Relationship Between Symmetrical Peripheral Gangrene Patients and Using Vasopressors in the Intensive Care Unit.

Symmetrical peripheral gangrene (SPG) is a rare yet severe condition characterized by peripheral ischemic lesions without significant vascular occlusion. Its clinical presentation includes peripheral cyanosis, mottling, and symmetrical ischemia of distal limbs, often progressing to gangrene. Recent years have seen a rise in SPG cases, with mortality rates ranging from 40% to 90%. The condition is associated with systemic diseases, such as sepsis, vasculitis, and coagulopathy. DIC frequently complicates SPG, reflecting a disturbed procoagulant-anticoagulant balance and depletion of natural anticoagulants. While vasopressor therapy, particularly high-dose administration, has been implicated in SPG pathogenesis due to sustained vasoconstriction or idiosyncratic responses, recent evidence suggests it may not be the underlying cause. Studies indicate a low incidence of ischemic limb necrosis associated with high-dose vasopressors, with DIC and shock liver potentially explaining limb ischemia instead. The characteristic temporal interval between the onset of shock liver and limb ischemic necrosis suggests a more complex pathophysiology. The role of infectious agents, such as bacteria and viruses, in SPG pathogenesis is under investigation, with both direct vascular invasion and immune-mediated mechanisms proposed. Diagnosis involves ruling out other causes of acral gangrene through clinical examination, laboratory tests, imaging studies, and biopsy. Treatment strategies aim to halt disease progression, eliminate causative factors, and prevent complications. While anticoagulants, vasodilators, and adjunctive therapies like hyperbaric oxygen show promise, the efficacy of interventions varies, emphasizing the need for individualized management. Notably, hemoadsorption has emerged as a promising treatment, demonstrating significant improvement in SPG cases. Amputation remains a last resort option in irreversible cases. Early recognition and multidisciplinary management are crucial for improving outcomes. Further research is needed to better understand SPG's etiology and develop effective treatments through collaborative efforts.

Digital gangrene: can be the first manifestation of essential thrombocythemia? a case report.

Introduction and importance: Essential thrombocythemia (ET) is a rare chronic myeloproliferative hematologic disorder, leading to an elevated platelet count. Two-thirds of patients are asymptomatic during their lifetime, while others may experience symptoms like redness, congestion, and erythromelalgia after long symptom-free intervals. Case presentation: The authors present a rare instance of a 55-year-old female who, despite receiving aspirin and losartan treatment, eventually developed digital gangrene. In further work-ups, she had an elevated platelet count and a positive JAK 2 mutation. Her platelet count was reduced throughout treatment with aspirin, hydroxyurea, and heparin, which was followed by the necrotic tip of her index finger being surgically debrided. Clinical discussion: Significant symptoms, such as severe acrocyanosis and even peripheral gangrene, can be treated with a single dose of aspirin. Daily aspirin consumption withstanding, this case developed the severe form of ET. In addition, while thrombocytosis predisposes patients to thrombotic complications in theory, there is little evidence to support a correlation between absolute platelet count and thrombosis. Conclusion: The initial symptom of ET could be such severe and uncommon that may develop arterial acral thrombosis despite previous daily low-dose aspirin consumption.

Use of prostaglandin and cyclophosphamide for pediatric lupus with digital gangrene: Back to safety before hitting the axe!

Digital gangrene is a rare presenting feature of childhood lupus and only a reported incidence of 1.3%. We describe two cases of pediatric onset systemic lupus erythematosus (SLE), both 16 years old, presenting with digital gangrene and the successful salvage of the digits after using intravenous cyclophosphamide for immunosuppression and use of intravenous prostaglandin E1 infusions for limb reperfusion. Both of the patients responded exceptionally to the infusions with resolution of gangrene and near-total preservation of the functionality of toes.

Digital Gangrene and Antiphospholipid Syndrome in a Retinoblastoma Patient with Chromosome 13q Deletion: A Case Report.

