ABSTRACT
Rickettsial diseases cover a broad spectrum of illnesses. Scrub typhus is present worldwide, and Orientia tsutsugamushi is the causative agent of this chigger mite-borne infectious illness. Infections exhibit a range of severity, from mild illness to the more severe manifestation of multiorgan failure. We report three cases of scrub fever (55-year-old female, 63-year-old female, and 29-year-old male), all cases developed focal or pan-digital gangrene. All cases were successfully treated by administering doxycycline and additional supportive measures. Digital gangrene is uncommon in scrub typhus. Clinical suspension for early diagnosis and appropriate treatment may avoid further complications.
Subject(s)
Orientia tsutsugamushi , Scrub Typhus , Male , Female , Humans , Middle Aged , Scrub Typhus/complications , Scrub Typhus/diagnosis , Scrub Typhus/drug therapy , Gangrene/complications , Gangrene/drug therapy , Doxycycline/therapeutic use , ResearchABSTRACT
INTRODUCTION: Although rare, Kaposi sarcoma is the most common malignant neoplasm associated with human immunodeficiency virus (HIV) infection. Several agents have now been approved in the treatment of this malignancy and are used with varying degrees of success. CASE REPORT: We present a unique case of a 64-year-old man with well-controlled HIV infection who developed necrotizing leg gangrene from invasive cutaneous Kaposi sarcoma. He responded very well to systemic chemotherapy, thereby avoiding limb amputation. MANAGEMENT AND OUTCOME: Pegylated liposomal doxorubicin (PLD) at a dose of 20â mg/m2 every 3 weeks was utilized, with a near-complete response after six cycles of therapy. The patient continues to receive maintenance treatment with PLD. His HIV infection remains in excellent control, with a high-normal CD4 T-cell count. Periodic echocardiogram evaluations have not shown any decline in left ventricular ejection fraction (LVEF) over time. CONCLUSION: Most patients with Kaposi sarcoma achieve partial responses to treatment with PLD. Our case illustrates that near complete and complete responses are possible with this agent, leading to potential limb salvage in necrotizing gangrene.
Subject(s)
HIV Infections , Sarcoma, Kaposi , Skin Neoplasms , Antibiotics, Antineoplastic/therapeutic use , Doxorubicin/analogs & derivatives , Doxorubicin/therapeutic use , Gangrene/complications , Gangrene/drug therapy , HIV Infections/complications , HIV Infections/drug therapy , Humans , Leg/pathology , Male , Middle Aged , Polyethylene Glycols/therapeutic use , Sarcoma, Kaposi/drug therapy , Skin Neoplasms/complications , Skin Neoplasms/drug therapy , Stroke Volume , Ventricular Function, LeftABSTRACT
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.
Subject(s)
Anemia, Hemolytic, Autoimmune/complications , Anemia, Hemolytic, Autoimmune/etiology , Gangrene/complications , Mycoplasma Infections/complications , Mycoplasma Infections/microbiology , Thrombosis/diagnosis , Thrombosis/etiology , Agglutination Tests , Anemia, Hemolytic, Autoimmune/diagnosis , Anti-Bacterial Agents/therapeutic use , Anticoagulants/therapeutic use , Biomarkers , Child , Cryoglobulins/immunology , Foot/pathology , Gangrene/diagnosis , Gangrene/drug therapy , Gangrene/etiology , Humans , Male , Mycoplasma Infections/drug therapy , Radiography, Thoracic , Thrombosis/therapy , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
Peripheral limb ischaemia and gangrene are devastating complications of pneumococcal sepsis. We report a 43-year-old professional pianist who presented with early sepsis and rapid development of this syndrome. No vasopressor medication was ever administered. We urgently reviewed the medical literature on a range of therapies recommended by consulting teams, to ensure he received optimal care. Based on our review and on feedback from the patient himself, we gained valuable insights into this illness and the merits of selected treatment options. His fingers ultimately recovered their function, intact, although several toes were later amputated. More recently published reviews postulate that imbalances in coagulation factors and natural anticoagulants occur as a result of disseminated intravascular coagulopathy and 'shock liver' in the sepsis syndrome, leading to microcirculatory thromboses. We submit this report as we believe it supports this hypothesis and adds further valuable information. We hope our observations will assist other critical care clinicians confronting this serious condition.
