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142 ATYPICAL CAUSES OF LEG ULCERATION
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excessive accumulation of calcium in the small blood vessels within
the skin and subcutaneous fatty tissue (Baby etal.2019), usually as
a consequence of problems with calcium and phosphorous
metabolism. The more common areas for calciphylaxis to develop
are those with larger amounts of adipose tissue, such as the lower
limb and abdomen (Isoherranen etal.2019). It can also be associated
with hyperparathyroidism (the parathyroid glands in the neck are
responsible for regulation of blood calcium levels) (Roncada
etal.2012).
Calciphylaxis is a life- threatening condition with a very poor
prognosis, even if detected in its early stages; the one- year mortality
rate has been calculated at between 45% and 80% (Bliss2002; Roncada
etal.2012) and it is closely associated with septicaemia.
History
An existing history of ESRF will be a key marker when assessing for
potential calciphylaxis. There is a documented link between the
development of calciphylaxis and the length of time for which
patients require dialysis, with the risk increasing after two years of
treatment (Nigwekar etal.2018).
Some of the risk factors associated with calciphylaxis include
hypertension, diabetes, obesity, certain medications including
warfarin, and coagulation disorders (Nigwekar et al. 2018).
Suggestions for why calciphylaxis is at higher risk of developing
alongside these factors include their link to chronic renal disease,
dialysis and a potential role of autoimmunity, although for many risk
factors the cause is unknown (Nigwekar etal.2018). It is also more
prevalent in females (Kuypers2009).
Examination
Calciphylaxis in the early stages is characterised by the presence of
dusky and mottled skin that is usually extremely painful (Figure3.11),
which can progress to necrotic lesions and nonulceration (Figure 3.12), often within a few days (Kodumudi
etal.2020; Nigwekar etal.2018). Areas of necrosis are irregular and
typically have inamed and undermining borders (Isoherranen
etal.2019).
healing areas of
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FIGURE3.11 Dusky, mottled skin seen in early stages of calciphylaxis.
Source: Ng and Peng2011 / John Wiley & Sons. Reproduced
withpermission.
FIGURE3.12 Necrotic lesion from calciphylaxis.
Source: Dissemond etal.2018 / John Wiley & Sons. Reproduced
withpermission.
Investigations
The investigations include blood tests to identify high levels of
calcium and phosphate and parathyroid hormone abnormalities.
An X- ray and bone scan can also be useful adjuncts to detect
calcication.
144 ATYPICAL CAUSES OF LEG ULCERATION
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A skin biopsy may be performed to rule out other conditions that
can present similarly, although there is some debate over whether
additional skin trauma may worsen the progression of the disease
(Chang2019).
Diagnosis
Diagnosis of calciphylaxis can be challenging due to the other
conditions that can present with similar symptoms, such as cellulitis,
vasculitis and warfarin- induced skin necrosis (Nigwekar etal.2018).
Calciphylaxis also shares common clinical patterns with Martorell’s
ulcers (Isoherranen etal.2019). Key indicators present in the medical
history, such as ESRF, need to be considered; blood tests and X- ray
results can often be inconclusive (Chang2019). A multidisciplinary
approach to diagnosis can be useful to reach an agreement due to the
multiple dierential diagnoses for the condition and the complexities
associated with co- morbidities.
Investigation results, such as high calcium and phosphate blood
levels, may support the diagnosis, as evidence of clear calcication
seen on the X- ray. If a biopsy is taken, usual ndings include calcication of vessel walls, thrombosis and infarction (Oakley2016).
Intervention
Treatment for calciphylaxis begins with the management of any known
risk factors, such as hypertension, diabetes and associated medications
(Isoherranen etal.2019). Patients may also be treated with medication
such as intravenous sodium thiosulfate during their dialysis sessions,
and dialysis regimes may become more aggressive to ensure that calcium and phosphate levels are managed (Nigwekar etal.2018).
Smaller ulcers are usually managed conservatively or with minor
debridement, but larger areas may be treated with skin grafts
(Isoherranen etal.2019). Antibiotics are often required since the risk
of developing a wound infection increases as necrosis progresses.
Patients typically experience acute ischaemic pain with
calciphylaxis, due to the damage to tissues from ischaemia and
infarction as small vessels are occluded. Some patients also experience
neuropathic pain with this condition. The pain can vary from patient
to patient, such as a constant background pain that is exacerbated on
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movement or during procedures, or breakthrough pain that is
unprovoked (Chinnadurai etal.2020). First- line treatment for pain
usually involves opioid- based analgesia, with paracetamol and antiseizure drugs, such as gabapentin, used as adjuvants and for
managing nerve pain. Consideration needs to be given to impaired
renal function in those patients with calciphylaxis associated with
renal failure and the potential for medication toxicity. With the level
of pain usually experienced by patients with calciphylaxis, input
from a pain management specialist is recommended.
