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122 ATYPICAL CAUSES OF LEG ULCERATION
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of associated vascular disease and the results of a vascular assessment.
Ulcer appearance and location can be indicative of the underlying
inammatory disorder as described earlier, and of the potential
impact of pressure and friction. All of these factors can be combined
with the results of a skin biopsy if one is performed to make a
diagnosis.
Intervention
A multidisciplinary approach involving the rheumatologist is
important to successfully manage rheumatoid ulcers. Due to the link
between the systemic treatments for RA and wound healing, advice
from the rheumatologist with regard to the therapies being used to
manage RA is paramount. Reduction of the dose of immunosuppressant
medication or temporary discontinuation until wound healing has
been achieved may be advocated in some cases (Bootan 2013), or
suggestions around switching to a dierent type of DMARD or antiTNF treatment may be made. Such discussions will assist in balancing
control of the underlying RA, prevention of disease progression and
minimising the impact of RA symptoms on a patient’s quality of life
with maximising the wound healing potential of associated ulceration.
Podiatrist and/or orthotist input can also be useful to manage
and ooad any foot deformities contributing to existing ulceration,
and also for prevention of further wounds. Input from a physiotherapist and occupational therapist can help with improving mobility,
functional activity and education on protecting the joints (Firth2011).
Management of the wound bed and exudate levels using
appropriate primary and secondary dressings is important– gentle
autolytic debridement of sloughy tissue is recommended as opposed
to less conservative methods to reduce the risk of infection in
immunocompromised patients. Good skincare using cleansing and
emollients will help to maintain skin integrity, particularly if the skin
is thin and fragile as a consequence of corticosteroid use. This should
also be a consideration when choosing appropriate dressings. If using
an adhesive dressing, gentle silicone dressings are advised, and a
silicone medical adhesive remover can also be useful to reduce the
risk of skin trauma and any dressingTopical antimicrobial dressings may be required if the wound is
displaying any signs of local infection.
related pain (Young 2019).
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Compression therapy following vascular assessment is a useful
adjunct when managing rheumatoid ulcers to address any venous
insuciency and dependent oedema once signicant arterial disease
has been ruled out. While peripheral neuropathy is not an absolute
contraindication to compression therapy, clinicians should be
mindful of the loss of protective sensation experienced by these
patients. Patients may not detect any pain or discomfort associated
with compression therapy that is too tight or that may have
inadvertently caused trauma to the skin from pressure damage
(Dissemond et al. 2016). It is recommended that compression
therapy instigated in patients with peripheral neuropathy is reviewed
more frequently for signs of complications from compression,
particularly in the early stages after commencing treatment, and a
reduced level of compression therapy (see Chapter 8) may be
indicated where there are concerns over the patient’s ability to detect
increasing pain or complications (Australian Wound Management
Association and New Zealand Wound Care Society 2011;
Todhunter 2019). Compression bandaging may be the most
appropriate option for therapy in view of the likelihood of reduced
dexterity and limb deformity making the application of compression
hosiery or adjustable compression wraps dicult. Also, the
requirement for adequate padding over bony prominences, nodules
and fragile areas susceptible to high pressure may be more easily
achieved with the use of subcompression bandages.
bandage wadding underneath
Systemic Scleroderma
Systemic scleroderma or sclerosis is a rare autoimmune disorder that
results in damage to connective tissue to the skin and internal organs,
and functional and structural abnormalities of small blood vessels
(Volkmann et al. 2023). Scleroderma is a long- term condition for
which there is no cure. It is associated with life- threatening conditions and has a high mortality rate (Tate et al.2019). Scleroderma
occurs due to an overproduction of collagen that is deposited in the
skin, blood vessels and internal organs, resulting in scarring and
brosis in aected areas (Rayner etal.2009). Immune dysfunction
and microvascular abnormalities are also common features (Tate
etal.2019).
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There are two main clinical forms of systemic scleroderma –
diuse, which primarily aects the upper arms, thighs and trunk;
and limited, which involves the lower arms and legs (Rayner
etal.2009).