Subject(s)
Fingers/pathology , Gangrene/etiology , Ischemia/etiology , Sepsis/complications , Toes/pathology , Adult , Amputation, Surgical , Anticoagulants/therapeutic use , Diagnosis, Differential , Gangrene/drug therapy , Heparin/therapeutic use , Humans , Ischemia/drug therapy , Male , Milrinone/therapeutic use , Music , Sepsis/drug therapy , Toes/surgery , Vasodilator Agents/therapeutic useABSTRACT
Kounis Syndrome is a rare allergic reaction that results in coronary vasospasm and may occur in patients with and without coronary artery disease. A 57-year-old man receiving pre-operative vancomycin for osteomyelitis and gangrene of the foot experienced an episode of anginal symptoms associated with transient ischemic 12-lead electrocardiogram (ECG) changes. The patient's symptoms and ECG changes abated with discontinuation of vancomycin and subsequent coronary angiography revealed no evidence of coronary artery disease. Treatment of Kounis Syndrome begins with cessation of the causative agent. Consensus guidelines for the management of Kounis Syndrome have not been established but treatment should both dilate the coronary vessels and suppress the allergic response. Coronary vasospasm after administration of antibiotics, including vancomycin, is a rare but serious reaction. It is important that Emergency Physicians recognize Kounis Syndrome as an uncommon yet dramatic and consequential reaction to such a commonly-administered antibiotic.
Subject(s)
Anti-Bacterial Agents/adverse effects , Coronary Vasospasm/chemically induced , Kounis Syndrome/etiology , Vancomycin/adverse effects , Administration, Intravenous , Anti-Bacterial Agents/administration & dosage , Electrocardiography , Gangrene/drug therapy , Humans , Male , Middle Aged , Osteomyelitis/drug therapy , Vancomycin/administration & dosageSubject(s)
Antiphospholipid Syndrome/diagnosis , Skin/pathology , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/drug therapy , Drug Therapy, Combination , Exanthema/diagnosis , Exanthema/drug therapy , Exanthema/etiology , Gangrene/diagnosis , Gangrene/drug therapy , Gangrene/etiology , Humans , Immunoglobulins, Intravenous/administration & dosage , Infant , Livedo Reticularis/diagnosis , Livedo Reticularis/etiology , Male , Prednisolone/administration & dosage , Scrotum , Severity of Illness Index , Skin Ulcer/diagnosis , Skin Ulcer/drug therapy , Skin Ulcer/etiology , Treatment OutcomeABSTRACT
A 62-year-old man with no pertinent medical history presented with lower extremity weakness and worsening distal fingertips and toe cyanosis/gangrene. In the outpatient setting, he was initially being treated for Raynaud's phenomenon with a calcium channel blocker. On presentation, the patient had elevated inflammatory markers and white blood cell count. Serum vasculitis panel (proteinase-3 antibody) supported the diagnosis of granulomatosis with polyangiitis. His hospital course was complicated by ischaemic stroke and a diagnosis of mononeuritis multiplex in his lower extremities. After initiating therapy with intravenous steroid and rituximab, his symptoms overall improved including cyanotic fingertips/toes. His inflammatory markers and leucocytosis also improved. Outpatient follow-up consisted of further rituximab infusions and unrelated umbilical hernia incarceration which required surgery. He was found incidentally to have subsegmental pulmonary emboli which most likely occurred during the initial presentation prior to his diagnosis. The patient moved out of state and was lost to follow-up.