A 19-month-old girl with Cornelia de Lange-like dysmorphic features presented with left eye leukocoria. She was diagnosed with 13q deletion retinoblastoma grade 4 with high-risk features and bone marrow involvement. She underwent enucleation, and the first course of intravenous chemotherapy was initiated. On day 10 after the first chemotherapy dose, she developed digital gangrene of her left hand. She was diagnosed with acute artery occlusion and limb ischemia. Thrombophilia work-up revealed antiphospholipid antibodies, and paraneoplastic syndrome is another possible cause of digital gangrene. The patient's left thumb and index finger were amputated. After 1 month of hospitalization, she was discharged. Before the second course of chemotherapy, the patient died of systemic metastatic retinoblastoma with respiratory failure due to pneumonia. Our postulation of the mechanism for digital gangrene was the combination of chemotherapy, paraneoplastic syndrome, and antiphospholipid syndrome. Digital gangrene could be a poor prognostic indicator in patients with retinoblastoma.

A review of the development of gangrene in patients with systemic lupus erythematosus - A 44-year follow-up study.

OBJECTIVES: This review addresses the question of what happens long-term to those systemic lupus erythematosus (SLE) patients who develop gangrene. It also seeks to find common clinical and serological features, risk factors and triggers and how best to manage this challenging complication. METHODS: We reviewed 850 patients with SLE attending a UK tertiary referral center, followed up over 44 years, assessing their demographics, clinical and serological features, treatment in the acute phase, their long-term outcome and long-term management. RESULTS: Ten out of 850 patients (1.2%) developed gangrene; the mean age of onset was 17 years (range 12-26 years) Eight out of 10 patients had a single episode of gangrene. One of the other two was not willing to have anticoagulation. The first episode of gangrene ranged from presentation to 32 years after SLE onset, mean duration of SLE at the onset of the gangrene was 18.5 years SD 11.5 years. Anti-phospholipid (PL) antibodies were over-represented in the patients with gangrene. All had active SLE at the time the gangrene developed. All patients were treated with intravenous (IV) iloprost infusions, and the antiphospholipid-antibody positive patients were anti-coagulated, most staying on long term anticoagulation. Underlying possible triggers were treated appropriately. Two patients who did not respond to the initial treatment needed further immunosuppression. All patients suffered digit loss. CONCLUSION: Although rare, gangrene is a sinister, potentially late developing complication of SLE, it rarely recurs. It is associated with anti-phospholipid antibodies, active disease, and other possible triggers such as infection and cancer. Anticoagulation therapy, steroids and iloprost, and further immunosuppression may be needed to stop the evolution of gangrene.

A Rare and Complex Case of Non-suicidal Self-Mutilation in a Patient With Schizophrenia.

The literature describing acts of non-suicidal self-mutilation (NSSM) in the adult population is limited. Of the cases that document NSSM, a disproportionate number of these individuals have a history of psychiatric illnesses. Although the motivation to perform NSSM varies across patients, the literature suggests that past self-injurious behaviors, extreme religious delusions, and command hallucinations are the most significant risk factors. The primary forms of NSSM include ocular, genital, and limb mutilation. Limb mutilation is the least common of the three and typically occurs proximal to the wrist or hand. Here, we present a rare case involving a 42-year-old man with schizophrenia who was hospitalized due to osteomyelitis of his autoamputated digits. This case is unique in involving multiple digits of the hand and using a rare amputation method. We aim to compare this case with the existing body of work on NSSM and identify factors that may predispose patients to act on these extreme impulses. We also highlight a novel interventional program that reduces psychiatric and medical comorbidities.

Identifying risk and prognostic factors in polyarteritis nodosa patients with digital gangrene.

