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Home , Healing, Necrosis, Urticarial vasculitis, Warfarin necrosis, Wound healing

Clinical PRACTICE DEVELOPMENT Clinical PRACTICE DEVELOPMENT How to diagnose and treat haemorrhagic

Haemorrhagic skin necrosis is a common manifestation of a number of different pathological processes that can evolve dramatically and carry a grave prognosis. The divergent patho-physiological basis of the condition and the many specialities involved in the initial and subsequent care of the patient means that not all patients are seen by care professionals. Yet such patients do present to wound clinics and invariably develop if the affected area is large. It is therefore important that wound care experts are familiar with the common causes of haemorrhagic skin necrosis and are able to instigate the appropriate investigations and treatment.

Girish K Patel

coloured skin. Diagnosing the upon the identification of a that of these symptoms may be a clinically causes vessel . KEY WORDS intimidating prospect. But when Haemorrhagic skin necrosis considering the diagnostic possibilities, Determining the cause Thrombosis it is important first to establish the of sudden thrombosis of the skin vessels Cutaneous necrosis pathological sequence of events that Rudolf Virchow worked extensively on have led to this presentation. the causes of thrombosis in the early 1900s and his findings still provide The process resulting in a useful framework. Virchow’s triad haemorrhagic skin necrosis begins consists of: with the sudden thrombotic occlusion 8 Alterations to flow he vast majority of wounds of single or multiple blood vessels (haemostasis) presenting to a supplying the skin. The compromised 8 to the vascular T clinic are either post-surgical blood vessels leak red cells into the 8 Alteration in blood constituents wounds or chronic ulcers; in over 90% surrounding tissues where they become (hypercoagulability) (Malone, 2005). of cases the aetiology of the wound can trapped within the ensuing be attributed to either venous disease necrosis. The subsequent deoxygenation that cause thrombosis with or without coexistent arterial of haemoglobin in the red blood typically fit into one or more of these disease, arterial disease, , cells results in the black colour of groups and each group in turn has a pressure ulceration or trauma (including the necrotic tissue. In addition, many particular pattern of skin necrosis. By ) (Banerjee et al, 2001; Patel are synchronously released by looking at the pattern and distribution et al, 2006). Therefore a patient dying cells and promote inflammation. At of skin necrosis it should be possible, with rapidly evolving, painful black the boundary of the necrosis the blood using Virchow’s triad, to shorten the list haemorrhagic skin necrosis is unusual vessels dilate, resulting in of possible diagnoses. and may be daunting for the clinician. which gives rise to the dusky grey-red This article describes the presentation, colour of the surrounding skin. Haemostasis leading to haemorrhagic skin necrosis diagnosis and management of Probably the most common example haemorrhagic skin necrosis. The necrotic tissue initially swells of microcirculation haemostasis is and later it will be sloughed off after skin necrosis resulting from pressure Haemorrhagic skin necrosis: the underlying tissue has healed with ulceration (Whitney et al, 2006). In this a diagnostic framework a . However, if the affected area is case the necrosis is extremely localised Patients with haemorrhagic skin sufficiently large the fibrin molecules and is characteristically centred over a necrosis may present with one or more will solidify to form an eschar that will bony prominence. painful and extremely tender black shrink as the underlying wound heals eschars surrounded by dusky grey-red (Carlson and Chen, 2007). The external pressure compresses the tissue against the underlying bone, Girish K Patel is Honorary Clinical Tutor, Department of Thus, the successful diagnosis of so that blood no longer flows effectively Dermatology, Cardiff University haemorrhagic skin necrosis depends through the tissue. There is a tendency