Subject(s)
Fingers/pathology , Gangrene/pathology , Granulomatosis with Polyangiitis/diagnosis , Immunologic Factors/therapeutic use , Methylprednisolone/therapeutic use , Rituximab/therapeutic use , Stroke/etiology , Toes/pathology , Diagnosis, Differential , Gangrene/drug therapy , Gangrene/etiology , Granulomatosis with Polyangiitis/complications , Granulomatosis with Polyangiitis/drug therapy , Granulomatosis with Polyangiitis/physiopathology , Humans , Male , Middle Aged , Raynaud Disease , Treatment OutcomeABSTRACT
BACKGROUND: Appendicitis presents on a spectrum ranging from inflammation to gangrene to perforation. Studies suggest that gangrenous appendicitis has lower postoperative infection rates relative to perforated cases. We hypothesized that gangrenous appendicitis could be successfully treated as simple appendicitis, reducing length of stay (LOS) and antibiotic usage without increasing postoperative infections. METHODS: In February 2016, we strictly defined complex appendicitis as a hole in the appendix, extraluminal fecalith, diffuse pus or a well-formed abscess. We switched gangrenous appendicitis to a simple pathway and reviewed all patients undergoing laparoscopic appendectomy for 12â¯months before (Group 1) and 12â¯months after (Group 2) the protocol change. Data collected included demographics, appendicitis classification, LOS, presence of a postoperative infection, and 30-day readmissions. RESULTS: Patients in Group 1 and Group 2 were similar, but more cases of simple appendicitis occurred in Group 2. Average LOS for gangrenous appendicitis patients decreased from 2.5 to 1.4â¯days (pâ¯<â¯0.001) and antibiotic doses decreased from 5.2 to 1.3 (pâ¯<â¯0.001). Only one gangrenous appendicitis patient required readmission, and one patient in each group developed a superficial infection; there were no postoperative abscesses. CONCLUSIONS: Gangrenous appendicitis can be safely treated as simple appendicitis without increasing postoperative infections or readmissions. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level II.
Subject(s)
Appendectomy/methods , Appendicitis/surgery , Laparoscopy/methods , Adolescent , Anti-Bacterial Agents/therapeutic use , Appendectomy/adverse effects , Appendicitis/complications , Appendicitis/drug therapy , Child , Female , Gangrene/drug therapy , Gangrene/surgery , Humans , Laparoscopy/adverse effects , Length of Stay/statistics & numerical data , Male , Patient Readmission/statistics & numerical data , Postoperative Complications/drug therapy , Postoperative Complications/epidemiology , Postoperative Complications/etiology , Prognosis , Quality ImprovementSubject(s)
Amputation, Surgical/methods , Fingers/surgery , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/immunology , Toes/surgery , Adult , Antibodies, Antiphospholipid/immunology , Aspirin/therapeutic use , Female , Fingers/physiopathology , Follow-Up Studies , Gangrene/drug therapy , Gangrene/etiology , Gangrene/surgery , Humans , Lupus Erythematosus, Systemic/diagnosis , Methylprednisolone/therapeutic use , Nifedipine/therapeutic use , Postoperative Care/methods , Toes/physiopathology , Treatment OutcomeSubject(s)
Benzocaine/adverse effects , Condoms/adverse effects , Dermatitis, Allergic Contact/etiology , Dermatitis, Atopic/complications , Penile Diseases/pathology , Skin Diseases/pathology , Adult , Anti-Bacterial Agents/administration & dosage , Anti-Bacterial Agents/therapeutic use , Debridement/methods , Dermatitis, Allergic Contact/microbiology , Dermatitis, Allergic Contact/pathology , Dermatitis, Atopic/chemically induced , Dermatitis, Atopic/microbiology , Dermatitis, Atopic/pathology , Gangrene/drug therapy , Gangrene/surgery , Humans , Male , Penile Diseases/microbiology , Pleasure , Skin Diseases/chemically induced , Skin Diseases/microbiology , Skin Transplantation/methods , Streptococcus pyogenes/isolation & purification , Treatment OutcomeABSTRACT
The cytokine blood profile in patients with complicated erysipelas was investigated. It was found that in patients with complications of erysipelas (gangrene, phlegmon, abscess, thrombophlebitis of the subcutaneous veins of the shin) levels of pro-inflammatory cytokines IL-1ß, TNF-α, IL-2, IL-6 in serum significantly increase and level of anti-inflammatory cytokine IL-4 increases slightly, as well as was found a significant increase in coefficients reflecting the ratio of pro-inflammatory and anti-inflammatory cytokines, which indicates the prevalence in the blood of examined patients with complications of erysipelas an anti-inflammatory properties. A more significant increase in pro-inflammatory cytokines serum levels is typical for patients with destructive forms of erysipelas - phlegmonous and gangrenous, a slight increase - for patients without purulent-necrotic component of complication (thrombophlebitis of the subcutaneous veins of the shin). In the future we plan to study pharmacological correction of shifts in cytokine blood profile with drugs with immunomodulating properties in patients with complicated erysipelas.
