Mixed arterial/ venous ulcers occur as a result of both venous and arterial peripheral disease and show clinical manifestations of both conditions. MIxed etiology ulcers are complex and can change very rapidly. In comparison with venous leg ulcers, mixed leg ulcers can be associated with lower health related quality of life, greater mobility impairments, and more deficits in self-care and usual activities.
Mixed arterial venous disease is estimated to affect up to 26% of patients with lower extremity ulcerations.
Patients with mixed etiology ulcers were found to typically be of older age, have lower body mass index, have a history of smoking, and more comorbid conditions than subjects with VLU.
Mixed etiology ulcers arise as a result of the presence venous disease in conjunction with a significant level of arterial disease. Chronic venous insufficiency results in venous hypertension (ambulatory venous pressures of up to 60 to 90 mmHg, as opposed to the normal levels of 20 to 30 mmHg), which can happen due to obstruction to venous flow or venous reflux from dysfunction of venous valves, and/or failure of the "venous pump". Inadequate tissue perfusion plays a major role in the pathogenesis of arterial ulcers.
A full assessment should be completed to determine the etiology of the ulcer and to identify any other disease processes and risk factors for ulceration and delayed wound healing. Assessment should include a comprehensive history of the patient’s current condition, recurrence, past medical and surgical history, medications, and other risk factors related to VLU, AU, PAD, and venous disease. For all patients with suspected chronic limb-threatening ischemia (CLTI)/PAD, guidelines recommend ankle brachial index (ABI) and ankle pressure (AP) and as the first-line screening tests to detect PAD. However, due to the high prevalence of arterial calcification among patients with AU, ischemia should also be documented by at least one of the following non-invasive arterial test modalities: toe pressure, toe brachial index, continuous doppler wave ultrasound, transcutaneous oximetry (TcPO2) or skin pressure perfusion. Venous disease should be documented with duplex ultrasound.
Note: Pitting edema may be present if ulcer is of mixed venous and arterial etiology.
Arterial ulcers, venous ulcers, neuropathic ulcers, pressure ulcers/injuries, metabolic (diabetes mellitus, gout, Gaucher disease, etc), hematologic (Sickle cell anemia, thalassemia, polycythemia vera, leucemia), autoimmune (Rheumatoid arthritis, leukocytoclastic vasculitis, polyarteritis nodosa), exogenous, neoplasia, infection, medication, skin disorders
In patients with mixed arterial and venous disease, revascularization is recommended in a staged fashion. Patients with an ABI > 0.7 should undergo wound care and compression therapy for venous disease, patients with an ABI < 0.7 should be considered for revascularization via an endovascular or open bypass procedure to enhance ulcer healing, and patients with an ABI < 0.5 may need earlier arterial intervention to allow for aggressive compression therapy. After arterial intervention, standard wound care and compression therapy remain crucial to wound healing and the prevention of ulcer recurrence.
Pyoderma gangrenosum is a rare but serious primary ulcerating condition of the skin that falls into the category of neutrophilic dermatoses. Pyoderma gangrenosum is often associated with systemic diseases including inflammatory bowel disease, arthritis, and myeloproliferative disorders.
Incidence: estimated to occur in 3-10 patients per million population per year
Prevalence: estimated to be of 0.01% among adult patients in the U.S
Current pyoderma gangrenosum pathogenic hypothesis is that the disease is autoimmune in nature with defects in cell-mediated immunity, neutrophil and monocyte function and humoral immunity. A patient’s genetic background can lead to aberrant activation of innate-immune complexes (inflammasomes). The activated immune system leads to increased levels of dermal cytokines and resultant neutrophilic tissue infiltration. Other inducers of pyoderma gangrenosum include drugs such as granulocyte colony stimulating factor, isotretinoin, propylthiouracil and sunitinib. Cocaine has also been implicated as a common agent to trigger pyoderma gangrenosum.