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Table 1 Table 2 Factors associated with the development of pressure ulcers Factors associated with reduced blood flow that predispose to haemorrhagic skin necrosis Extrinsic factors Intrinsic factors Reduced Reduced blood flow in Moisture Systemic blood flow multiple vessels Friction Sensory neuropathy with or without motor neuropathy in a solitary Shear Tissue oedema vessel Malnutrition Trauma Trauma Hypotension Chilblains Chilblains Medications that interfere with healing, e.g. systemic corticosteroids Severe hypotensive shock Diabetes mellitus Drugs, e.g. sympathomimetics and ergot alkaloids Reduced consciousness Vibration white finger Incontinence Raynaud’s disease Coexistent skin diseases Berger’s disease Blood hyperviscosity, e.g. multiple myeloma to think that pressure ulceration is sites, the digits. Digital necrosis may be limited to the sacral area or hip, but a prominent feature (Figures 1 and 2). these same factors can result in ulcers The causes of this type of skin necrosis Atherosclerosis is the most over any bony prominence, including can be divided into those affecting common cause of injury to the vascular the scalp, thoracic vertebrae, elbows, a solitary vessel or those affecting endothelium and it results in blood knees and heels. Occasionally, pressure multiple vessels (Table 2). vessel occlusion from thrombosis ulcers can also develop in the absence or from emboli (Figure 3). The of a bony prominence when there are Injury to the vascular endothelium majority of arterial ulcers arise from two solid surfaces that compress the leading to haemorrhagic skin necrosis peripheral arterial disease caused by tissues between them, as has been Injury to the vascular endothelium can atherosclerosis (Weitz, 1996). Moreover observed with nasogastric tubes, blood- also lead to haemorrhagic necrosis. atherosclerosis is also involved in the pressure monitoring devices or plaster Diseases in this group can present of arterial aneurysms casts (Devbhandari et al, 2006). either in a localised manner, such as which can rupture, thrombose and in popliteal artery aneurysms that cause emboli that can present with The pathophysiology of pressure cause necrosis in the forefoot, or be haemorrhagic skin necrosis. While the ulceration is relatively straightforward, more widespread such as in Henoch- pathogenesis of atheroma that gives but in addition to treating the wound, Schonlein . Occasionally rise to atherosclerosis is complex, management of patients with pressure the disease may be widespread as reversible risk factors associated with ulcers should address internal and in atherosclerosis, but then sudden atherosclerosis include hypertension, external exacerbating factors (Table 1) plaque rupture and thrombosis may a history of , hyperlipidaemia (Reddy et al, 2006). These same factors result in a solitary localised area of and diabetes mellitus (Grey et al, 2006). can affect the extent of tissue in skin necrosis. Thus, in addition to treating the area all forms of haemorrhagic skin necrosis associated with haemostasis and, to a lesser extent, haemorrhagic skin necrosis from other causes.

When the larger vessels are affected or when there is severe hypotension, haemostasis and therefore haemorrhagic skin necrosis may affect multiple sites, often in a Figure 2. A patient with sudden onset Raynaud’s symmetrical distribution. The sites Figure 1. A patient with systemic sclerosis and disease. Skin necrosis can be a presentation of affected will be in the areas of lowest associated Raynaud’s disease, complicated by underlying malignancy. In this case, the patient had . Pressure-bearing areas will painful skin necrosis and ulceration affecting the an adenocarcinoma which is the most common type be affected as well as the most distal left distal middle finger. of malignancy with this presentation.

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Table 3 Vasculitides associated with different types of infiltrate

