! The Northern Ohio Foot and Ankle Journal Official Publication of the NOFA Foundation Purpura Fulminans in the Lower Extremity: A Case Report and Review by Dustin Huntsman DPM1, Chad Knight, DPM2 and Michael Maier, DPM3

The Northern Ohio Foot and Ankle Journal 4(22): 1-4

Abstract: Purpura fulminans (PF) is an extremely rare condition that is associated with very high mortality rates secondary to severe multi-systemic thrombotic events. The mechanism by which this occurs is poorly understood but is often associated with bacterial infections. Patients who survive are often faced with extensive skin necrosis, resulting in amputations and significant scarring. Early recognition, treatment with anticoagulation, transfusion of deficient proteins, eradication of infection, and delayed surgical debridement are essential in management of PF. In this case report we discuss the case of PF in a 34-year-old intravenous drug user (IVDU).

Key words: purpura fulminans, disseminated intravascular coagulation, protein C deficiency

Accepted: February, 2018 Published: March, 2018

This is an Open Access article distributed under the terms of the Creative Commons Attribution License. It permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. ©The Northern Ohio Foot and Ankle Foundation Journal. (www.nofafoundation.org) 2014. All rights reserved.

infections and coagulopathy have been found to

trigger PF [5]. PF occurs most frequently in pediatrics Purpura fulminans is a rare disorder with a history of meningococcemia. Purpuric lesions

on the upper and lower extremities are characteristic characterized by rapid skin necrosis and disseminated of PF. Skin necrosis is a result of acute thrombotic intravascular coagulation (DIC). It is postulated that a events in small deficiency of protein C and dysfunction of the

Address correspondence to: [email protected]. 1Podiatric Medicine and protein C-thrombomodulin pathway, as well as other Surgical Resident, Mercy Regional Medical Center. 2Podiatric Medicine and Surgical Resident, CCF-Oklahoma City. 3Podiatric Medicine Physician, systems, exert a negative regulatory effect on Cleveland Clinic. coagulation but overall the mechanism is still poorly to medium sized blood vessels [4]. In severe instances, understood [1]. Acute sepsis, viral or bacterial multi-systemic thrombotic events can lead to multi-

The Northern Ohio Foot & Ankle Foundation Journal, 2018 Volume 4, No. 22, March 2018 Huntsman, Knight, Maier positive for tricuspid valve repair and permanent organ failure and/or death [5]. There is no consensus pacemaker placement one year ago. on the most appropriate treatment because there are Patient was transferred to the intensive care unit only a few studies, with limited sample sizes, to draw (ICU) febrile and intubated. She had profound septic shock with acute respiratory failure, and acute kidney from. Early recognition and aggressive supportive injury. On physical exam, the patient had palpable radial pulses and audible dorsalis pedis and posterior management have been essential in decreasing the tibial pulses upon Doppler ultrasound bilaterally. Dermatologically, cyanotic to violaceous hues were mortality rates. It is estimated that greater than 50% noted to the nose, ears, arms and legs. Stellate of patients will undergo surgical debridement and/or purpuric plaques were also noted on the arms and legs along with scattered bullae to the lower partial/total limb amputation [6]. Surgical timing is of extremities. Skin temperature was warm to cold, from the tibial tuberosity to the lesser digits. Neurologically, debate in the literature and can be controversial. Some protective sensation was absent distal to the midfoot on the left and intact on the right. Musculoskeletal authors suggest early surgical debridement while examination was deferred due to pain. others recommend delaying surgical intervention until demarcation to maintain maximum limb length and prevent unnecessary surgeries [8]. Clinicians should use caution and clinical judgment when treating Figure 1. Diffuse purpura and bullae formation patients with PF. The goal of treatment is to preserve Laboratory findings were significant for leukocytosis, low fibrinogen and protein C, elevated aPPT, PT, and maximal functionality without causing additional D-Dimer. Given her clinical presentation and systemic inflammatory insult [4]. laboratory findings the diagnosis of PF was made and heparin and protein C concentration were administered. Methods Several imaging studies were performed. Radiographs A search of the literature was conducted regarding PF of bilateral feet were unremarkable. Transesophageal until February 2018. References from the appropriate echocardiography showed vegetations on the repaired articles were also reviewed to find all reports and tricuspid valve, which later required a subsequent outcomes of PF in the literature. repair of her tricuspid valve. Bilateral lower extremity venous duplex ultrasounds were positive for deep Case Report vein thromboses in bilateral popliteal veins. A 34-year-old female IVDU was transferred from an outside hospital after sustaining a fall at home. She Throughout her hospitalization, her dermatological reported several episodes of nausea, vomiting, and anterior chest pain. Her past medical history was exam continued to worsen. On the right lower significant for hepatitis C and acute MRSA tricuspid valve endocarditis. Her past surgical history was extremity, necrotic lesions were noted to the The Northern Ohio Foot & Ankle Foundation Journal, 2018 Volume 4, No. 22, March 2018 Huntsman, Knight, Maier anterolateral and posterior aspect of the leg. The allow for full demarcation of all wounds. The patient distal tufts of toes 3-5 developed gangrenous was discharged and will undergo a below-the-knee changes. On the left lower extremity, necrosis was amputation on the left and distal symes amputations noted to the anterolateral aspect of the leg, to the to the affected digits on the right. entire forefoot and to the plantar lateral aspect of the midfoot and rearfoot. All wounds appeared to be dry and stable with mild peri-wound erythema.

