Management of purpura fulminans skin loss at a regional burns centre: Sixteen-year experience.

INTRODUCTION: Purpura fulminans (PF) is a rare syndrome of cutaneous purpura which is the consequence of severe circulatory shock causing intravascular thrombosis, haemorrhagic necrosis, and consequent tissue loss. The aim of this study was to present our 16-year experience of managing PF in a regional burns centre. METHODS: We performed a single-centre retrospective case series of all patients admitted to the St Andrews Burns Centre at Broomfield Hospital, Chelmsford, Essex, UK, between June 2006 and July 2022 with a diagnosis of PF. Data were extracted by retrospectively searching hospital case notes. RESULTS: Thirteen individuals were identified [five children (mean age 5, range 1-14) and eight adults (mean age 39, range 24-54)]. The total body surface area of cutaneous necrosis ranged from 5% to 80%, with a mean of 27.2%. Patients were treated with an established surgical sequence of total wound debridement and immediate coverage with a cadaveric allograft, followed by staged wound autografting. The mean time from disease onset to wound autografting was 37.3 days (range 20-64 days). Eight individuals (61.6%) required major amputation of at least one limb (proximal to the ankle or wrist joint). Only one mortality (of 80% total body surface area skin loss) was observed in the identified cohort. CONCLUSIONS: The large body surface areas often involved in PF cases make management of these wounds well suited for burns centres, wherein established facilities and multidisciplinary teams exist that are familiar with managing large cutaneous burns. We provide a suggested algorithm to aid the management of PF.

Free flap for lower limb salvage in infectious purpura fulminans.

BACKGROUND: Infectious purpura fulminans is a disabling disease often leading to amputations. Free flaps preserve limb length, covering exposed areas. We examined the efficacy of free flaps for lower limb salvage in infectious purpura fulminans survivors by evaluating surgical management, walking ability and quality of life. METHODS: This single-center, observational, descriptive, retrospective study was conducted in from 2016 to 2019. Adult purpura fulminans survivors who received a free flap for lower limb salvage were included. Patient characteristics and data on surgical management and rehabilitation were collected. Quality of life (SF-36 questionnaire), limb function and walking ability were later evaluated post-surgically. RESULTS: The 6 patients included, mean age 38 years, had all required amputations. Nine free flaps were performed to cover important structures in 7 cases and for stump resurfacing in 2. All flaps were successful. Patients resumed walking at a mean of 204±108 days after the onset of purpura fulminans. Post-surgical evaluation was performed at a mean of 30±9.3 months. Five patients required secondary revision. All were independent for the activities of daily living. Mean physical component score was 37.6±9.4 and mental component score was 44.6±13.2 (minimum 0, maximum 100). CONCLUSIONS: Use of the free flap in patients with infectious purpura fulminans, after multidisciplinary reflection, is an appropriate procedure that preserves limb length. In spite of secondary complications, preservation of limb length enables patients to resume walking, with relatively good independence and quality of life.

Reconstructing Severe Lower Extremity Skin Necrosis in a Pediatric Patient.

ABSTRACT: Purpura fulminans can result in significant full-thickness wounds, posing a challenge in the pediatric population, given the paucity of donor sites for reconstruction. The authors present the case of an 11-month-old patient for whom a split-thickness skin allograft (TheraSkin) was successfully implemented as a temporizing measure for a large leg wound.

Early fasciotomies and plastic-surgical reconstruction may enhance preservation of functional extremity length in purpura fulminans.