BACKGROUND: Many patients with polyarteritis nodosa (PAN) complicated by digital gangrene have poor outcomes and related research information is limited. Our aim is to identify the associated risk and prognostic factors in PAN patients with digital gangrene. PATIENTS AND METHODS: We conducted a retrospective study of 148 PAN patients admitted to Peking Union Medical College Hospital from Octorber 2001 to December 2018. Forty-seven (31.8%) PAN patients had digital gangrene. The average age was 40.4 ± 17.9 years. RESULTS: The presence of digital gangrene was correlated with current smoking (P = .008, odds ratio [OR] 2.99, 95% CI, 1.33-6.73), eosinophil elevation (P = .003, OR 4.21, 95% CI, 1.62-10.91) and elevated leukocytes (P = .001, OR 4.26, 95% CI, 1.86-9.78). Thirty-two (68.1%) gangrene patients received methylprednisolone pulse therapy and all of these patients were treated with cyclophosphamide. Nine patients suffered irreversible organ injury and 2 died. Survival analysis showed higher serum C-reactive protein (CRP) was associated with poor prognosis in patients with gangrene (log-rank P = 0.042 and generalized Wilcoxon P = .020). CONCLUSIONS: PAN patients with current smoking and eosinophil elevation were more prone to digital gangrene and a high serum CRP level predicted poor outcomes. The CRP level should be efficiently controlled to ensure a good prognosis.

Severe digital ischemia as an unrecognized manifestation in patients with antisynthetase autoantibodies: Case series and systematic literature review.

Objective: Severe digital ischemia, including digital ulcers and gangrene, is considered rare in patients with antisynthetase antibodies. This study aimed to elucidate the clinical features of antisynthetase-positive patients complicated with digital ulcers and/or gangrene using a systematic literature review and case series in a single-center cohort. Methods: A systematic literature review was conducted to identify reports describing antisynthetase-positive cases with digital ulcers and/or gangrene. Our cohort of consecutive patients with antisynthetase antibodies was stratified by the history of severe digital ischemia. Demographic and clinical features and outcomes in patients with severe digital ischemia identified in the systematic literature review and our cohort were compared with those in patients without severe digital ischemia in our cohort. Results: The systematic literature review revealed 12 antisynthetase-positive patients with severe digital ischemia from one case series and eight case reports. Seven (7%) of 100 patients with antisynthetase antibodies in our cohort had a record of severe digital ischemia. Severe digital ischemia was often found at presentation and was associated with the classification of systemic sclerosis with or without myositis overlap. Clinical features associated with severe digital ischemia in antisynthetase-positive patients included Raynaud's phenomenon (p < 0.001), digital pitting scars (p = 0.001), and nailfold capillary abnormality (p = 0.02). Outcomes of severe digital ischemia were generally favorable with vasodilators. Conclusion: Severe digital ischemia is an overlooked complication in antisynthetase-positive patients. Antisynthetase antibodies should be measured in patients presenting with digital ulcers or gangrene, especially in those with systemic sclerosis phenotype and features associated with antisynthetase antibodies in the absence of systemic sclerosis-specific autoantibodies.

Bilateral Upper Limb Symmetrical Digital Gangrene: A Rare Presentation of Anti-Phospholipid Syndrome.

Antiphospholipid syndrome (APS) is an autoimmune disease present most commonly in young women, characterized by the presence of antibodies against various phospholipids and culminating in alteration of the flow of blood, leading to arterial and venous thrombosis. Although it can present with a wide range of manifestations, digital gangrene is one of the important ones. We present a case of a young female with antiphospholipid syndrome who presented with acute onset bilateral upper limb symmetrical digital gangrene with prior history of multiple fetal losses. Acute onset, symmetrical gangrene, limited to the bilateral upper limbs without venous system involvement, that too in association with systemic lupus erythematosus (SLE) which does not usually manifest as such make this case a unique and interesting one.

Digital gangrene and pulmonary consolidation in a young girl with Takayasu arteritis.

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.

Case Report: Extensive digital gangrene as a primary manifestation of late-onset systemic lupus erythematosus.