Pyoderma gangrenosum presents with several variations and is easy to misdiagnose. Diagnosis of pyoderma gangrenosum is based mainly on clinical findings because biopsies show no specific diagnostic features. however, a biopsy is indicated for all potential cases in order to exclude other conditions such as malignancy, infections, or cutaneous vasculitis.
add in diagnostic criteria from WR
Currently, there is no definitive guideline or gold standard in management of pyoderma gangrenosum, as data from controlled clinical trials are scarce.
Local wound care:
For simple/ limited pyoderma gangrenosum: use topical therapy, agents include:
For more severe cases, see above plus consider:
Sytemic therapy agents:
Targeted Therapy:
See section 'Coding' in topic "Pyoderma Gangrenosum - Introduction and Assessment"
Calciphylaxis is a rare but life-threatening calcific vasculopathy affecting the microvessels in the dermis and subcutaneous tissue, characterised by painful cutaneous ischemic lesions. It has an insidious disease course, and detailed medical history and identification of risk factors are essential for its clinical diagnosis. It can mimic many diseases, including warfarin- and heparin-induced skin necrosis, pyoderma gangrenosum, peripheral arterial disease, systemic lupus erythematosus, and antiphospholipid. Most cases of calciphylaxis are in patients with end-stage renal disease (ESRD), who require dialysis. However, it can also occur in other diseases with normal renal function, including autoimmune disease, polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome, cancer, and coronavirus disease 2019. Calciphylaxis not only involves the skin but also the heart, lungs, and other internal organs.
Incidence: rare
Diagnosis
The diagnosis of calciphylaxis is complex and requires careful clinical evaluation and pathological examination. Detailed medical history and identification of risk factors are crucial for its clinical diagnosis. In clinical practice, a biopsy of the deep skin and subcutaneous tissue is essential for the differential diagnosis of calciphylaxis. The histological manifestations of calciphylaxis include skin microvascular or extravascular calcification, thrombosis, and intimal fibrosis. However, routine skin biopsy is not recommended because the biopsy process can induce ulcer formation in the incision area and increase the risk of infection, sepsis, and death. When a biopsy is contraindicated or not diagnostic, radiography, bone scanning, and bone scintigraphy can identify microcalcification and extravascular calcification, which can help confirm the histopathologic results or monitor the treatment response.
Cryoglobulinemic vasculitis is caused by the formation of immunoglobins called cryoglobulins. At temperatures below regular body temperature, cryoglobulins can become solid or gel-like, and can restrict blood flow in the vessels. Cryoglobulinemia typically presents with vasculitis with lower extremity purpura, glomerulonephritis, and peripheral neuropathy.
Prevalence: fewer than 5 cases per 10 000 individuals in Europe and North America
The onset of cryoglobulinemic vasculitis is associated with circulating immunoglobulins that undergo reversible precipitation from plasma or serum upon cooling (below 98.6 F). Most patients diagnosed with type II or type III mixed essential cryoglobulinemia have the disease as an immune response to chronic hepatitis C infection. The cryoglobulins in these patients are enriched with anti–hepatitis C antibody and hepatitis C RNA.. The build up of the immunglobins iin the vessels can result in vascular occlusion and ischemic damage of tissues.
Testing for cryoglobulins is necessary for diagnosis, done via collection of venous blood in citrate, ethylenediamine tetraacetic acid, or oxalate. Keep at 37°C (98.6°F) until the specimen gets to the laboratory. In the laboratory, the sample is then cooled to 4°-5°C (39.2°-41°F) for detection of cryoprotein. Hepatitis panel should be ordered in suspected type II and III cryoglobulinemia. Biopsy may also be helpful.
For type I, the underlying hematologic disease should be treated directly. For mixed cryoglobulinemias, if patients positive for hepatitis C, they should receive antiviral therapy, If negative, other possible underlying infectious, autoimmune, or myeloproliferative diseases should be identified and treated. For severe vasculitis and end-organ involvement, immunosuppressive therapy including systemic steroids and/or rituximab may be used. Cytotoxic agents such as mycophenolate, azathioprine, or cyclophosphamide may be used as a secondary action.