Haemorrhagic skin necrosis with a rich vessel wall inflammation: Leucocytoclastic vasculitis Sweet’s syndrome Bowel associated dermatosis-arthritis syndrome Rheumatoid neutrophilic dermatosis Behcet’s syndrome Figure 5. A histological feature of leucocytoclastic Pyoderma gangrenosum vasculitis, in keeping with the clinical features Erythema elevatum diutinum Figure 4. Sudden onset of palpable purpura of the presented in figure 4, is based on the presence of lower legs can be a manifestation of small vessel transmural infiltration by and fibrinoid Haemorrhagic skin necrosis with a lymphocyte vasculitis, as in this case. The leucocytoclastic vessel necrosis (highlighted by the arrows). rich vessel wall inflammation: vasculitis was restricted to the skin and the Often as in this case there is fragmentation of Pyoderma gangrenosum patient subsequently recovered with conservative neutrophils (leucocytoclasis) and extravasation of Leukaemic vasculitis treatment. red cells. Polymorphic light eruption Pityriasis lichenoides forms of vasculitis of the skin, lesions immunoglobulin A in Henoch-Schonlein (usually purpura and haemorrhagic purpura or complement in systemic Haemorrhagic skin necrosis with a skin necrosis) tend to be widespread erythematosus (Carlson et al, granulomatous vessel wall inflammation: and symmetrically distributed, usually 2005). Once the type of vasculitis is Wegener’s granulomatosis on weight-bearing (dependent) sites diagnosed, a causal association may Lymphomatoid granulomatosis (Figure 4). become evident such as drug usage, Giant arteritis infection (as in ), Takayasu’s arteritis The skin lesions arising as a result co-existent inflammatory disease of the various causes of vasculitis (e.g. systemic lupus erythematosus or tend to be similar and so for further ) and likelihood characterization of the disease it is of malignancy. In addition to aiding necessary to do a skin biopsy for a diagnosis, interpretation of clinical histological identification. Skin biopsies findings and investigations is necessary need to be taken from the edge of a to determine the extent of the disease, new lesion to include unaffected skin in particular to establish if internal and in some instances the biopsy needs damage to vital organs has occurred to include deeper vessel (as in the case (Crowson et al, 2003). of , when the temporal artery needs to be biopsied). The Leucocytoclastic vasculitis is the Figure 3. A diabetic female smoker presented with pathologist is then able to confirm the most common histological pattern skin necrosis and a pulseless left foot. Subsequent presence of vessel wall inflammation and is characterised by transmural arterial dupplex confirmed severe atherosclerosis and the type of inflammatory infiltrate, neutrophil-rich inflammation and and occlusion of the posterior tibial artery. which in turn facilitates further associated fibrinod necrosis (Figure classification of the causes (Table 5). Often extravasated red cells, of haemorrhagic skin necrosis, such 3). Additional investigations may be neutrophilic debris (leucocytoclasis) patients need to be counselled and necessary to confirm a particular form and deposition of immunoreactants given therapy for these preventable of vasculitis, such as antineutrophil around the vessel wall are also evident. risk factors. cytoplasmic (ANCA) studies In keeping with other forms of vasculitis, for Wegner’s granulomatosis or while the disease may present with In contrast to the insidious nature microscopic polyangitis (Lagford, 2007; skin lesions, the same can of atherosclerosis, vascular endothelial Ozaki, 2007). also involve vessels supplying vital inflammation can also have an acute internal organs such as the kidneys, onset. Vascular endothelial inflammation Thus, histology is fundamental lungs, heart or brain. Leucocytoclastic that results in vasculitis can present when making a diagnosis of vasculitis. In vasculitis can be further divided into dramatically with widespread purpura addition, tissue can also be submitted two groups; those with and without and haemorrhagic skin necrosis for direct immunofluorescence to a definable precipitating cause. About (Carlson and Chen, 2006). In most identify immune deposits, such as half of all cases are caused by infection,

