Two cases of meningococcal purpura fulminans: the ‘less is more’ approach

Abstract

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.

Introduction

Meningococcal purpura fulminans (MPF) is a rare complication of meningococcaemia presenting with dermal and epidermal tissue necrosis and was first described by Guelliot in 1884 1. MPF first presents with high fever, chills, severe myalgia, headache and skin and mucosal petechiae 2. The condition may rapidly progress manifesting as septic shock, disseminated intravascular coagulation, multiorgan dysfunction syndrome and a worsening of the skin condition 3. Neisseria meningitidis, the causative organism, reaches the peripheral tissues through white blood cells and releases a powerful endotoxin that induces oedema formation and capillary thrombosis 4. This thrombosis of small vessels typically manifests in an end‐arteriole pattern leading to tissue necrosis of body extremities 2, 4, 5, 6. Alternative diagnoses may have similar cutaneous presentations, including vasculitis, simple purpura and other causes of gangrene 7.

Colonisation with strains of N. meningitidis serogroups B and C is very common leading to potential for haematogenous spread through nasopharyngeal transmission 4, 8. The incidence of meningococcal infections ranges between 0·5 and 1·5 cases per 100,000 per year with children and adolescents more prone than adults 8. Five to 25% of patients with meningococcal disease develop the more severe end of the spectrum of MPF 3, 8. Although N. meningitidis is the commonest haematogenous infection known to induce MPF, the condition can be caused in a synergistic fashion by several microorganisms such as Staphylococcus aureus, Streptococcus pneumoniae, Haemophilus influenzae, Group A streptococci, Legionella pneumophil and viral infections 2, 3, 4, 8. The incidence of MPF from N. meningitidis is certainly higher in children and adolescents and it is very rare in the adult population 8. This severe systemic illness has an overall high mortality rate of 20–70%, although reports in the literature are from small patient populations 8, 9, 10.

The literature has shown that aggressive acute treatment, both in the critical care approach (fluid resuscitation, specific antibiotic therapy and respiratory and inotropic support) and in terms of surgery (excision and skin grafts), can improve the survival rate 2, 3. This study presents two cases of MPF where an initial conservative approach was chosen to allow the necrotic areas to clearly demarcate with resultant preservation of native tissue.

Case 1

A 36‐year‐old male patient presented feeling generally unwell and having developed a purpuric rash over the preceding 24 hours. On admission he was in severe septic shock, empirical antibiotics were commenced and he was transferred directly to intensive care for ventilatory, inotropic and renal support. He was later diagnosed with meningococcal septicaemia through a positive meningococcal polymerase chain reaction (PCR) screening test. As illustrated in Figures 1, 2, 3, the purpura fulminans affected his toes, heels, limbs, nose, ears and penis. The patient was treated on the intensive care unit for 22 days and no surgical intervention was performed during the acute illness. Sharp debridement of necrotic areas was undertaken on day 32; this included the amputation of multiple toes and disarticulation of his right hand through the wrist joint. The resulting soft tissue wounds were managed conservatively with regular conventional dressings, and one further surgical debridement of the foot wounds was carried out on day 89. Secondary to his meningitis he had bilateral sensorineural hearing loss, for which he was provided with hearing aids and is awaiting cochlear implants. He received intensive rehabilitation from physiotherapists and occupational therapists, and was seen regularly in the diabetic foot clinic for individualised footwear to allow mobility whilst aiding wound healing. He was discharged home after 64 days as an inpatient with follow‐up from a variety of specialities: otolarynogologists, plastic surgeons, infectious diseases, diabetic foot clinic, physiotherapists and prosthetic services.

Figure 1.

Case 1. The two top images illustrate the purpuric areas seen on upper limbs whilst patient was acutely unwell on intensive care. The bottom images show his right hand after demarcation of the necrotic tissue prior to wrist disarticulation.

Figure 2.

Case 1. The top image represents the appearance of the patient's leg on the intensive care unit, whereas the bottom images were taken after demarcation of necrotic tissue prior to debridement.

Figure 3.

Case 1. The top images illustrate the purpuric areas on his face and ear whilst acutely unwell, and the bottom images show his nose after demarcation of necrotic tissue.

