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![Figure1.Macroscopic and microscopic findings of the skin lesion. (A) Palpable purpura progressed rapidly into extensive ecchymoses on the back (left), trunk (right upper), and right foot and ankle (right lower). (B) A fascia biopsy of the right crus showing fresh fibrin thrombi (arrows), including neutrophils within a small dilated vessel [Hematoxylin and Eosin (H&E) staining; original magnification 20×]. (C) A skin biopsy showing infiltration of neutrophils with karyorrhectic nuclear debris, and extravasation of erythrocytes around small vessels in the dermis, leading to a diagnosis of leukocytoclastic vasculitis (H&E staining; original magnification 20×). (D) A skin biopsy showing immunoglobulin M and C3 deposition within the walls of a small vessel in the dermis (immunofluorescence stain; original magnification 20×).](profile/Ikue-Okamura-Shiki/publication/309164615/figure/fig1/AS:417447673909248@1476538689330/Figure1Macroscopic-and-microscopic-findings-of-the-skin-lesion-A-Palpable-purpura.png)
Figure1.Macroscopic and microscopic findings of the skin lesion. (A) Palpable purpura progressed rapidly into extensive ecchymoses on the back (left), trunk (right upper), and right foot and ankle (right lower). (B) A fascia biopsy of the right crus showing fresh fibrin thrombi (arrows), including neutrophils within a small dilated vessel [Hematoxylin and Eosin (H&E) staining; original magnification 20×]. (C) A skin biopsy showing infiltration of neutrophils with karyorrhectic nuclear debris, and extravasation of erythrocytes around small vessels in the dermis, leading to a diagnosis of leukocytoclastic vasculitis (H&E staining; original magnification 20×). (D) A skin biopsy showing immunoglobulin M and C3 deposition within the walls of a small vessel in the dermis (immunofluorescence stain; original magnification 20×).
Source publication
Purpura fulminans (PF) is a life-threatening syndrome comprising progressive hemorrhagic necrosis due to disseminated intravascular coagulation and dermal vascular thrombosis that leads to purpura and tissue necrosis. Various therapies have been used to arrest the progression of this disease, however, there is no established treatment because of th...
Citations
... The offending drug may act as a hapten, triggering a systemic immune response to an antibody-drug complex. 3 Some patients appear to have developed a leucoerythroblastic vasculitis precipitating small vessel thrombosis and purpura fulminans. 3 A T-cell mediated mechanism has been proposed in other cases due to absence of detectable pathological antibodies or immune complexes. ...
... 3 Some patients appear to have developed a leucoerythroblastic vasculitis precipitating small vessel thrombosis and purpura fulminans. 3 A T-cell mediated mechanism has been proposed in other cases due to absence of detectable pathological antibodies or immune complexes. 9 Drug-induced liver injury may cause acquired protein C or S deficiency, 4,5 or enhanced release of coagulation factors, leading to a hyper-coagulable state. ...
Purpura fulminans is a life-threatening condition in which there is extensive cutaneous haemorrhagic necrosis in the setting of disseminated intravascular coagulation (DIC). Three sub-types of purpura fulminans have been described in the literature: acute infectious purpura fulminans (often due to overwhelming sepsis commonly due to Neisseria meningitidis bacteraemia), neonatal purpura fulminans (due to hereditary protein C or S deficiency) and idiopathic purpura fulminans (either drug-induced
or of unknown aetiology). Here, we describe a case precipitated by trimethoprim-sulfamethoxazole, and review the relevant literature for possible mechanistic explanations.
... Rarely, PF may be caused by medications such as fluoroquinolones. In cases of medication-related PF, corticosteroid therapy is recommended [27]. Finally, acquired protein C and S deficiency may put patients at greater risk of developing this condition. ...
Patient: Male, 67-year-old
Final Diagnosis: Purpura fulminans • septic shock • Streptococcus pneumoniae bacteremia
Symptoms: Diarhea • nausea • shortness of breath • weakness • rash
Medication: Ceftriaxone
Clinical Procedure: Blood culture
Specialty: Infectious Diseases • Critical Care Medicine
Objective
Rare co-existance of disease or pathology
Background
Despite proven efficacy of vaccinations against Streptococcus pneumoniae in preventing infection, only 70% of eligible individuals receive the vaccine in the United States. Pneumococcal bacteremia represents a form of invasive pneumococcal disease and is associated with high mortality, especially in immunocompromised patients and the elderly. Purpura fulminans is a rare complication and manifestation of disseminated intravascular coagulation and sepsis. It is exceedingly rare in the setting of pneumococcal bacteremia, particularly in immunocompetent individuals.
Case Report
We report a generally healthy 67-year-old male with schizophrenia who refused pneumococcal vaccination. He had an intact and functional spleen with a functional immune system. The patient presented with fever and diarrhea. He subsequently progressed to develop purpura fulminans and septic shock due to S. pneumoniae bacteremia. Despite an extensive search for the primary source of infection, none could not be identified. Due to timely initiation of appropriate antibiotic therapy and aggressive supportive care in an intensive care unit, he recovered despite multi-organ failure that developed throughout his hospitalization.