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inflammatory disease, drugs and malignancy (Carlson and Chen, 2006). Table 4 Classification of vasculitis based on size of vessel affected About 15–20% of all vasculitides result from underlying infection Small vessel Medium vessel Large vessel from such as B Wegener’s granulomatosis Classical polyarteritis Takayasu’s arteritis and C, bacteria such as group A nodosum beta-haemolytic streptococcus and Churg-Strauss syndrome Giant cell (temporal) arteritis Staphylococcus aureus, fungi or parasites (Carlson and Chen, 2006). Infection Microscopic polyangitis should be considered when there is Henoch-Schonlein purpura an antecedent history of high fever associated with signs of purulence. Essential cryoglobulinaemia Recognising infection as a cause of Cutaneous leukocytoclastic angiitis vasculitis is particularly important as the condition may resolve with alone, but also because causing haemorrhagic skin necrosis underlying infection can be exacerbated primarily affect small and medium- by the immunosuppressants commonly sized vessels (Jennette et al, 1994). used to treat severe vasculitis. The small vessel primary vasculitides Another 15–20% of individuals that are cutaneous small vessel vasculitis, develop vasculitis have an underlying urticarial vasculitis and Henoch- inflammatory disease, typically systemic Schonlein purpura. Only polyarteritis lupus erythematosus, Sjogren’s nodosum, of the medium-sized syndrome or inflammatory bowel vessels primary vasculitides causes disease. In such patients the underlying haemorrhagic skin necrosis (Piette, autoimmune disease tends to be 2001). In addition, there are vasculitides severe, with high antibody titres, and that cause disease of both small and results in extensive vasculitis affecting medium-sized vessels that cause skin both small and medium-sized vessels. necrosis; cryoglobulinaemia, Wegener’s Figure 6. Thrombosis of vessels (highlighted by the Management strategies should aim granulomatosis, Churg-Strauss arrows) in the in the presence of limited or to treat the underlying disease, syndrome and microscopic polyangitis. absent inflammation is a feature of skin necrosis vasculitis and provide support for Each of the primary vasculitides have caused by a prothrombotic state. Often, as in this affected organs. distinct clinical features and they differ case, the vessels appear dilated and filled with in presentation, organs affected and or undergoing re-canalisation as seen in Recurrent bouts of vasculitis treatment (Crowson et al, 2003). There the lower right vessel. occurring after taking certain drugs are many review articles (Langford, account for 10–15% of cases. Drug 2007; Ozaki, 2007) describing these groups commonly associated with diseases, but a thorough description is dermis (Figure 6) with relatively few leucocytoclastic vasculitis include the beyond the scope of this review. associated inflammatory cells. The penicillins, sulfonamides, thiazides and pattern of necrosis is often generalised, oral contraceptives. In addition to internal causes of symmetrical and is dependent upon endothelial damage, extrinsic factors the size of vessels that are occluded. Last, internal malignancies can also damage the microcirculation. In conditions such as necrosis, especially those involving expansion These include injury from , thrombosis begins by affecting small of B lymphocytes, such as , scalds, radiation and drug vessels but then progresses to involve lymphoproliferative disorders and extravasation (Carlson et al, 2005). much larger vessels which can result paraproteinaemias, can also cause in fatal thrombosis. Hence, there is an vasculitis. However, in 50% of cases an Hypercoagulability leading to haemorrhagic urgency to establish the diagnosis and underlying cause cannot be identified skin necrosis implement therapy. and the vasculitis may be part of a Haemorrhagic skin necrosis primary vasculitic syndrome which has frequently results from an alteration A working knowledge of the characteristic diagnostic features in blood constituents, leading to a cascade (Schenone (Piette, 2001). hypercoagulable or prothrombotic et al, 2004) and assistance from a state (Bick, 2006). The resultant skin haematologist are essential to establish The primary vasculitides are necrosis is often painful and potentially the diagnosis and begin therapy. The classified according to the size of fatal. Typically skin histology reveals coagulation cascade has two competing vessel affected (Table 4). Those thrombosed vessels throughout the components: the prothrombotic arm