History
Patients with scleroderma may present with a known history of
systemic scleroderma, thus making the association with leg ulceration
and a subsequent diagnosis of the underlying aetiology a more
obvious consideration for the assessing clinician. Scleroderma can be
dicult to diagnose, particularly in the earlier stages of the disease,
so patients may present with cutaneous symptoms and wounds
before a formal diagnosis of the disease has been made (Volkmann
et al. 2023). Skin changes become more apparent as the disease
progresses over time, with skin thickening of the ngers being the
primary diagnostic criterion. Patients may present with overlapping
symptoms of other autoimmune diseases, such as RA and Sjögren
syndrome, which can co- exist with systemic scleroderma (Volkmann
etal.2023). Positive family history is also the strongest identied risk
factor for systemic scleroderma (Arnett etal.2001).
Raynaud’s phenomenon is commonly associated with scleroderma
and is often the rst reported symptom along with swelling and
inammation of the hands and feet (Volkmann etal.2023). Patients
may present with the typical symptoms of Raynaud’s: pain, numbness,
white skin and pins and needles to the ngers and toes caused by
reduced blood ow from vasoconstriction. Raynaud’s may be quite
severe when it occurs alongside scleroderma, with ulceration
occurring to the digits in up to 50% of cases (Khimdas etal.2011).
Patients may report some diculties with swallowing because of
scleroderma and consequently have a reduced nutritional intake,
which can aect wound healing (Tate etal.2019). Malnutrition, particularly protein deciency, adversely aects the formation of new
blood vessels and the synthesis of collagen, which are essential for
rebuilding the extracellular matrix during the proliferative phase of
wound healing (Ghaly etal.2021). Micronutrients (e.g. amino acids)
and key vitamins also play important roles in the inammatory phase
for collagen synthesis. Wound healing requires signicant amounts
of energy that would normally be provided by the body’s natural
Atypical Causes of Leg Ulceration 125
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energy stores, but these are signicantly depleted when nutritional
intake is poor (Ghaly etal.2021).
Examination
Known skin- related symptoms of systemic sclerosis are thickened
skin, nger ulcerations, joint contractures, skin pigmentation
changes and itching (Hudson etal.2009; Tate etal. 2019). Patients
may also present with dry, scaly skin (Figure3.5).
Calcium deposits may be visible or palpable within the wound bed
(Figure3.6)– calcinosis cutis, a deposition of insoluble calcium salts in
the skin and/or subcutaneous tissues that can occur in damaged,
inamed, neoplastic or necrotic skin (Ngan2005), is a common feature
with systemic sclerosis (Tate et al.2019), and ulceration can occur
secondary to the calcinosis in some places. Calcium deposits can
contribute to a prolonged inammatory response within the wound as
they are perceived as a foreign body; thus they can delay healing and
increase the risk of infection. They can also cause pain and strongly
adhere to the wound bed, making removal dicult.
Ulcers related to scleroderma are often very painful due to the
ischaemia caused by Raynaud’s, and in more severe cases gangrene
may be evident.
FIGURE3.5 Ulceration associated with systemic scleroderma with visible
skin brosis.
Source: Used with permission from Cardi & Vale University Health Board.
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FIGURE3.6 Calcium deposits in the wound bed related to calcinosis cutis
and scleroderma.
Source: Chave etal. (2001) / John Wiley & Sons. Reproduced with
permission.
Investigation
If systemic scleroderma is suspected but not yet diagnosed, blood
tests to detect the presence of specic autoantibodies can aid in the
diagnostic process, as can skin biopsy. The involvement of the heart,
lungs, kidneys and gastrointestinal system would also need to be
assessed (Tate etal.2019). Referral to a specialist team for diagnosis
and management of both scleroderma and any associated ulceration
is recommended (Tate etal.2019).
Vascular assessment is advised to identify any concomitant
venous and/or arterial disease in the lower limb, with appropriate
onward referral to vascular for intervention as indicated. An Xmay be ordered if calcinosis is present to check the extent of calcium
deposition in and around the wound.
ray
Diagnosis
Diagnosis of systemic scleroderma itself is usually based on the classication criteria stipulated by the American College of Rheumatology and European League Against Rheumatism collaborative
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(Table3.5; van den Hoogen et al.2013). Early identication is key
and referral to appropriate specialists will aid in timely diagnosis and
management.
Diagnosis of leg ulcers associated with scleroderma will primarily
be based on the medical history and patient examination. Skin biopsy
can indicate the blocking of the small vessels and the presence of
inammatory mediators (Shanmugam etal.2010).