Subject(s)
Abscess/blood , Cellulitis/blood , Erysipelas/blood , Gangrene/blood , Thrombophlebitis/blood , Abscess/complications , Abscess/drug therapy , Abscess/immunology , Adult , Anti-Bacterial Agents/therapeutic use , Anti-Inflammatory Agents/therapeutic use , Biomarkers/blood , Cellulitis/complications , Cellulitis/drug therapy , Cellulitis/immunology , Erysipelas/complications , Erysipelas/drug therapy , Erysipelas/immunology , Female , Gangrene/complications , Gangrene/drug therapy , Gangrene/immunology , Humans , Interleukin-1beta/blood , Interleukin-1beta/immunology , Interleukin-2/blood , Interleukin-2/immunology , Interleukin-4/blood , Interleukin-4/immunology , Interleukin-6/blood , Interleukin-6/immunology , Male , Middle Aged , Thrombophlebitis/complications , Thrombophlebitis/drug therapy , Thrombophlebitis/immunology , Tumor Necrosis Factor-alpha/blood , Tumor Necrosis Factor-alpha/immunologyABSTRACT
BACKGROUND: Critical limb ischaemia (CLI) is a severe manifestation of peripheral arterial disease, characterised by chronic ischaemic rest pain, ulcers or gangrene. Management of ischaemic pain is challenging in patients with no options for revascularisation and optimal pharmacological therapies have not been established. OBJECTIVES: To identify and evaluate the effectiveness of pharmacological therapies to treat ischaemic pain secondary to non-reconstructable CLI. METHODS: This systematic review was reported in accordance with PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guideline. Comprehensive searches of three electronic databases, a PubMed-related articles link search, grey literature search and hand-searches of the bibliographies of relevant papers and textbooks were performed. Studies recruiting adult patients with CLI of any aetiology were eligible for inclusion. Surgical and revascularisation procedures, and all invasive interventions were excluded. RESULTS: Of 792 studies, six met full inclusion criteria. These studies researched the use of intravenous lidocaine, intravenous ketamine, oral gabapentin and the combination of transdermal buprenorphine and epidural morphine/ropivacaine infusion. All studies showed an improvement in severity of ischaemic pain in CLI but with varying side effect profiles and quality. The extracted studies showed substantial heterogeneity and therefore a meta-analysis was not performed. CONCLUSION: The pharmacological management of pain secondary to non-reconstructable CLI is a challenging review topic. No recommendations of pharmacological agents can be made following this review but a number of novel approaches to manage pain in this cohort have shown positive results and require further investigation.
Subject(s)
Analgesics/administration & dosage , Chronic Pain/drug therapy , Extremities/blood supply , Ischemia/drug therapy , Musculoskeletal Pain/drug therapy , Pain Management/methods , Chronic Pain/etiology , Female , Gangrene/drug therapy , Gangrene/etiology , Humans , Ischemia/etiology , Male , Musculoskeletal Pain/etiology , Peripheral Arterial Disease/complications , Randomized Controlled Trials as Topic , Skin Ulcer/drug therapy , Skin Ulcer/etiologyABSTRACT
Ecthyma gangrenosum is a rare skin infection classically associated with Pseudomonas aeruginosa. We performed a retrospective study of all cases diagnosed with ecthyma gangrenosum from 2004-2010 in a university hospital in Mexico (8 cases, 5 female patients and 3 male patients, ages between 4 months and 2 years). The most common risk factor for ecthyma gangrenosum is neutropenia in immunocompromised patients. In previously healthy patients, immunological evaluation is important to rule out underlying immunodeficiency. Ecthyma gangrenosum in healthy patients has a high mortality rate and early diagnosis and aggressive antibiotic treatment is imperative as it can improve patients' prognosis.