BACKGROUND: Purpura fulminans (PF) is a distinct form of rare meningococcal septicaemia mostly in childhood which is characterized by high lethality, extensive necroses and mutilations of extremities. Other ethiologies are idiopathic forms or purpura neonatorum, which is marked by deficiency of Protein-C. PF is caused by micro-embolism of the vascular system, followed by quickly spreading necroses of skin and different organs.Modern concepts of intensive care treatment of the acute phase of this disease and early surgical intervention lead to a rising number of surviving patients requiring limb salvage.Aim of this study is to evaluate a possible lower morbidity and the grade of lower loss of function because of early surgical intervention. PATIENTS AND METHODS: Between the years 1998 and 2017 eight cases of PF at two large centers for critical wound care, the department of plastic-, hand- and reconstructive surgery of the university hospital of Regensburg and the department of plastic-, hand- and reconstructive surgery of the BG-clinic Ludwigshafen were included into our study.We retrospectively examined patients with PF who were stabilized by intensive care medicine and received surgical interventions. After survival of the acute phase (10 days), patients received plastic surgical reconstructions. RESULTS: A total number of eight patients could be included into our study. All patients survived the initial phase (10 days). All patients required plastic-surgical interventions to address extensive skin and soft tissue defects. Flap operations were judged necessary in five cases. Hereby four defects could be reconstructed by free tissue transfer, one with a local flap. Flap survival was 100 percent. One flap required revision of the arterial anastomosis of the flap arteria. Another flap got partial necrosis. One patient died due to multiorgan failure.Early debridements with consequent fasciotomies and secondary plastic-surgical reconstruction achieved good functional results. Limb salvage was accomplished in three patients. One patient died due to fulminant progress of the disease. CONCLUSION: Management of PF requires a multidisciplinary approach and close communication between the different subspecialties. Early debridements with consequent fasciotomies showed good results in salvaging subfascial muscle tissue in the extremities with satisfying functional results. Early surgical intervention is an important factor for improved limb salvage and survival.

Four-extremity amputation following disseminated intravascular coagulation and purpura fulminans.

Purpura fulminans (PF) is a rare but serious complication of septic shock in adults. The complex disease course makes it challenging to manage the condition. Here, we present the case of a healthy young woman who presented with sepsis and new-onset erythematous lesions 4 days after the vaginal delivery of a healthy baby. The infectious source could not be identified, and the patient was started on antibiotics and resuscitated. However, her condition worsened, and she developed disseminated intravascular coagulation and PF. The septic episode slowly decreased in severity, but she sustained extensive ischaemic injuries to her extremities, for which she underwent four-limb amputation.

Mowat-Wilson Syndrome Presenting With Purpura Fulminans.

Purpura fulminans is a rapidly progressive syndrome of intravascular thrombosis and hemorrhagic infarction of the skin. The most common infectious etiology is Neisseria meningitidis sepsis, and less commonly it has been documented as a complication of invasive Streptococcus pneumoniae In children who are otherwise healthy, splenic dysfunction is a significant predisposing factor for invasive pneumococcal infection. We present the case of a 10-month-old girl with a history of developmental delay, who developed an overwhelming infection complicated by purpura fulminans and was found to have previously undiagnosed Mowat-Wilson syndrome with anatomic asplenia. We propose screening patients with clinical features suggestive of Mowat-Wilson syndrome for asplenia to evaluate the need for additional preventive care.

[Management of purpura fulminans lesions in children]. / Prise en charge des lésions des parties molles du purpura fulminans chez l'enfant.

Purpura fulminans is a pediatric life-threatening emergency with a significant mortality, combining: septic shock, extensive purpuric lesions and disseminated intravascular coagulation. The most frequent bacterial pathogen is the meningococcus. The medical management includes antibiotics, corticoids, vascular filling and catecholamines. Purpura fulminans is characterized by the extent of hemorrhagic and mainly thrombotic lesions, attributed to the alteration in the vascular endothelium functions. Damage of soft tissues combines large necrotic areas and more or less extensive distal ischemic lesions. Necrotic lesions can be deep, reaching skin, subcutaneous tissue, fascia, muscle and sometimes even the bone. The importance of the aesthetic and functional sequelae as well as future quality of life, depend on the quality of surgical management for these wide and deep lesions. Fasciotomy is sometimes urgently needed in the case of a clinical compartment syndrome, confirmed by a high-pressure measurement in the muscle compartments. Debridement of necrotic lesions and amputations are only performed after a clear delineation of necrotic areas, between 10 days and 3 weeks of evolution. If an amputation is necessary, it must focus on the residual bone length, considering the child's growth potential. The coverage of tissue loss uses all the plastic surgery techniques, more or less complex, in order to reduce scars to minimum for these children. Rehabilitation follow-up includes physical and psychological care, which are essential until adulthood.