Background: Digital gangrene is a rare but serious complication of systemic lupus erythematosus (SLE). It occurs usually in middle-aged patients with longer disease duration. Case: Herein we report the case of a 56-year-old man (with no history suggestive of Raynaud's phenomenon, diabetes mellitus, smoking, trauma, infection, or chemical exposure), who presented with SLE and digital gangrene was among the first signs. He presented with a one-month history of joint pain, hair loss, photosensitivity, mouth ulcers, malar rash, dyspnea, and digital pain. Physical examination revealed painful and diffuse erythematous skin lesions in the extremities and back, as well as cyanosis in the fingers. We noted lymphocytopenia (600 cells/mm 3), and an elevated C-reactive protein (15.1 mg/l) on laboratory tests. Immunological tests were positive for antinuclear antibodies (ANA) with Title 1:400. Pulmonary computed tomography revealed pulmonary fibrosis, and pulmonary function tests revealed the restrictive pulmonary disease. Diagnosis of SLE with lung involvement was retained. The immunological assessment in search of elements in favor of a vascular origin of the patient's skin lesions was negative. Treatment was initiated with 200 mg/day hydroxychloroquine. For dermal and pulmonary involvement, intravenous (IV) pulse therapy was used with methylprednisolone (1,000 mg/d for three consecutive days monthly) and cyclophosphamide (1 g/month). Calcium blocking agents were also prescribed. However, the lesions did not improve. The patient was given two infusions of rituximab (1 g) at a 14-day interval with a marked improvement ofthe majority of vasculitis lesions, and a partial improvement of dyspnea. Conclusions: Digital gangrene is a rare complication of late-onset SLE, especially as a primary manifestation.

Purpura fulminans following hump-nosed pit viper (Hypnale hypnale) envenoming: A rare complication of snakebites.

Hump-nosed pit vipers of the genus Hypnale are highly venomous and reputed for the commonest venomous snakebites in Sri Lanka. They frequently cause local manifestations and less commonly cause systemic effects such as acute kidney injury and coagulopathy. There is no antivenom currently available in Sri Lanka for their envenoming. However, more and more complications of Hypnale bites are being recently described. Purpura fulminans, one of the rare complications of snakebites that we report following authentic Hypnale hypnale bite. A 58-year-old female was bitten by a hump-nosed viper and developed bilateral toe gangrenes, ultimately ended up with amputations. She got recovered with loss of toes in both feet for 46 days treatment at hospital.

Asymptomatic coronary aneurysms in a patient with eosinophilic granulomatosis with polyangiitis who developed a digital gangrene.

A 33-year-old male with a history of bronchial asthma and allergic rhinitis was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) eight years ago. The diagnosis was based on the presence of fever, remarkable eosinophilia, and painful digital ulcer. His signs and symptoms improved with a moderate dose of glucocorticoids. Thereafter, he was lost to follow-up, failing to attend any of the scheduled appointments. Three years later, he presented with painful digital gangrene on the lateral fold of the right ring fingernail and abdominal pain triggered by meals. Angiography showed multiple occlusions and severe stenoses of the peripheral arteries and coronary aneurysms, which confirmed the diagnosis of medium vessel vasculitis of the coronary and peripheral arteries due to flare up of EGPA. EGPA predominantly affects the small-sized vessels, but rarely the medium-sized vessels. Coronary vasculitis might occur asymptomatically, until the coronary stenosis becomes severe or myocardial infarction develops; hence, its prevalence is underestimated. In this case, a digital gangrene prompted us to perform a systemic angiography, leading to the diagnosis of coronary vasculitis. Careful observation for coronary lesions is necessary in patients with EGPA who develop digital gangrene.

A Late Presentation of Hand Pain with Skin Changes.

A middle-aged homeless man presented with ongoing left-hand pain and worsening cutaneous phalangeal color changes. On examination, digital ischemia was observed with associated dry gangrene. Doppler examination showed impaired distal arterial flow. Angiography showed irregular digital perfusion. The patient later provided vital information to secure the diagnosis without further clinical workup. This quiz discusses the presentation, differential diagnosis, and treatment of a rare clinical scenario.

Peripheral Gangrene as the Initial Presentation of Systemic Lupus Erythematosus in Emergency Department.

Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disease. Among the cutaneous manifestations of SLE, digital gangrene is considered to be very rare. This complication, which may lead to severe ischemic necrosis and amputation, is suggested to be the result of poor perfusion that is usually caused by vasculitis, vasospasm, thromboembolism, or atherosclerosis. Digital gangrene is seen mostly at a late stage of the disease proposing that a long history of SLE is a considered risk factor. Only 0.2% of patients with SLE presented initially as digital necrosis. This is a case report of a 20-year-old Saudi female who presented to the emergency room primarily with acute painful localized dry digital gangrene associated with bilateral lower limbs petechial rash. Her medical history was not suggestive of autoimmune diseases. Serology was positive for SLE. A diagnosis of SLE, lupus nephritis, and vasculitis has been established clinically and serologically. The patient adequately responded to rituximab and steroids as a medical therapy. To our knowledge, cases of acute peripheral gangrene as the initial and only presentation of SLE have rarely been documented in Emergency Medicine.