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There are many acquired mostly affects women usually in their prothrombotic states, three of 50s or 60s, and typically involves the which require specific mention: breasts, hip, buttock, and thigh. The antiphospholipid syndrome (Figure use of results in a transient 8), warfarin-induced necrosis and decrease of sensitive thrombotic thrombocytopenia factors, including C, resulting (which when caused by in a temporary hypercoagulable heparin, is also called heparin state which then spontaneously necrosis). In contrast, paroxysmal corrects itself. Therefore in affected nocturnal haemoglobulinuria and patients warfarin should be continued Figure 7. This patient presented with 3 day myeloproliferative disorder, which are with advice and supervision by a malaise, followed by widespread cutaneous necrosis also acquired prothrombotic states, haematologist. affecting her face, arms and legs; with particular tend to predispose to deep vein involvement of the digits. Histology showed thrombosis and therefore to venous Heparin necrosis is rare and may widespread intravascular thrombosis and further leg ulceration. be caused by both unfractionated investigations revealed a heterozygous factor V and low molecular weight heparin Leiden mutation and complicating . Antiphospholipid syndrome (Figure 9) (Patel and Knight, 2005). It led to resolution of the initial infection, however is characterised by the presence is associated with the formation of her recovery was complicated by a further episode of autoantibodies against various against heparin that lead to of septicaemia from wound infection leading to negatively charged clumping. The continued use of her death. phospholipids, such as lupus heparin can lead to the formation of and anticardiolipin larger emboli that can occlude vessels consisting of clotting factors that lead antibodies. It remains to be established supplying both the skin and internal to the formation of and how these antibodies result in organs. Initially, skin necrosis occurs at fibrin the major components of a widespread thrombosis. Patients with the injection site, but can later involve thrombus, and the modulatory arm these antibodies display abnormalities distant areas. Continuation of heparin which is responsible for preventing clot in one or more of the following therapy aggravates the condition, with propagation (Schenone et al, 2004). coagulation tests: activated partial potentially fatal consequences, so it Either an excess of pro-coagulant thromboplastin time, kaolin clotting should be stopped immediately. factors or deficiencies in naturally- time, dilute or occurring can lead to dilute Russell viper test Certain can also cause a prothrombotic state, typically as a (Moll, 2006; Abo and Debari, 2007). , which can arise from result of genetic abnormality. These Antiphospholipid syndrome mostly a systemic cause as in disseminated conditions predispose to haemorrhagic affects women and can be associated intravascular coagulopathy associated skin necrosis (Simioni et al, 2006). with systemic lupus erythematosus. with septicaemia. This form of sudden To date mutations or deficiencies of Antiphospholipid syndrome may thrombosis and resultant haemorrhagic the following anti-coagulant factors present as a cause of multiple arterial skin necrosis is often widespread have been or may be associated with and venous thrombotic episodes, and develops rapidly, and it is known haemorrhagic skin necrosis: factor recurrent spontaneous abortions, and as . A number of V (Figure 7) (Patel et al, 2000) and with livedo reticularis. different bacterial infections that result prothrombin of the pro-cogulant in septicaemia have the potential to factors, as well as deficiencies in protein Antiphospholipid syndrome is also cause purpura fulminans, such as C, protein S, anti-thrombin III, heparin a cause of livedoid vasculopathy, a septicaemia from: group A streptococci, co-factor II and factor XII. Protein disorder characterised by painful meningococci, staphylococci, and C and S deficiency can also present ulceration in association with livedo pneumococci. Purpura fulminans is as purpura fulminans, in which skin reticularis and atrophie blanche. most common with menigococcal necrosis favours the extremities, such Livedoid vasculopathy has also septicaemia, arising in up to 20% of as digital skin necrosis. In addition, been described in association with cases (Betrosian et al, 2006). The homocystineuria is associated with factor V Leiden mutation (Patel et development of purpura fulminans a greater risk of thrombosis and al, 2000). This progressive, painful, with these infections is thought to possibly venous formation and debilitating disease requires result from the consumption and (Franchini, 2006). Usually, these genetic anticoagulation and drugs to treat the eventual depletion of endogenous anti- prothrombotic states only manifest underlying disease. coagulant factors, such as anti-thrombin with haemorrhagic skin necrosis when III, and S (Betrosian et al, activated by either severe trauma or is an uncommon 2006). Infection with these bacteria sepsis but can result in the condition transient phenomenon which occurs engenders a variety of inflammatory in the absence of a clear precipitant in at the start of warfarin treatment in and procoagulant host responses that older people. the absence of heparin. The condition interact to result in vascular and tissue