Case 2

A 23‐year‐old male patient presented in septic shock with a history of fever, being generally unwell and having developed a purpuric rash. He was treated with intravenous antibiotics and required a 17 days intensive care admission for ventilatory, inotropic and renal support. He was later diagnosed with meningococcal septicaemia through a positive meningococcal PCR screening test and the purpura fulminans involved his limbs, feet and heels (see Figures 4, 5, 6). After initial successful treatment, he underwent a period of medical optimisation with a high degree of input from the nutritional team before the first debridement was carried out on day 33. This procedure involved sharp debridement of all necrotic areas, but excluded debridement of the heels with concern that exposure of the calcaneus may result. The defects over both patellae were managed with topical negative vacuum pressure therapy with the remaining areas treated with conventional dressings. Two further debridements were performed in theatre before the wounds were deemed suitable for split skin grafting on day 44, and as can be seen in Figure 6 these took successfully. The heels were not debrided until day 88 of admission; these were initially dressed using PICO therapy, but when discharged home on day 104 conventional dressings were being applied. Intensive rehabilitation was required with high input from the physiotherapy and occupational therapy departments, again the input from the diabetic foot clinic proved essential in providing individualised footwear to allow mobility whilst aiding wound healing (Figure 7).

Figure 4.

Case 2. The purpuric areas on the upper limbs.

Figure 5.

Case 2. From left to right, purpuric areas on his lower limbs whilst on intensive care, the lesions over his patellae after demarcation and on the right after successful debridement and split skin grafting.

Figure 6.

Case 2. From top to bottom, purpuric areas on his heels whilst on intensive care, the lesions after demarcation and after debridement.

Figure 7.

Clinical photographs of the limbs 8 months following surgery.

Discussion

This study describes two cases of MPF from presentation, initial resuscitation and intensive care support through to the acute and late phases of reconstructive surgery. Patients' consent was obtained. As shown in Figures 1, 2, 3, 4, 5, 6, the cutaneous manifestations of MPF characteristically affect end arterioles such as digits, nose, ears and penis. These cases although rare illustrate an important clinical entity and the requirement for multimodal and multidisciplinary management. Awareness of its presentation allows clinicians to recognise this challenging clinical problem early and instigate prompt management that can lead to better outcomes than previously achieved. Rapid advances in supportive therapy in intensive care and involvement of multidisciplinary teams have resulted in more of these complex cases surviving their acute illness and being in a position to undergo reconstruction 2, 3, 5.

Traditionally, the function and rehabilitation of these patients was not addressed specifically as stabilisation and immediate management was understandably the clinical priority. However, because of the abovementioned advances, more and more patients will need careful interspecialty discussion as well as careful counselling themselves to achieve effective rehabilitation, thus returning to their pre‐morbid state. The involvement of multiple medical specialities is likely to be required but must be targeted to the individual patient requirements. For example, in case 1, the patient suffered sensorineural hearing loss secondary to irreversible vestibulocochlear nerve damage and ongoing input from Otolaryngology colleagues was required; the patient is now awaiting a cochlear implant. Such aspects of these cases emphasise the true and holistic nature of the patient management. Intensive rehabilitation is also required for these patients who have been critically unwell for a prolonged period of time. Specific multidisciplinary teams including occupational therapy, physiotherapy and social care are needed to address issues regarding mobility and activities of daily living to facilitate seamless integration back into society.

With respect to such soft tissue injuries, some authorities would argue the merits of early excision and grafting 2, 3, 5. In both of our cases, we opted for a vigilant approach, instead allowing the wounds to manifest themselves whilst the bacterial load was reduced through supportive therapies 11. Great emphasis was placed on traditional wound care, conventional dressings and optimising the patient. Thus, even wounds that initially appeared incurable healed with conservative treatment, whilst reconstructive surgery (skin grafting) was reserved for optimal wounds. This ‘less is more approach’ proved favourable in both of these cases as less native tissue required excision leading to improved aesthetic and functional outcomes 12, 13.

These cases were approached using the Jackson's Burn Wound model (Figure 8). In the acute management of burns through careful and considered fluid resuscitation, the zone of stasis can be converted into a zone of hyperaemia rather than a zone of necrosis. Similarly, we waited for the wounds to manifest themselves, allowed for peripheral tissue perfusion to improve and then made judgements on the tissues thereafter.

Figure 8.

The Jackson's burn wound model 14.

Reconstruction in the clinical setting of MPF challenges some reconstructive philosophy. Flap reconstruction provides superior results in terms of blood supply, wound healing, reducing infection as well as in regard to aesthetic and functional outcome 3, 4, 5, 11. However, extremity circulation that is sub‐normal in the acute–chronic phase perhaps would not confer any further advantage as flap physiology would be sub‐optimal and free flaps may not be a viable option (because of concurrent medical factors, comorbidities and donor sites). As most MPF patients are young they have a good chance of spontaneous healing.

MPF is a rare condition that requires early recognition, instigation of antibiotic therapy and supportive therapy on the intensive care unit. We have illustrated that the soft tissue manifestations of the disease can be successfully managed with a conservative approach which we believe is the right choice to preserve maximal native tissue. Rehabilitation of these patients is lengthy and requires the involvement of a variety of medical specialties to allow optimisation of functional outcome and integration back into society.