TABLE3.5
Against Rheumatism collaborative criteria for classication of systemic
sclerosis
Item Sub- item(s) Weight/score
Skin thickening of the ngers
of both hands extending
proximal to the
metacarpophalangeal joints
(sucient criterion)
Skin thickening of the ngers
(only count the higher score)
Fingertip lesions (only count the
higher score)
Telangiectasia —
Abnormal nailfold capillaries — 2
Pulmonary arterial hyperten-
sion and/or interstitial
lung disease
(maximum score is 2)
Raynaud’s phenomenon — 3
The American College of Rheumatology and European League
a
.
— 9
Puy ngers;
Sclerodactyly of the
ngers (distal to
the metacarpophalangeal joints
but proximal to
the proximal
interphalangeal
joints)
Digital tip ulcers 2
Fingertip pitting scars 3
2
Pulmonary arterial
hypertension
Interstitial
lung disease
2
4
2
2
b
(Continued)
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TABLE3.5
(Continued)
Item Sub- item(s) Weight/score
SSc- related autoantibodies
(anticentromere, anti–
topoisomerase I [anti–Scl- 70],
anti–RNA polymerase III)
Anticentromere
Anti–topoisomerase I
Anti–RNA pol-
ymerase III
3
(maximum score is 3)
a
These criteria are applicable to any patient considered for inclusion in a systemic
sclerosis study. The criteria are not applicable to patients with skin thickening
sparing the ngers or to patients who have a scleroderma- like disorder that better
explains that manifestation (e.g. nephrogenic sclerosing brosis, generalised
morphea, eosinophilic fasciitis, scleredema diabeticorum, scleromyxedema,
erythromyalgia, porphyria, lichen sclerosis, graft- versus- host disease, diabetic
cheiroarthrophy).
b
The total score is determined by adding the maximum weight (score) in each
category. Patients with a total score of ≥9 are classied as having denite systemic
sclerosis. SSc, systemic sclerosis.
Source: van den Hoogen etal. (2013) / BMJ Publishing Group. Reproduced by
permission.
Implementation
Pharmacological management of systemic scleroderma usually
involves some form of immunosuppressant agent, such as rituximab
or cyclophosphamide, which can exert anti- inammatory and/or
anti- brotic properties to manage the underlying brosis and
vasculopathy at a cellular level (Volkmann etal.2023).
As well as the potential for these types of drugs to aect the
wound healing process, they can diminish the immune response to
the extent that there is a higher risk of opportunistic infection,
particularly if malnutrition is also involved (Volkmann etal.2023).
Calcinosis in the wound bed also increases the risk of infection. Overt
and covert signs of local and spreading wound infection should be
closely observed and treated appropriately (see Chapter5). Patients
should also undergo regular nutritional screening using validated
tools as per local policy, and a referral made to a dietician for
nutritional support as recommended by local pathways.
b
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Treatment of leg ulcers related to scleroderma focuses on the
management of symptoms and prevention of deterioration. Pain
management is an important part of symptom control due to the
severe pain that can be experienced both from the scleroderma
generally and from the scleroderma- related leg ulceration, which can
aect the patient’s quality of life. Appropriate pain assessment should
be conducted and documented and local pain management algorithms
followed; referral should be made to pain management specialists as
required (see Chapter6). Pain from the reduced blood ow associated
with the Raynaud’s component of the disease is often treated with
systemic vasodilators, such as sildenal or nifedipine, or prostacyclins
(e.g. iloprost), alongside traditional analgesia such as paracetamol and
NSAIDs. Other recommendations for preventing and managing
Raynaud’s attacks include avoiding the cold where possible; wearing
warm mittens and hand and foot warmers can be useful (Pope2022).
Treating Raynaud’s phenomenon is also important from a calcinosis
perspective, as underlying ischaemia is known to worsen calcinosis,
which tends to form in areas of local trauma (Pope2022).
Skincare is another key component of the management of skin
thickening from scleroderma and for maintaining the integrity of periwound skin. Patients are also prone to pruritic rashes, which can be
uncomfortable and bothersome. Regular use of skin emollients to
promote adequately moisturised skin is essential; joint guidelines
from the British Society of Rheumatology (BSR) and British Health
Professionals in Rheumatology (BHPR) recommend the use of lanolinbased moisturisers specically (Denton et al.2016). Antihistamines
are useful to help reduce skin itch alongside moisturisation; other
practical recommendations include limiting time in hot baths and
showers and avoiding harsh soaps and fragranced moisturisers
(Volkmann etal.2023).