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There is evidence to suggest that respiratory compromise may require a localised consumptive coagulopathy ventilator support and potential is responsible for the haemorrhagic transfer of the patient to the intensive skin necrosis in necrotizing faciitis care unit. Acute surgery may also and clostridium-associated gas be necessary, for example, in the Figure 8. Anti-phospholipid syndrome can present (Bryant, 2003). However, management of sudden with explosive onset necrosis associated with while histology confirms the presence and occlusion of an important vessel. internal damage, which in this case of intravascular thrombosis, it also Therefore, while a diagnosis is being proved fatal. demonstrates the presence of a sepsis- formulated and thereafter, it will be associated vasculitis; as such the precise necessary to support vital organs and mechanism remains to be determined determine: (Wong and Wang, 2005). 8 The best location for the patient (consider intensive care) Management of haemorrhagic skin necrosis 8 Mattress requirement The most important aspect in the 8 Pain management management of a patient with 8 Fluid management and nutritional haemorrhagic skin necrosis is the care 8 Figure 9. This patient with a suspected deep vein establishment of a diagnosis. There Infection risk assessment and thrombosis was given subcutaneous low molecular are many clinical and investigational infection management 8 weight heparin for only 3 days, while awaiting clues that can narrow down the hunt Wound care strategy. a venogram that showed no venous thrombosis. for the cause. Based upon the original After 10 days she developed widespread cutaneous works of Rudolf Virchow it has been Consideration should be given necrosis associated with a mild reduction in platelet possible to categorise the causes of as to how the areas of skin necrosis count. She eventually required surgical haemorrhagic skin necrosis into three can be managed. Intuitively areas of followed by skin grafts. subgroups: haemostasis, vessel wall extensive necrosis should be treated inflammation and coagulopathy.The with surgical debridement, to promote injury, some of which are mediated by clinical presentations also differ subtly healing of the wound and to reduce bacterial . The host inflammatory between these categories (Table 5) so the risk of secondary infection. cytokines associated with sepsis based on the clinical presentation alone However, extensive debridement activate coagulation by stimulating it should be possible to narrow the may cause release of cytokines the release of thromboplastin, from focus of investigations. that may further exacerbate any and the endothelium. This difficulties with vital organ function. leads to the formation of thrombin Over and above investigations to Also surgical debridement in the and a fibrin clot. The thrombin in find the cause, in many instances of absence of a diagnosis can be harmful, turn stimulates multiple inflammatory haemorrhagic skin necrosis there is particularly in the case of vasculitis pathways and also activates the associated internal damage to vital or coagulopathy where upon it may fibrinolysis cascade. This explosive organs, and so tests are needed precipitate the development of further activation of both pro-coagulant to identify potential problems and lesions. Once surgical debridement of and anti-thrombotic cascades, called monitor function. In some cases it the lesions has been planned, another disseminated intravascular coagulopathy, will be apparent — based upon the consideration is whether to promote perpetuates the consumption of factors natural history of the disease — which healing by secondary intention or skin in the coagulation cascade, including organs are likely to be affected, for grafting; often the two procedures the endogenous anticoagulants. example, Wegner’s granulomatosis has are combined when the loss of tissue The preferential consumption a predilection to cause renal damage; results in deep wounds. of endogenous anticoagulants is while in the case of purpura fulminans responsible for the prothrombotic there may be multiple organs affected. The eventual treatment of the state that results in purpura fulminans. It is damage to the internal organs that disease will vary based upon the The end result of the host response will in the end determine the morbidity diagnosis, in some instances such as is diffuse endovascular injury, and mortality associated with the severe hypotension the diagnosis will microvascular thrombosis, organ haemorrhagic skin necrosis episode. As be reached quickly and the treatment , multiorgan dysfunction, and such, multiple clinical sub-specialities option will be clear. Indeed many of death in over 40% of cases (Gordon, may be involved to ensure the well- the disease entities mentioned have 2001). Activated protein C and anti- being of the patient; but it may fall upon specific treatment guidelines, take thrombin III can be efficacious the wound care specialist to coordinate for example the management of when administered to patients with and administer treatment. purpura fulminans where in addition purpura fulminans to reverse the to treatment with antibiotics and pathophysiology and facilitate recovery Renal involvement may necessitate anticoagulants, activated protein C and (Gordon, 2001). a renal biopsy and dialysis, while anti-thrombin III can be administered

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Haemorrhagic skin necrosis PRACTICE DEVELOP (painful, tender, haemorrhagic skin necrosis with surrounding hyperaemia) UK ,