Conclusions
We present a rare manifestation of a potentially preventable disease and emphasize the importance of pneumococcal vaccination in order to decrease the risk of developing invasive pneumococcal disease. Furthermore, we discuss etiology, diagnosis, differential diagnosis, and evidence-based management of purpura fulminans and invasive pneumococcal disease with a literature review. Purpura fulminans due to S. pneumoniae is exceedingly rare in immunocompetent patients and an unusual clinical manifestation of pneumococcal bacteremia.
... Una combinación de sepsis más un defecto hereditario parcial PS-PC es suficiente para iniciar PF 1 . Hay otras causas pocos frecuentes como: el síndrome antifosfolípido, la colitis ulcerosa, el lupus eritematoso sistémico, o luego de la administración de fármacos 10 . ...
La púrpura fulminante (PF) es una emergencia médica caracterizada por necrosis cutánea y coagulación intravascular diseminada (CID), que puede progresar rápidamente a falla multiorgánica por la oclusión trombótica de vasos. Aunque existen diversas causas de PF, el meningococo es el principal agente causante. Se presenta el caso de una mujer de 57 años de edad con clínica abrupta de fiebre, deterioro general rápido y progresivo, y lesiones purpúricas generalizadas, en las que se aisló en medio de cultivo agar sangre Neisseria meningitidis en sangre y líquido cefalorraquídeo. La evolución tórpida a pesar de antibioticoterapia, hemoderivados y terapia intensiva, demuestra una vez más la alta mortalidad de esta entidad a pesar de un tratamiento precoz.
Purpura fulminans is a rare and rapidly progressive septic process characterized by the development of hemorrhagic and ecchymotic lesions and skin necrosis. In this work, we report a case of a 52-year-old woman admitted to the Department of Emergency due to progressive purpura. The physical examination demonstrated a decreased skin temperature, unpalpable dorsalis pedis arteries and ecchymoses covering both lower extremities. Laboratory tests indicated DIC with prolonged aPTT, low PT, elevated D-dimer levels and a low platelet count. A diagnosis of purpura fulminans was made, and steroids, therapeutic plasma exchange (TPE) and empiric therapy, including antibiotic and anticoagulation therapy, were initiated immediately. Our treatment resulted in a good and sustained clinical response, as evidenced by the receding of blood blisters and the normalization of the patient's coagulation factors, but bilateral below-knee amputation was inevitable. Finally, the patient recovered well and was discharged home without any complications other than amputation.
Background:
Cutaneous manifestations of purpura fulminans (PF) present many challenges for clinicians and surgeons. In a state of septic shock complicated by limb ischemia, surgical interventions are necessary to control the pathological cascade and improve patient outcomes. The objective of this article was to report etiologies and surgical outcomes associated with cutaneous manifestations in adults.
Methods:
This systematic review and meta-analysis compared 190 adult patients with etiologies, signs and symptoms, and surgical outcomes associated with cutaneous manifestations of PF. The PubMed, EMBASE, Cochrane Library, Web of Science, and Scopus databases were systematically and independently searched. Patient and clinical characteristics, surgical interventions, outcomes, and complications were recorded.
Results:
Seventy-nine studies were eligible for the systematic review, and 77 were eligible for meta-analysis using Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) and Cochrane guidelines. A total of 71/190 (38%) cases reported surgical debridement. Fasciotomies were reported in 12/190 (6%) cases and 20 procedures. Amputations were reported in 154/190 (81%) cases. Reconstruction was reported in 45 cases. Skin grafts were applied in 31 cases. Flaps were used for reconstruction in 28 cases. Median (IQR) surgical procedures per patient were 4 (4, 5) procedures. Infectious organisms causing PF were 32% Neisseria meningitidis (n = 55) and 32% Streptococcus pneumonia (n = 55). Coagulase-negative Staphylococcus (95% confidence interval (CI)(8.2-177.9), p = 0.032), Haemophilus influenza (95%CI (7.2-133), p = 0.029), Streptococcus pneumonia (95% CI (13.3-75.9), p = 0.006), and West Nile Virus (95%CI (8.2-177.9), p = 0.032) were associated with significantly more extensive amputations compared to other organisms.
Conclusion:
This systematic review and patient-level meta-analysis found the most common presentation of PF was septic shock from an infectious organism. Neisseria meningitidis and Streptococcus pneumonia were equally the most common organisms associated with PF. The majority of cases were not treated in a burn center. The most common surgeries were amputations, with below-the-knee-amputations being the most common procedure. Skin grafting was the most commonly performed reconstructive procedure. The most common complications were secondary infections. Organisms with significantly more extensive amputations were coagulase-negative Staphylococcus, Haemophilus influenza, Streptococcus pneumonia, and West Nile Virus. Interpretation of findings should be cautioned due to limited sample data.