Delayed Surgical Debridement and Use of Semiocclusive Dressings for Salvage of Fingers After Purpura Fulminans.

Background: Purpura fulminans is a condition characterized by rapidly evolving skin necrosis and disseminated intravascular coagulation. Early recognition and aggressive supportive management has led to a decrease in its mortality rate, but most of these patients must undergo extensive soft tissue debridement and partial or total limb amputation. There is controversial evidence about the timing of surgery, suggesting that some patients may benefit from delayed debridement with limb preservation. Methods: We present a case of an 86-year-old patient who developed skin necrosis of his four limbs after infectious purpura fulminans. He was treated in the ICU with supportive measures and antibiotic treatment. Surgical debridement was delayed for 4 weeks until necrosis delimitation. Results: Only upper extremity debridement was necessary. Four fingers, including one thumb, were salvaged and successfully treated with semi-occlusive dressing without complications. Conclusion: Early recognition of infectious PF and timely supportive management are important pillars of its treatment. Delayed surgical debridement allows for less aggressive resection and good functional outcome.

Purpura fulminans managed with multi-limb amputation: substituted judgment and surrogate decision-making in the surgical management of necrotizing soft tissue infections.

BACKGROUND: Purpura fulminans (PF) is a rare but lethal complication of severe infection. Aggressive surgical debridement of irreversibly devitalized tissue improves survival frequently at the cost of disfigurement. The ethical dilemma of surrogate decision-making for these often incapacitated patients presents a unique challenge for acute care surgeons managing necrotizing soft tissue infections (NSTI). METHODS: Case presentation and scholarly discussion of substituted judgment. RESULTS: A previously healthy 72-y-old fisherman developed PF as a consequence of Neisseria meningiditis severe sepsis requiring bilateral partial finger amputations and bilateral below-knee amputations of the affected gangrenous extremities. Skin biopsy confirmed the clinical impression of disseminated intra-vascular coagulation (DIC). During his 55-d hospitalization, medical decisions were made by a surrogate because the patient's mental status failed to recover to his pre-morbid baseline. A literature review revealed a paucity of data on the accuracy of a health care agent's ability to represent a patient's preferences accurately in elective as well as emergency surgery. CONCLUSIONS: Patients with NSTI and the surgeons who care for them are often confronted with the need to make prompt decisions of radical debridement or amputation. These patients are frequently incapable of making these decisions because of the severity of systemic illness. In such cases, physicians must help patient surrogates or health care agents (when identified) navigate a complex process of acute interventions balancing known or inferred patient's wishes. We urge surgeons to become familiar with the concept of substituted judgment and the challenges of surrogate decision-making.

Two cases of meningococcal purpura fulminans: the 'less is more' approach.

This study presents a case series of patients with meningococcal purpura fulminans who were treated at a tertiary referral centre within a few days of each other. Presenting with signs and symptoms of florid meningococcal sepsis, they were managed initially by physicians and intensivists, whereas the development of large purpuric areas and tissue necrosis was managed expectantly by plastic surgeons. When the patients were deemed to have recovered clinically and the necrosis delineated, surgical management was implemented with subsequent involvement of various rehabilitation services. This article highlights the cases of two patients, and their clinical presentation, management and rehabilitation together with a current literature review on this area.

Management of septic skin necroses.