Digital gangrene induced by inadvertent intra-arterial cocktail injection of anesthetic agents such as pentazocine, promethazine, and atropine: A serious adverse drug experience.

Gangrenous changes in skin due to accidental intra-arterial injection of promethazine and pentazocine have been reported. Accidental intra-arterial injection is most commonly encountered in the antecubital fossa. However, recent reports in the radial and ulnar arteries have also been encountered. We hereby report a serious, preventable adverse drug experience in the form of digital gangrene induced by inadvertent intra-arterial cocktail injection of anesthetic agents such as pentazocine, promethazine, and atropine, which seems to be in the radial artery as the lateral three digits and dorsum of the hand are affected.

[Extensive digital gangrene revealing late-onset systemic lupus erythematosus]. / Nécrose digitale extensive révélant un lupus systémique tardif.

INTRODUCTION: Late-onset systemic lupus erythematosus represents a specific sub-group of the disorder, beginning after 50 years of age. The incidence is rarer and the course of the disease is considered to be more benign. Digital gangrene is an uncommon complication of systemic lupus erythematosus reported especially among middle-aged patients with long disease duration. OBSERVATION: We report a 53-year-old man, who presented with systemic lupus erythematosus revealed by an extensive digital gangrene. CONCLUSION: Digital gangrene is a rare complication of late onset systemic lupus erythematosus. Clinicians should be aware of this complication in order to initiate early and aggressive treatment.

Subclavian Artery Pseudoaneurysm in an Unusual Case of Digital Gangrene.

A young male patient presented at a tertiary care hospital with cold and bluish left upper limb accompanied with digital gangrene arousing suspicion of peripheral vascular disease. History did not reveal any high-risk behavior. Clinical examination and subsequent investigations lead to the diagnosis of acute infective endocarditis of native aortic valve along with peripheral embolism caused by methicillin-resistant Staphylococcus aureus. Fogarty's balloon embolectomy was done following which patient developed pseudoaneurysm of the left subclavian artery. These iatrogenic sequelae were managed with the resection of the pseudoaneurysm and prolonged antibiotic therapy as per the culture and sensitivity report.

Haemolytic uraemic syndrome with peripheral gangrene and Kawasaki disease in a 15-month-old child.

A 15-month-old boy presented with atypical haemolytic uraemic syndrome (HUS) (without antecedent diarrhoea or dysentery) following a gluteal abscess and subsequently developed digital gangrene. During plasma infusion therapy for HUS, the clinical features of Kawasaki disease (KD) evolved. Intravenous immunoglobulin and aspirin therapy led to resolution of the KD. The case is notable for development of digital gangrene, a rare phenomenon described with HUS, as well as the development of features of KD. This is the first report of atypical HUS in association with peripheral gangrene and KD. The possible pathophysiological mechanisms are discussed.

Post-traumatic Digital Gangrene Associated with Epinephrine Use in Primary Raynaud's Phenomenon: Lesson for the Future.

BACKGROUND: Following digital surgical procedures, the ensuing post-operative course may be complicated by the presence of underlying ischaemic or vasospastic process. In the presence of such conditions, post-operative ischaemic changes may be further exacerbated with the use of local anaesthetics in combination with epinephrine. CASE DETAILS: We report a 21 year-old female who presented with an amputated fifth digit due to a rapidly spreading gangrene which started immediately after the surgical repair of a traumatic laceration which was infiltrated with a pre-mixed solution of lignocaine and epinephrine 3 hours earlier. The patient's final diagnosis was epinephrine-associated digital gangrene in the background of primary Raynaud's Phenomenon (RP). CONCLUSION: The author reports this case in order to reiterate the importance of thorough clinical evaluation prior to the use of epinephrine in digital anaesthesia as well as to increase awareness on how primary RP can be complicated by gangrene.