D89.1 – Cryoglobulinemia
Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are both necrotizing vasculitides that typically affect small-sized arteries and vary in terms of organ manifestations and severity. The most commonly and severely affected organs include the upper and lower respiratory tract and the kidneys. Because of their strong association with antineutrophil cytoplasmic autoantibody (ANCA), they are also referred to as ANCA-associated vasculitis (AAV). Patients typically present with symptoms including fever, malaise, anorexia, weight loss, myalgias, and arthralgias.
Incidence: GPA: 0.4 to 11.9 cases per million person-years
MPA: 0.5 to 24.0 cases per million person-years
Prevalence: GPA: 2.3 to 146.0 cases per million persons
MPA: 9.0 to 94.0 cases per million persons
GPA Risk Factors:
MPA:
GPA and MPA are two types of ANCA-associated vasculitis, or AAV. AAV causes inflammation of the small blood vessels. The presence of ANCAs in the bloodstream cause neutrophils to stick and clump to the walls of small blood vessels in different tissues and organs of the body, leading to inflammation and narrowing and/or weakening of the vessels.
The diagnosis of GPA and MPA is based upon the combination of characteristic clinical findings, laboratory tests, and imaging studies.
Testing for ANCA should be performed in any adult patient who presents with symptoms suggestive of a vasculitis. In clinical practice, ANCA can be detected using an indirect immunofluorescence (IIF) assay or antigen-specific enzyme-linked immunosorbent assays (ELISAs) for proteinase 3 (PR3) and myeloperoxidase (MPO).
Whenever possible, the diagnosis of GPA or MPA should be confirmed by biopsy of a site of suspected active disease. Histologic examination of tissue obtained by biopsy of an affected organ (usually kidney, skin, or lung) remains the most definitive method to establish a diagnosis.
The overall management approach is the use of systemic glucocorticoids plus cyclophosphamide or rituximab. Induction of remission is followed by maintenance therapy using medications such as azathioprine, methotrexate, or rituximab.
See "2022 ICD-10-CM Diagnosis Code M31.7"
Polyarteris nodosa (PAN) is a multisystem disease with symptoms including fever, sweats, weight loss, and severe muscle and joint aches/pains. It can also cause skin abnormalities such as purpura, livedo reticularis, ulcers, nodules or gangrene, mostly located on the legs and can be very painful.
PAN is a necrotizing vasculitis of medium-sized and occasionally small arteries. The exact cause is unknown, but it likely involves immune complex deposition, autoantibodies, inflammatory mediators, and adhesion molecules.
A skin biopsy should be used to identify vasculitis in the small/medium vessels. PAN should be considered in a patient who presents with the following symptoms: fever, weight loss, myalgias, arthralgias, and signs of multiorgan involvement such as neurologic dysfunction, skin lesions, abdominal pain, renal insufficiency, and hypertension.
Provide local wound care to any skin ulcerations. Surgery may be required for the debridement of necrotic tissue or in the context of an acute abdomen. The use of graduated compression stockings may be helpful. Systemic corticosteroids may be used to treat mild disease. For severe systemic disease, methylprednisolone may be administered followed by prednisone.
Arterial or ischemic leg ulcers (AU) are leg ulcers that develop due to inadequate blood supply to the skin (arterial insufficiency). The decrease in blood supply may be caused by underlying peripheral arterial disease (PAD) that results from narrowing of the arteries to the legs (atherosclerosis), or may be caused by other non-atherosclerotic diseases. It is essential to differentiate AUs from venous leg ulcers (VLUs). Among chronic ulcers, AUs pose the largest financial burden to Medicare in the U.S. In addition to the financial burden, chronic limb-threatening ischemia (CLTI) patients have an increased risk of limb amputation.