2007,Vol Causes of acute vessel thrombosis

Haemostasis Vessel wall inflammation Hypercoagulability Multiple lesions, often Localised solitary lesionsMultiple, symmetrical Multiple, symmetrical Unilateral, regional, distalSymmetrically distributed Multiple lesions, often Multiple lesions, Clinical with a predilection for multiple lesions often over with a predilection for 3 presentation of peripheral lesions peripheral lesions lesions often with a MEN , No weight-bearing area necrosis fatty areas distal sites predilection for sites of trauma T 4 Typically affects distal Common sites of Often over weight- Typically affects distal Lower limbs, either When ambulatory lesionsTypically buttocks, Digits Arms and legs involvement bearing bony digits tips digits tips the area supplied by predominate over the lowerabdomen and breasts prominences, such as the dorsalis pedis or legs (females). sacrum or hip posterior tibial arteries

or both Typical Pressure ulceration Hypoperfusion Blood hyperviscosity Atherosclerosis Vasculitis Warfarin necrosis Factor V Leiden Purpura fulminans pathophysiology (e.g. polycythaemia mutation (Protein C rubra vera or multiple deficiency) myeloma) Claudication, cool skin Livedo reticularis, poor Often there is a history temperature distal to Initiation of warfarin Immobility Severe hypotension/ return and Systemic symptoms such as of trauma or infectionSepticaemia Preceding features involved vessel, loss of therapy shock, cool skin peripheral cyanosis malaise, lethargy and fever; hairs and poor capillary temperature, poor abrupt onset with lesions refill capillary return and appearing in waves peripheral cyanosis

Full blood count, renal Investigations: and , No Blood pressure,No blood No No Skin histology,Yes skin Yes Yes Yes Skin biopsy helpful C-reactive protein, cultures, full blood immunofluorescence, full immunoglobulins, count, renal and Blood pressure, bloodblood count, renal and liver Initial Sensory assessment, plasma and urine Skin histology, full blood count, renal and liver function tests, C-reactive liver function tests, cultures, full blood function tests, C-reactive investigations assessment for infection, electrophoresis, protein, immunoglobulins, protein electrophoresis, cryoglobulins, hepatitis x-ray for underlying C-reactive protein, count, renal and protein, immunoglobulins, B and C antibody titres, rheumatoid factor, kaolin clotting time, dilute bone , fullelectrocardiograph and liver function tests, pro-thrombin time, activated partial thromboplastin time, factor V blood count, renal andechocardiogram C-reactive protein, mutational analysis, complement levels, protein C levels, Protein S levels, liver function tests, C- electrocardiograph, anti-thrombin III levels and dilute Russell viper venom test echocardiogram, arterial reactive protein, thyroid hepatitis B and C antibody Doppler and possibly function test, , titres, anti-nuclear antibody, hepatitis B and C arteriogram transferrin, vitamin B12 extranuclear antibodies, antibody titres and and folate levels complement levels, bone marrow biopsy rheumatoid factor, anti- streptolysin O titre, blood cultures, convalescent viral titres, antineutrophilic cytoplasmic antibodies. Chest and sinus xrays, electrocardiogram and