Necrotizing fasciitis (NF) and purpura fulminans (PF) are conditions with extensive septic skin necroses that are associated with significant morbidity and mortality. NF is caused by fulminant bacterial spread on the superficial muscle fascia, Group A streptococcus being the main microorganism responsible for it. The major challenge NF poses is timely recognition. Although crucial for patient survival, early diagnosis is difficult because paucity of specific early findings does not allow setting NF apart from other, less severe, differential diagnoses. Surgical therapy consists of early and aggressive debridement of all affected tissue, even if large disfiguring wounds are left back. The responsible microorganism for PF in children is predominantly Neisseira meningitidis. Endotoxin triggered misbalance of anticoagulant and procoagulant activities of endothelial cells leads to disseminated intravascular coagulation (DIC) followed by microvascular thrombosis and bleeding, resulting in hemorrhagic skin infarction and limb ischemia. Although survival in PF is not dependent on surgery, and surgery plays not a key role in the early phase of the disease, early surgical consult to assess if limb perfusion can be improved to achieve limb salvage is still absolutely necessary. Debridement should be postponed until clear demarcation has established. Large defects after NF and PF can be successfully reconstructed with vacuum-assisted fixation of Integra (Integra LifeSciences Corporation, Plainsboro, New Jersey, United States) artificial skin before split-thickness skin grafting. This provides good functional and cosmetic results as well as good stump coverage in case of amputation in PF.

Meningococcal sepsis and purpura fulminans: the surgical perspective.

Meningococcal sepsis and purpura fulminans is a rare but highly lethal disease process that requires a multidisciplinary team of experts to optimise morbidity and mortality outcomes due to the breadth of complications of the disease. The surgical perspective involves the critical care management which utilises all currently available measured outcomes of critical care management as well as experimental therapies. Limb loss is common, and is reflective of the high incidence of compartment syndrome compounded by the significant soft tissue loss secondary to purpura and limb ischaemia, presumptively due to digital microemboli. A multidisciplinary approach involving current standards in critical care and early surgical evaluation are important in improving patient outcomes and limb salvage.

Toxic shock syndrome due to group A beta-hemolytic streptococcus presenting with purpura fulminans and limb ischemia in a pediatric patient treated with early microsurgical arteriolysis.

We describe a 2.5 year-old child with toxic shock syndrome due to group A beta-hemolytic streptococcus (GABHS) who presented with purpura fulminans and limb ischemia treated with early microsurgical arteriolysis. The clinical picture of toxic shock syndrome (TSS) presenting with purpura fulminans and limb ischemia is an exceptionally uncommon finding in sepsis due to GABHS. This is the first case of purpura fulminans caused by GABHS reported in Europe and the third one described in the literature (Dhodapkar et al., 2000[1]; Renaud et a., 2011[2]).

Plastic surgical treatment of purpura fulminans: Long-term follow-up of two patients.

Purpura fulminans (PF) is a rapidly progressing, potentially life-threatening condition characterised by disseminated intravascular coagulation (DIC) and haemorrhagic infarction of the skin. Plastic surgical treatment of PF has never been reported in Scandinavia. The aim of this report was to review plastic surgical treatment of PF and the long-term results of two patients treated in our department. Both patients presented to a community hospital with skin lesions looking like simple traumatic skin bleeds a period after a Varicella infection. They were initially treated at the community hospitals with broad-spectrum antibiotics and adjunctive therapies. When their condition permitted, they were transferred to the department of paediatrics of Skåne University Hospital where their DIC was treated further. The patients were transferred to the department of plastic and reconstructive surgery, when medically stable, and operated on with debridement, and amputation of a toe in one patient, and the application of autologous skin grafts. The children made an excellent recovery and were discharged home after 1.5 months and 3 weeks, respectively. At follow-up, 14 years and 8 years later, respectively, the patients were fully recovered and no secondary corrections were indicated. In conclusion, debridement of necrotic tissue should be performed in a department of plastic and reconstructive surgery as soon as the child is clinically stable, and skin grafting when the wound bed permits it. Follow-up should be performed in the same fashion as for full-thickness burns.

Assessment of subtotal nasal reconstruction in a child after 6 years of follow-up.