Subject(s)
Ecthyma/microbiology , Gangrene/microbiology , Pseudomonas Infections/complications , Pseudomonas aeruginosa/isolation & purification , Child, Preschool , Ecthyma/drug therapy , Female , Gangrene/drug therapy , Humans , Infant , Male , Retrospective StudiesABSTRACT
Symmetrical peripheral gangrene (SPG) is a rare clinical syndrome characterized by ischemic necrosis of 2 or more limbs, without involvement of large vessels. It is often associated with disseminated intravascular coagulation and septic shock. Usually caused by Gram-positive and Gram-negative organisms, tuberculosis as a cause is extremely rare. We present the case of a 46-year-old man, who initially presented with signs and symptoms suggestive of tuberculosis but later developed SPG along with septic shock after his initial visit. The case highlights the progression of this dreaded complication and touches upon recent developments in its etiology as well as pathogenesis.
Subject(s)
Disseminated Intravascular Coagulation/etiology , Extremities/pathology , Gangrene/etiology , Shock, Septic/etiology , Tuberculosis, Miliary/complications , Antitubercular Agents/therapeutic use , Biopsy , Disseminated Intravascular Coagulation/pathology , Extremities/blood supply , Gangrene/diagnosis , Gangrene/drug therapy , Gangrene/pathology , Humans , Male , Middle Aged , Shock, Septic/pathology , Treatment OutcomeABSTRACT
Abstract: Ecthyma gangrenosum is a rare skin infection classically associated with Pseudomonas aeruginosa. We performed a retrospective study of all cases diagnosed with ecthyma gangrenosum from 2004-2010 in a university hospital in Mexico (8 cases, 5 female patients and 3 male patients, ages between 4 months and 2 years). The most common risk factor for ecthyma gangrenosum is neutropenia in immunocompromised patients. In previously healthy patients, immunological evaluation is important to rule out underlying immunodeficiency. Ecthyma gangrenosum in healthy patients has a high mortality rate and early diagnosis and aggressive antibiotic treatment is imperative as it can improve patients' prognosis.
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Pseudomonas aeruginosa/isolation & purification , Pseudomonas Infections/complications , Ecthyma/microbiology , Gangrene/microbiology , Retrospective Studies , Ecthyma/drug therapy , Gangrene/drug therapyABSTRACT
Despite advances in antibiotic and surgical management and supportive care for necrotizing soft tissue infections, morbidity and mortality remain substantial. Although there are clinical practice guidelines in place, there still remains much variability in choice and duration of antibiotic therapy, time to initial surgical debridement, and use of adjuvant medical therapies. This article offers an overview of necrotizing soft tissue infections with a focus on current diagnostic and treatment modalities.
