Clinical PRACTICE DEVELOPMENT Clinical PRACTICE DEVELOPMENT How to diagnose and treat haemorrhagic skin necrosis 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 wound care professionals. Yet such patients do present to wound healing clinics and wounds 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 cause upon the identification of a disease that of these symptoms may be a clinically causes vessel thrombosis. 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 Vasculitis 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 blood flow he vast majority of wounds of single or multiple blood vessels (haemostasis) presenting to a wound healing supplying the skin. The compromised 8 Injury to the vascular endothelium 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 tissue be attributed to either venous disease necrosis. The subsequent deoxygenation Diseases 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, diabetes, 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 surgery) (Banerjee et al, 2001; Patel cytokines 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 hyperaemia 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 scar. 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 40 Wounds UK, 2007, Vol 3, No 4 Wounds UK, 2007, Vol 3, No 4 41 40-54Patel.indd 40 31/10/07 4:20:20 pm Clinical PRACTICE DEVELOPMENT Clinical PRACTICE DEVELOPMENT Table 2 Table 1 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 infection 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 Atherosclerosis 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 pathogenesis 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 purpura. 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 smoking, 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 death 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 perfusion. 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. 42 Wounds UK, 2007, Vol 3, No 4 Wounds UK, 2007, Vol 3, No 4 43 40-54Patel.indd 42 31/10/07 4:20:21 pm Clinical PRACTICE DEVELOPMENT Clinical PRACTICE DEVELOPMENT Table 3 Vasculitides associated with different types of infiltrate Haemorrhagic skin necrosis with a neutrophil 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 neutrophils 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