Incidence: The monthly incidence rate of AU is estimated to be 1.8 cases per 100 patients with advanced illness
Prevalence: In the United States, the prevalence of AUs among Medicare beneficiaries in 2014 was estimated to be 0.4%.
Patients who are at increased risk of PAD:
Inadequate tissue perfusion plays a major role in the pathogenesis of AUs. AUs are often precipitated by trauma or infection. Limbs with arterial compromise may have minimal but adequate blood flow to maintain tissue viability. Trauma or infection increase demand for blood supply, leading to a non-healing ulcer
For all patients with suspected chronic limb-threatening ischemia (CLTI)/PAD, guidelines recommend ankle brachial index (ABI) and ankle pressure (AP) and as the first-line screening tests to detect PAD. However, due to the high prevalence of arterial calcification among patients with AU, ischemia should also be documented by at least one of the following non-invasive arterial test modalities: toe pressure, toe brachial index, continuous doppler wave ultrasound, transcutaneous oximetry (TcPO2) or skin pressure perfusion
Mixed ulcers (i.e. ulcer with mixed etiology, for instance, arterial/venous, arterial/neuropathic, etc), diabetic foot ulcer, venous ulcers, pressure ulcers/injuries, skin manifestations of COVID-19 (e.g. chilblains-like lesions, acute limb ischemia due to hypercoagulability)
Patients with healable AUs should undergo either prompt revascularization or a 4-week trial of conservative treatment followed by revascularization, depending on the WIfI classification. For foot infections involving wet gangrene, abscess, gas, or necrotizing fasciitis urgent surgical debridement is recommended. For non-healable/maintenance AUs, debridement is not recommended.
For all patients (healable, non-healable, maintenance), appropriate local wound care should be implemented to avoid infection and further injury. Interventions to improve circulation in AU patients include:
See section 'Coding' in topic "Arterial Ulcer - Introduction and Assessment"
A pressure ulcer (PU), also known as pressure injury (PI), pressure sore, decubitus ulcer or bed sore, is an area of localized injury to the skin and/or underlying tissue, usually over a bony prominence or related to a medical or other device. A pressure ulcer/injury (PU/PI) can present as intact skin and/or ulcer and may be painful. It occurs as a result of pressure, or pressure in combination with shear. PU/PIs represent a large burden to healthcare systems. It is estimated that PUs/PIs cost the U.S. between $9.1-11.6 billion a year. Despite optimal care, not all PUs/PIs are avoidable.
Incidence: Hospital discharge data from 210 academic medical centers in the USA show decreased incidence rates of PU/PI from 11.8/1000 cases in 2008 to 0.8/1000 cases in 2012.
Prevalence: Data from a sample of 918,621 patients in the USA showed that the overall prevalence of PU/PI at all facilities decreased from 13.5% (2006) to 9.3% (2015)
PUs/PIs develop as a result of an imbalance between an individual's tolerance to external mechanical loads and mechanical loads that exceed this tolerance. PUs/PIs occur over predictable pressure points where bony protuberances are more likely to compress tissues when the patient is in contact with hard surfaces. When the damage threshold of an individual is reached, a series of events occur and result in tissue damage. Tissue deformation may cause immediate muscle damage in susceptible areas/individuals. Cell death due to tissue deformation leads to local inflammatory reactions with edema and increased interstitial pressure. This additional tissue deformation leads to more cell distortion and cellular damage. External mechanical loads cause blood vessels to compress and nutrients cannot be delivered, leading to local hypoxia and tissue damage.
Diagnosis of PU/PI is clinical, based on information gathered during history and physical examination. Laboratory tests ordered upon initial assessment help establish a baseline and monitor any chronic underlying medical conditions, as well as the patient’s nutritional status, which may be factors that impair wound healing. Diagnostic tests can be considered when investigating non-healing ulcers and/or complications.