Subject(s)
Fasciitis, Necrotizing/diagnosis , Fasciitis, Necrotizing/therapy , Soft Tissue Infections/diagnosis , Soft Tissue Infections/therapy , Administration, Intravenous , Anti-Bacterial Agents/therapeutic use , Debridement , Disease Management , Fasciitis, Necrotizing/complications , Fasciitis, Necrotizing/drug therapy , Gangrene/drug therapy , Gangrene/microbiology , Humans , Hyperbaric Oxygenation , Immunoglobulins/administration & dosage , Immunoglobulins/therapeutic use , Sepsis/drug therapy , Sepsis/microbiology , Sepsis/therapy , Soft Tissue Infections/complications , Soft Tissue Infections/drug therapySubject(s)
Cryoglobulinemia/etiology , Ear Auricle/pathology , Lymphoma, Large B-Cell, Diffuse/complications , Lymphoma, Non-Hodgkin/complications , Skin/pathology , Stomach Neoplasms/complications , Aged, 80 and over , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Cryoglobulinemia/diagnosis , Cryoglobulinemia/drug therapy , Gangrene/drug therapy , Gangrene/etiology , Humans , Lymphoma, Large B-Cell, Diffuse/diagnosis , Lymphoma, Large B-Cell, Diffuse/drug therapy , Lymphoma, Non-Hodgkin/diagnosis , Lymphoma, Non-Hodgkin/drug therapy , Male , Stomach Neoplasms/diagnosis , Stomach Neoplasms/drug therapy , Treatment OutcomeABSTRACT
RATIONALE: Ecthyma gangrenosum (EG) is an aggressive cutaneous disease caused by local or systemic infection with Pseudomonas aeruginosa. EG is characterized by cutaneous manifestations ranging from nodule and papule, to necrotic ulceration with surrounding erythema, especially with black eschar or central crust. EG presents with characteristic skin lesions which is important to establish diagnosis of sepsis caused by P aeruginosa, a serious condition that can be treated efficiently if diagnosed early. PATIENT CONCERNS: A 3-month-old female infant was presented with characteristic skin lesions of EG and developed sepsis 3 days later. DIAGNOSES: Ecthyma gangrenosum and sepsis caused by Pseudomonas aeruginosa. INTERVENTIONS: Meropenem was used in combination with ceftazidime at first and excision of necrotic skin lesions was performed later. OUTCOMES: Cure. LESSONS: Early recognition of EG plays an important role in providing appropriate empiric antibiotic treatment at early stage of sepsis, and improves the prognosis. Surgical excision may be helpful if no improvement was achieved via antibiotic treatment.
Subject(s)
Gangrene/microbiology , Pseudomonas Infections/drug therapy , Pseudomonas aeruginosa/isolation & purification , Pyoderma Gangrenosum/microbiology , Sepsis/microbiology , Anti-Bacterial Agents/therapeutic use , Ceftazidime/therapeutic use , Female , Gangrene/drug therapy , Gangrene/surgery , Humans , Infant , Meropenem , Pseudomonas Infections/surgery , Pyoderma Gangrenosum/drug therapy , Pyoderma Gangrenosum/surgery , Sepsis/drug therapy , Sepsis/surgery , Thienamycins/therapeutic useABSTRACT
BACKGROUND: Thrombotic cutaneous gangrene is a rare extra-intestinal manifestation of ulcerative colitis with a severe prognosis. CASE REPORT: A 35-year-old woman with a 7-year history of ulcerative colitis presented with extensive ecchymotic lesions that began a few hours earlier. On examination, she was febrile with multiple necrotic lesions. Skin biopsy showed multiple microthrombi in the dermal vessels. A diagnosis of thrombotic cutaneous gangrene was established. The patient was treated with heparin and systemic corticosteroids. The majority of cutaneous lesions showed improvement after 1 month. Thrombophlebitis of the left lower limb occurred subsequently. CONCLUSION: Thrombotic cutaneous gangrene is attributed to microvascular thrombosis, which arises from the hypercoagulability observed in ulcerative colitis. Complete blood and coagulation tests must be performed and early anticoagulation with heparin must be considered in order to prevent the progression of cutaneous infarction.
Subject(s)
Colitis, Ulcerative/diagnosis , Colitis, Ulcerative/pathology , Skin/pathology , Thrombosis/diagnosis , Thrombosis/pathology , Adrenal Cortex Hormones/therapeutic use , Adult , Biopsy , Colitis, Ulcerative/drug therapy , Drug Therapy, Combination , Female , Gangrene/diagnosis , Gangrene/drug therapy , Gangrene/pathology , Heparin/therapeutic use , Humans , Necrosis , Skin/blood supply , Skin/drug effects , Thrombosis/drug therapyABSTRACT
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.