Nasal amputation in the child is infrequent, and the procedure is not clearly defined. Early reconstruction certainly improves the child's life and social integration. The major risk is unsatisfactory growth of the reconstructed nose, which may persuade some surgeons to postpone the operation. The evaluation of this nasal reconstruction in a 5-year-old boy who had undergone subtotal amputation of the nose after 6 years of follow-up tends to confirm the therapeutic choice of early nasal reconstruction in the child. The results were considered quite satisfactory esthetically and psychologically beneficial and are concordant with those of other studies. Early management allowed us to obtain an esthetically acceptable reconstruction with respect to growth, thereby enabling the child to resume his studies rapidly and to achieve good social integration.

[Purpura fulminans: functional results in two paediatric patients after suffering multiple amputations]. / Púrpura fulminante: resultado funcional en 2 pacientes pediátricos tras sufrir múltiples amputaciones.

Purpura fulminans (PF) is an unusual haemorrhagic process that is usually associated with meningococcal sepsis and other infectious processes. It usually affects neonates and young children, and starts with a benign infection that progresses to a high fever, purpura ecchymosis, disseminated intravascular coagulopathy, necrosis and gangrene. The treatment of these children usually requires making difficult decisions, since the surgeon and the families must come to terms with the possibility of following an aggressive line of treatment that could lead to multiple mutilating sequelae, or follow palliative treatment. In this study, we review the clinical presentation, treatment and results of two cases of PF treated in our hospital between the years 2002 and 2005. The children presented in this study had a good long-term functional result and an acceptable quality of life, despite being subjected to multiple amputations.

Nose and upper lip reconstruction for purpura fulminans.

BACKGROUND: Purpura fulminans (PF) is a rare syndrome of intravascular thrombosis and haemorrhagic infarction of the skin. The initial symptom of PF is peripheral purpura which progresses to necrosis very rapidly. The prognosis of PF is poor, and the mortality is reported to be around 40%. Even if the patient survives, the patient may require amputation or reconstruction for limbs and facial necrosis. CASE REPORT: A 48-year-old male suffered from PF following a left cerebellopontine angle tumour excision. His nose and upper lip fell into necrosis afterwards. We performed nose and upper lip reconstruction at 8 months after the onset. We used a forehead flap for the nasal reconstruction and a free forearm flap for the lining. His upper lip was reconstructed with bilateral nasolabial orbicularis oris myocutaneous flaps. RESULTS: The colour and texture match of the reconstructed nose and lip is good. He could open his mouth wide enough and close completely. CONCLUSIONS: Facial reconstruction after PF is very difficult, because the patient has extensive scarring around the defect and there is little intact facial tissue. However, we performed a facial reconstruction using local flaps as much as possible, and obtained good results.

Surgical management of acute infectious purpura fulminans.

Purpura fulminans is a syndrome characterized by hemorrhagic infarction of the skin and underlying soft tissue as a result of disseminated intravascular coagulation and intravascular thrombosis. In this study, the authors report their experience with surgical intervention for acute infectious purpura fulminans (AIPF). A retrospective chart review was performed including all patients diagnosed with AIPF from January 1, 2006, to December 31, 2008, and treated at an academic medical center. Primary endpoints of interest were overall survival rate and the need for and level of eventual amputation. Improvement in limb perfusion was included as a secondary endpoint. Nine patients were diagnosed with AIPF at the authors' institution during a 3-year period, and seven of these diagnoses were made within 12 months. Overall mortality was 44% (5/9). Amputation was required in 80% of survivors (4/5). All patients explored within 24 hours of diagnosis had evidence for compartment syndrome with visible bulging muscle on fascial release. AIPF is a devastating disease with significant mortality and morbidity primarily related to the loss of multiple limbs. This study suggests that early diagnosis and surgical intervention in the form of compartment release and sympathectomy should be performed concurrently with the initial treatment of sepsis to minimize amputations in surviving patients.