31/10/07 4:20:31 pm throat swab Clinical PRACTICE DEVELOPMENT

resoultion of haemorrhagic skin Gordon R, Bernard et al (2001) necrosis. and safety of recombinant human Key Points activated protein C for severe sepsis. New Eng J Med 345: 699 Conclusions 8 Haemorrhagic skin necrosis is The diagnosis and management Grey JE, Harding KG, Enoch S (2006) painful and potentially fatal. of patients that present with Venous and arterial leg ulcers. Br Med J 332(7537): 347–50 haemorrhagic skin necrosis can be 8 Common causes of lower leg challenging. This article has highlighted Jennette J C, Falk R J, Andrassy K et some of the more common, as well al (1994) Nomenclature of systemic ulceration can present with vasculitides: The proposal of an haemorrhagic skin necrosis, as some rarer, causative diseases. It is international consensus conference. although occasionally it can be hoped that the strategy and approach Arthritis Rheum 37(2): 187–92 outlined provides a useful framework a manifestation of a serious Langford CA (2007) Small-vessel systemic disease. for the diagnosis of haemorrhagic vasculitis: therapeutic management. Curr skin necrosis, and helps to direct the Rheumatol Rep 9:328-35 practitioner in initiating appropriate 8 The management of Malone PC (2005) Further reflections on treatment. WUK haemorrhagic skin necrosis Virchow’s triad. South Med J 98(1): 125 is dependent upon a rapid Moll S (2006) — diagnosis. References practical implications and testing caveats. J Thromb Thrombolysis 21(1): 7–15 8 Haemorrhagic skin necrosis Abo SM, Debari VA (2007) Laboratory evaluation of the antiphospholipid Ozaki S (2007) ANCA-associated is associated with vessel syndrome. Ann Clin Lab Sci 37: 3–14 vasculitis: diagnostic and therapeutic thrombosis. strategy. Allergol Int 56: 87–96 Banerjee D, Jones V, Harding KG (2001) The overall clinical approach to chronic Patel GK, Morris E, Rashid MR, Anstey 8 Virchow’s triad is a framework wounds. In: Falanga V, ed. Cutaneous AV. (2000) Severe digital necrosis in an that is clinically useful to Wound Healing. Martin Dunitz Ltd, elderly patient with heterozygous factor consider the causes of vessel London: 165–85 v Leiden mutation. Br J Dermatol 143: 1302–5 thrombosis. Betrosian AP, Berlet T, Agarwal B (2006) Purpura fulminans in sepsis. Am J Med Sci Patel GK, Knight AG (2005) Generalised 332: 339–45 cutaneous necrosis: a complication of low molecular weight heparin. Int Wound J 2: to prevent excessive tissue thrombosis. Bick RL (2006) Hereditary and acquired 267–70 Occasionally these therapies may thrombophilic disorders. Clin Appl Thromb Hemost 12(2): 125–35 Patel GK, Grey JE, Harding KG (2006) even be counter-intuitive, such as Unusual causes of ulceration. Br Med J the continuation of warfarin therapy Bryant AE (2003) Biology and 332: 594–6 in warfarin-associated necrosis. It is pathogenesis of thrombosis and Piette WW (2001) Therapy of beyond the scope of this article to procoagulant activity in invasive infections caused by streptococci and clostridium leukocytoclastic (necrotising) cutaneous outline all the treatment options for perfingens. Clin Microbiol Rev 16: 451–62 vasculitis. Dermatol Ther 14: 95–101 the diseases mentioned and these are clearly described elsewhere. Ultimately, Carlson JA, Ng BT, Chen KR (2005) Reddy M, Gill SS, Rochon PA (2006) Cutaneous vasculitis update: diagnostic Preventing pressure ulcers: a systematic it is not be necessary for the wound criteria, classification, epidemiology, review. JAMA 296: 974–84 healing expert to be aware of all the etiology, pathogenesis, evaluation and Schenone M, Furie BC, Furie B (2004) therapeutic options, because often prognosis. Am J Dermatopathol 27: 504–28 The blood coagulation cascade. Curr Opin the care of such patients demands Carlson JA, Chen KR (2006) Cutaneous Hematol 11: 272–7 involvement from several different vasculitis update: small vessel neutrophilic Simioni P, Tormene D, Spiezia L, et al specialities. vasculitis syndromes. Am J Dermatopathol (2006) Inherited and 28: 486–506 venous thromboembolism. Semin Thromb Even after recovery, additional Carlson JA, Chen KR (2007) Cutaneous Hemost 32(7): 700–8 management may be necessary, for pseudovasculitis. Am J Dermatopathol 29: 44–55 Weitz J, Bryne J, Clagett G (1996) example, physiotherapy to prevent Diagnosis and treatment of arterial wound-associated . Also, Crowson AN, Mihm MC Jr, Magro CM insufficiency of the lower extremities: a compromised internal organs may (2003) Cutaneous vasculitis: a review. critical review. Circulation 94: 3026–49 require long-term support, such as J Cutan Pathol 30: 161–73 Whitney J, Phillips L, Aslam R, et al the need for renal dialysis. Some Devbhandari MP, Shariff Z, Duncan AJ (2006) Guidelines for the treatment of diseases can be associated with late (2006) Skin necrosis in a critically ill pressure ulcers. Wound Rep Regen 14: complications, for example, Henoch- patient due to a blood pressure cuff. J Post 663–79 Grad Med 52: 136–8 Schonlein purpura can result in renal Wong CH, Wang YS (2005) The diagnosis failure up to a year or more after Franchini M (2006) and of necrotising fasciitis. Curr Opin Infect the acute episode of vasculitis and aging. Crit Rev Oncol Hematol 60: 144–51 Dis 18: 101–6

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