In terms of local wound care, competent practitioners may
choose to remove supercial and loose pieces of calcium where
possible and as tolerated by the patient; this in essence removes the
perceived foreign body to promote healing and reduce infection risk.
Surgical intervention is usually only considered if the calcinosis is
severe and aects the patient’s ability to function and their quality of
life (Tate etal.2019). Dressing choice should be guided by the TIMES
assessment of the wound environment, with consideration given to
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minimising wound pain at dressing change and managing infection.
Exudate management can be supported through the use of
compression, often at a reduced dose unless accompanied by
signicant venous disease.
Sickle Cell Disease
Sickle cell disease (SCD) is an umbrella term for various genotypes
that all present with sickle- shaped blood cells. It is an inherited
haematological disorder where a mutation in the ᵝb globin genes in
the bone marrow produces red blood cells with defective haemoglobin
HbS. Where both parents carry the gene there is a one in four chance
of a child having the disease. The HbS produces blood dyscrasia
aecting multiple organs and related systems, with sickle cell
anaemia occurring when there are two abnormal HbSS genes from
two parents. Variants present where genes from one parent combine
with another haemoglobin variant as with thalassaemia, which
reduces the concentration of haemoglobin. Sickle cell anaemia and
thalassaemia variant are the most severe forms of SCD. Sickle cell
trait occurs where there are genes from one parent only, resulting in
the child being a carrier of the disease but asymptomatic.
The genetic presence of the disease has been linked to areas
where malaria occurs, commonly in Africa and among people of
African origin, but also in the Caribbean, South and Central America,
the Eastern Mediterranean and Asia. The highest prevalence is found
in populations from sub- Saharan Africa. In the United Kingdom
SCD aects 15 000 people with almost 300 babies born each year.
Children with SCD are at an increased risk of stroke, the highest risk
being between 2 and 16 years of age (www.sicklecellsociety.org). The
median UK survival is 66/67 years (DeBaun et al.2019; Gardner
etal.2016).
History
The sickle shape is caused by long rigid polymer chains forming
within the red blood cell once it has become deoxygenated. This
distinguishes it from normal red blood cells and has multiple eects.
The sickle cell has a shorter lifespan (normal 90–120days; sickle
10–20days) and is less able to travel through the circulation due to its
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shape. It is dehydrated, rigid and sticky with a weakened cell
membrane, which along with its shape makes it vulnerable to
adhesion and aggregation. As a result, it is easily trapped in smaller
vessels leading to impaired blood ow preventing oxygen delivery to
the tissues, ischaemia and tissue death. This produces an acute crisis
with chronic damage. Narrowing and occlusion of larger vessels
produce chronic sheer damage and adherence of red blood cells to
the endothelium alongside vasoconstriction and nitric oxide
deciency. This leads to pulmonary hypertension and stroke.
Examination
The severity of the disease varies from mild to severe, including being
life limiting. Dierent areas of the body may be aected, and the
consequences include lung and cardiopulmonary disease, central
nervous system complications including infarcts, retinopathy and
blindness, renal disease and hip necrosis. Symptoms therefore vary
between individuals, with chronic anaemia and severe intermittent
pain being the most common clinical manifestations. Chronic daily
pain has been found to increase with age, aecting 30–40% of adults
and adolescents (Brandow and Liem2022). Painful acute episodes
are triggered by the inammatory process and when the red blood
cell changes shape after oxygen has been released. A sickle cell crisis
is severely painful and occurs in one isolated site or multiple locations
including pelvis, ribs, spine and sternum. Chronic pain develops
with the sensitisation of the central and/or peripheral nervous
system. It may be diuse and have neuropathic elements (see
Chapter7).
Sickle cell leg ulceration is one of the clinical presentations of
this disease and is associated with low haemoglobin concentrations
combined with a high rate of intravascular haemolysis (Koshy
etal.1989). Minniti and Kato (2016) imaged, measured and examined
the eect of SCD and found that ulcerated sites contained a high
blood ow with evidence of chronic inammation, cutaneous
vasodilation, venostasis and thrombosis, and that leg ulcers were an
end-
organ complication.
Minniti and Kato (2016) outline three dierent presentations of
ulceration: the one- time ulcer, the stuttering ulcer that comes and
goes, and the chronic recurrent disabling ulcer. Common sites of
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