Stage 1:
Stage 2:
Stage 3 or stage 4:
Ensure adequate pressure redistribution on bed and seating surfaces: assess need for a new support surface to reduce the forces of pressure and shear against the patient's body. Select support surfaces according to individual needs/resources and care setting. Reposition patient and encourage mobility if not contraindicated.
Provide effective local wound care:
Surgical consult is indicated for full thickness ulcers that are extensive or refractory, if important structures are exposed (e.g., vessels, nerve, bone, muscle, fascia) or osteomyelitis is suspected.
Unstageable:
Deep tissue:
See section 'Coding' in topic "Pressure Ulcers/Injuries - Introduction and Assessment"
Infected arterial ulcers (AU) are leg ulcers that develop due to inadequate blood supply to the skin (arterial insufficiency) and become infected. The decrease in blood supply may be caused by underlying peripheral arterial disease (PAD) that results from narrowing of the arteries to the legs (atherosclerosis), or may be caused by other non-atherosclerotic diseases. It is essential to differentiate AUs from venous leg ulcers (VLUs). Among chronic ulcers, AUs pose the largest financial burden to Medicare in the U.S. In addition to the financial burden, chronic limb-threatening ischemia (CLTI) patients have an increased risk of limb amputation.
Infection of ischemic ulcers is a primary risk factor for major amputation.
Inadequate tissue perfusion plays a major role in the pathogenesis of AUs. AUs are often precipitated by trauma or infection. Limbs with arterial compromise may have minimal but adequate blood flow to maintain tissue viability. Trauma or infection increase demand for blood supply, leading to a non-healing ulcer.
By default, chronic wounds are contaminated by several types of bacteria. Bacterial biofilms are estimated to be present in ~78.2% of chronic wounds.
When the host (patient) does not adequately respond to bacterial contamination, this contamination can turn into colonization, which can further turn into infection.
Infection: The NERDS mnemonic (Nonhealing ulcer, increased Exudate, Red-friable tissue, Debris, Smell;) can help identify soft tissue infection. If any 3 NERDS are present, superficial soft tissue infection is likely and topical antimicrobial treatment is justified.
The STONEES mnemonic (Size, Temperature, Os (orifice), New breakdown, Exudate, Erythema + edema (cellulitis), Smell) can help identify systemic infection. ystemic antibiotics and topical antimicrobial treatment are justified if 3 or more of the STONEES signs are present.
Infected arterial ulcers have a higher risk of amputation, especially if neuroischemic ulcer. Evaluate need for hospital admission:
A pressure ulcer (PU), also known as pressure injury (PI), pressure sore, decubitus ulcer or bed sore, is an area of localized injury to the skin and/or underlying tissue, usually over a bony prominence or related to a medical or other device. A pressure ulcer/injury (PU/PI) can present as intact skin and/or ulcer and may be painful. It occurs as a result of pressure, or pressure in combination with shear. PU/PIs represent a large burden to healthcare systems.
Infected pressure ulcers are a common problem, occurring in 4 to 6 percent of nursing home patients
Patients with hip fractures
Risk Factors for Infection of PU/PI:
Tissue biopsy or quantitative validated swab technique for culture, obtained after debridement, can be used to diagnose wound infection.
Address infection and control bioburden: Interventions to manage PU/PI infection include debridement, topical antiseptics/antimicrobial agents, systemic antibiotics and surgical procedures.
Topic antimicrobials:
"It is important to treat the whole patient and not just the 'hole' in the patient."
Dressing selection is just a small part of wound care. Without a holistic, individualized patient assessment and plan that addresses the cause of the ulcer, patient concerns and local wound care, all clinicians’ efforts may not result in complete and timely healing. Thus, it is strongly recommended that dressings be selected after a holistic patient assessment. Assessment can be performed with WoundReference’s Assessment Tool and other tools.
As part of the assessment, clinicians should determine if the ulcer is:
The dressing type will change as the needs of the person and their wound change
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