Figure 3. Progressive necrosis noted after 60 days

! Discussion Figure 2a. Increased necrosis, after 30 days, to the The prevalence and incidence of PF is not exactly right lower extremity. known but is rare, especially in the adult population. PF presents acutely with painful skin lesions, erythematous macules, and purpuric rashes with vesicles and bullae. There are other conditions with purpuric entities such as calciphylaxis, necrotizing fasciitis, thrombotic thrombocytopenic purpura, meningococcemia, and toxic shock syndrome [4]. Figure 2b. Increased necrosis, after 30 days, to the The mechanism of infectious causes is not well left lower extremity. understood. Treatment of sepsis-induced PF is centered on supportive management, usually in the ICU. Early recognition is key. Typical management includes ventilation, fluid resuscitation, pressor support, and blood transfusions [4]. Heparin infusion has shown a 12% mortality reduction in sepsis and DIC [12]. Evidence of the use of activated protein C and high-dose anti-thrombin have failed to show a decrease in the mortality rates and is reserved for severe cases [13]. Many physicians are faced with the A multi-specialty decision was made to delay surgical decision to use vasopressors, which are known to significantly increase the risk of acral necrosis in debridement and continue conservative treatment, patients with sepsis, endocarditis, DIC and vasculitis. It is recommended that pressor support be used with which consisted of betadine wet to dry dressings and caution in these patients. Modern medicine has semi-occlusive Vaseline gauze to areas of necrosis, to increased the survival rates and early recognition of The Northern Ohio Foot & Ankle Foundation Journal, 2018 Volume 4, No. 22, March 2018 Huntsman, Knight, Maier PF throughout recent years, which has changed the Dysregulated Hemostasis." Transfusion medicine reviews timing of surgical intervention. (2017). 2. Chalmers E, Cooper P, Forman K, Grimley C, With decreasing mortality rates, there is increasing Khair K, Minford A, Morgan M, Mumford AD controversy of surgical debridement and sequel (2011). "Purpura fulminans: recognition, diagnosis management. Surgical treatment is aimed at and management". Arch Dis Child. 96 (11): 1066– preserving maximum functionality and limb length. 1071. Davis et al. performed early fasciotomies in 9 pediatric patients without evidence of preventing 3. Ghosh SK, Bandyopadhyay D, Dutta A (2009). amputation in those patients. There has been "Purpura fulminans: a cutaneous marker of controversy over acute intervention versus delayed disseminated intravascular coagulation". West J Emerg surgical intervention. The trend throughout the 4. Pino, Paula A., Javier A. Román, and Felipe literature is towards delayed surgical debridement, to Fernández. "Delayed Surgical Debridement and Use allow the soft tissue to fully demarcate before of Semiocclusive Dressings for Salvage of Fingers intervening. However, there is a report of 7% of After Purpura Fulminans." HAND 11.4 (2016): patients with PF having compartment syndrome NP34-NP37. requiring early fasciotomies [9]. 5. Davis, Mark DP, Katrina M. Dy, and Steven Nelson. "Presentation and outcome of purpura Most of the literature represents cases in the pediatric fulminans associated with peripheral gangrene in 12 population. There are few reported cases in adult patients at Mayo Clinic." Journal of the American patients, such as ours. In the pediatric population Academy of Dermatology 57.6 (2007): 944-956. skin grafts, free flaps, and advanced wound care products have been described with success. Many 6. Davies, MS, Nadel, S, Habibi, P, Levin, M, Hunt, studies, such as Pino et al., report the success of DM. The orthopaedic management of peripheral semi-occlusive material in preserving length of digits, ischaemia in meningococcal septicaemia in children. J when treating these wounds conservatively. Bone Joint Surg Br. 2000;82(3):383-386 7. MacLennan, SE, Kitzmiller, WJ, Yakuboff, KP. PF is an extremely rare and complex condition. Free-tissue transfer for limb salvage in purpura Patients present with extensive past medical histories fulminans. Plast Reconstr Surg. 2001;107(6): and severe systemic illnesses including systemic 1437-1442 shock, multi-organ failure, DIC and extensive soft tissue necrosis. Due to the impending and extensive 8. Warner, PM, Kagan, RJ, Yakuboff, KP Current necrosis, the morbidity associated with PF is management of purpura fulminans: a multicenter crippling. Mortality rates vary throughout the study. J Burn Care Rehabil. 2003;24(3):119-126 literature and may range from 25-44%. Roughton et 9. Childers, Ben J., and Brando Cobanov. "Acute al. reported a 44% mortality rate in nine patients with infectious purpura fulminans: a 15-year retrospective PF [5, 12]. Treatment should be focused on early review of 28 consecutive cases." The American recognition, supportive treatment, delayed surgical Surgeon 69.1 (2003): 86-90. debridement, and semi-occlusive dressings for 10. Edlich, Richard, et al. "Modern concepts of the conservative management, in order to maximize diagnosis and treatment of purpura functional outcomes. fulminans." Journal of Environmental Pathology, Toxicology and Oncology 27.3 (2008). References 11. Roughton, Michelle C., Shailesh Agarwal, and Lawrence J. Gottlieb. "Surgical management of acute 1. Colling, Meaghan E., and Pavan K. Bendapudi. infectious purpura fulminans." Journal of Burn Care & "Purpura Fulminans: Mechanism and Management of Research 32.2 (2011): 231-236. The Northern Ohio Foot & Ankle Foundation Journal, 2018 Volume 4, No. 22, March 2018 Huntsman, Knight, Maier 12. Yamakawa K, Ogura H, Fujimi S, et al. Recombinant human soluble thrombomodulin in 13. Warren BL, Eid A, Singer P, et al. Caring for the sepsis-induced disseminated intravascular coagulation: critically ill patient. High-dose antithrombin III in a multicenter propensity score analysis. Intensive Care severe sepsis: a randomized controlled trial. JAMA. Med. 2013;39(4):644-652 2001;286(15):1869-1878.

The Northern Ohio Foot & Ankle Foundation Journal, 2018