.5 and Wagner grade 4 diabetic foot gangrene. The average ankle brachial index was 0.42 (0.32-0.48; SD = 0.06), and male patients smoked an average of 1.28 packs/day (0.4-2; SD = 0.63). The average wound duration was 45.86 days (14-63 days; SD = 18.46). The average wound healing time was 8.86 months (5-13 months; SD = 2.36). The follow-up time was 37.71 months (3-84 months; SD = 25.04; median = 36 months). Patient 1 received endovascular interventional therapy twice for the lower extremity artery, and the wound healed. After 3 months of follow-up, the patient exhibited recurrence. After the third application of endovascular interventional therapy for the lower extremity artery, the blood supply was improved, and the wound healed after 1 month. In summary, the treatment of 7 cases of diabetic foot gangrene with severe lower extremity ischemia using the STAGE principle resulted in remarkable efficacy.

Background: Heparin-induced thrombocytopenia (HIT) is a prothrombotic disorder characterized by heparin-dependent antibodies that activate platelets (PLTs) via PLT FcγIIa receptors. "Autoimmune" HIT (aHIT) indicates a HIT subset where thrombocytopenia progresses or persists despite stopping heparin; aHIT sera activate PLTs strongly even in the absence of heparin (heparin-independent PLT-activating properties). Affected patients are at risk of severe complications, including dual macro- and microvascular thrombosis leading to venous limb gangrene. High-dose intravenous immunoglobulin (IVIG) offers an approach to interrupt heparin-independent PLT-activating effects of aHIT antibodies., Case Report: A 78-year-old male who underwent cardiopulmonary bypass for aortic dissection developed aHIT, disseminated intravascular coagulation, and deep vein thrombosis; progression to venous limb gangrene occurred during partial thromboplastin time (PTT)-adjusted bivalirudin infusion (underdosing from "PTT confounding"). Thrombocytopenia recovered with high-dose IVIG, although the PLT count increase began only after the third dose of a 5-day IVIG regimen (0.4 g/kg/day × 5 days). We reviewed case reports and case series of IVIG for treating HIT, focusing on various IVIG dosing regimens used., Results: Patient serum-induced PLT activation was inhibited in vitro by IVIG in a dose-dependent fashion; inhibition of PLT activation by IVIG was much more marked in the absence of heparin versus the presence of heparin (0.2 U/mL). Our literature review indicated 1 g/kg × 2 IVIG dosing as most common for treating HIT, usually associated with rapid PLT count recovery., Conclusion: Our clinical and laboratory observations support dose-dependent efficacy of IVIG for decreasing PLT activation and thus correcting thrombocytopenia in aHIT. Our case experience and literature review suggests dosing of 1 g/kg IVIG × 2 for patients with severe aHIT., (© 2019 AABB.)

A 14-year-old female patient presented with acute generalised abdominal pain and two attacks of non-bilious vomiting for 2 days. She visited the emergency department and at presentation she was pale, dyspnaeic and there was no jaundice. Abdominal examination showed moderate abdominal distension with generalised abdominal tenderness. The bowel sounds were negative on auscultation. Plain abdominal X-ray showed hugely distended stomach with no free air detected in the peritoneal cavity. During laparotomy there was huge distension and gangrene of the stomach involving the whole stomach up to the fundus. Total gastrectomy done with roux-en-y reconstruction of the gastrointestinal continuity. The histopathological study of the sample showed gastric necrosis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Varicella gangrenosum is a gangrenous ulceration of varicella lesions involving the skin and soft tissues of the body. The term was coined more than 100 years ago. This occurs due to superimposed bacterial infection. The presentation of primary varicella in adults is more severe with catastrophic systemic complications as compared with children. These complications include necrotising fasciitis, disseminated intravascular coagulation, wet/dry gangrene and death, as was seen in our case. Survival is dependent on early diagnosis and treatment. Adequate antibiotic treatment and particularly early radical surgical debridement should be the cornerstone of management. Less than 10 such cases are reported in the literature., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Relatively little scientific attention has been given to the small subset of critically ill patients with circulatory shock who develop ischaemic limb losses (symmetrical peripheral gangrene [SPG]). The clinical picture consists of acral (distal extremity) tissue necrosis involving lower limbs in a largely symmetrical fashion and with detectable arterial pulses; in one-third of patients the upper extremities are also affected (potential for four-limb amputations). The laboratory picture includes thrombocytopenia, coagulopathy, and normoblastemia (circulating nucleated red blood cells). The explanation for limb losses is microvascular thrombosis caused by disseminated intravascular coagulation usually secondary to cardiogenic or septic shock. A common myth is that vasopressors cause the ischaemic limb injury. However, the more likely explanation is failure of the natural anticoagulant systems (protein C and antithrombin) to downregulate thrombin generation in the microvasculature. This is because more than 90% of patients with SPG have preceding 'shock liver', which occurs 2 to 5 days (median, 3 days) prior to ischaemic limb injury, with impaired hepatic production of protein C and antithrombin., Competing Interests: The author declares the following conflicts of interest: consulting fees from Aspen Global and Octapharma; research support and consulting fees from W.L. Gore and Instrumentation Laboratory; royalties from Informa (Taylor & Francis); and consulting fees related to medical–legal expert testimony., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: Gangrenous cholecystitis (GC) must be promptly treated for its high morbimortality. The object of our study is to identify clinical, laboratory or ultrasound factors that might us diagnose GC., Method: A Retrospective cohort study is devised including all patients admitted to Hospital de Laredo (Cantabria, Spain) between 2015 and 2017 with the diagnose of acute cholecystitis and having been operated. Patients were classified in two groups according to pathology: GC and non-GC. We compared their demographics characteristics, comorbidities, laboratory parameters and ultrasound findings., Results: A total of 115 patients were operated, of whom 32 had CG and 83 CNG. Neutrophil-to-lymphocyte ratio and C-reactive protein (CRP) showed significantly increased levels in GC group (p = 0.042) and CRP (p < 0.0001). To CRP showed an area under the ROC curve of 0.872 (95% confidence interval: 0.797-0.946). Acalculous cholecystitis was significantly associated to GC (24.1 vs. 7%; p < 0.005). In the multivariate analysis only the CPR showed as a predictive factor. A cutting point of CRP at 15.25 mg/dl, that had high sensibility (90.6%) and high negative predictive value (95%)., Conclusion: CRP helped identify patients with CG to indicate early surgical intervention., (Copyright: © 2019 Permanyer.)

A 36-year-old man who complained of epigastric pain was transferred to the emergency room. We performed contrast enhanced computed tomography (CT) but were unable to confirm a diagnosis at that time. Because this patient had symptoms of gallbladder inflammation following hospitalization, we reviewed the CT images. We found luminal obstruction between the celiac artery and proper hepatic artery due to celiac artery dissection in the images. Gangrenous cholecystitis was suspected based on the findings;therefore, the patient underwent emergency cholecystectomy. To the best of our knowledge, there are only a few reports on cases with celiac artery dissection and none of those with acalculous gangrenous cholecystitis. Here, we report an exceptionally rare case with celiac artery dissection.