The primary management aim is the removal of necrotic tissue,
following a specialist and multidisciplinary review. Dietitian advice
should be sought to support optimisation of nutritional needs
(helpful for promoting wound healing) and the control of calcium
and phosphorous intake by limiting certain foods in the diet
(Hess 2002). Local wound care measures, exudate and odour
management and decontamination using local antimicrobial
cleansers and treatments should form part of the wound management
plan. The use and tolerance of compression therapy will be dependent
on the presence of oedema and the need to control exudate.
MALIGNANT/NEOPLASTIC WOUNDS
As with other unusual aetiologies, lower limb wounds that are
unresponsive to standard treatments should raise suspicion of an
atypical cause, which can include malignancy/skin cancer. There is
also the potential that the wound has undergone a malignant
transformation from an otherwise benign aetiology. Clinicians
managing leg ulcers need to be familiar with the normal characteristics
of common leg ulcer aetiologies to be able to identify abnormalities
such as malignancy. Primary skin cancers that ulcerate when located
on the leg are often misdiagnosed as chronic leg ulcers (Senet2014).
Neoplastic or malignant ulcers can be classied as primary skin
cancers or as metastatic secondary skin cancers. Primary cancers
result from the direct extension of a tumour to the skin surface,
initially presenting as an inamed and indurated area with pain,
tenderness and sometimes an ‘orange peel’ appearance. This area can
go on to ulcerate and proliferate as the tumour grows, becoming
commonly referred to as fungating wounds.
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Metastatic tumours occur when detached cells from the initial
site travel to other organs, including the skin (Rayner etal. 2009).
Between 5% and 15% of patients with metastatic cancer will develop
a fungating wound (Stringer etal.2014).
Research suggests that around 2–4% of leg ulcers are found to be
malignant (Misciali etal.2013), although one study indicated a prevalence as high as 10.4% (Senet etal. 2012). Their appearance can
range from completely innocent- looking lesions typical of a chronic
leg ulcer to more overt growths. When mimicking a chronic leg ulcer,
the malignancy will often present as a single area of ulceration with
indurated or pigmented edges (Senet 2014). It is suggested that
abnormal excessive granulation tissue, particularly at the wound
edges, can be highly indicative of a malignant leg ulcer, as can abnormal bleeding (Senet etal.2012). It should be remembered that not all
malignant lesions will ulcerate, so anything atypical in appearance
should be investigated further– early diagnosis can reduce the risk of
metastases developing and of a simpler and potentially less complex
and disguring treatment (Hayes and Dodds 2003). Also, it should
not be assumed that multiple ulcers to the same limb are of the same
aetiology.
Basal Cell Carcinoma, Squamous Cell Carcinoma, Malignant
Melanoma andMarjolin’s Ulcers
The most common types of primary skin cancers that occur on the
lower limb are basal cell carcinoma (BCC), SCC and malignant melanoma (MM) (Table3.6). BCCs and SCCs are sometimes referred to as
‘non- melanoma skin cancers’ or keratinocyte carcinomas as they
arise from the keratinocyte cells in the skin (Karimkhani etal.2015),
whereas melanomas arise from melanocytes.
BCCs are sometimes referred to as ‘rodent ulcers’, a traditional
term coined because the ulcerated area may look like a tiny rodent
bite on the skin. Some SCCs may be referred to as Bowen’s disease, or
squamous cell carcinoma in situ, indicating a precursor to SCC before
the development of any invasive malignancy (Shimizu et al. 2011).
These typically present as multiple, supercial, scaly, irregular
plaques most commonly to the legs, and can be treated with a variety
of options following a diagnostic biopsy, including excision,
cryotherapy and photodynamic therapy (Tillman2004).
TABLE3.6 Key risk factors andpresentation ofbasal cell carcinoma (BCC), squamous cell carcinoma (SCC)
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and malignant melanoma (MM).
BCC SCC MM
Risk factors/history All of these skin cancers are more common in people with pale skin and light hair (blond/red)
and light eye colour and in areas of sun- exposed skin
Twice as common in men than women No sex- associated risk globally
Intermittent intense
sun exposure
Cumulative pattern of
sun exposure
Smoking (Diepgen and
Maher2002)
Varying patterns of sun exposure
High number of moles on the
skin– 80% arise as new moles
rather than changes in an
existing mole
Family or personal history
of melanoma
(Continued)
TABLE3.6 (Continued)
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BCC SCC MM
Typical
presentation/
examination
Development Localised, slow growing
Source: Adapted from Freak (2005); Rayner etal. (2009); Senet (2014). Photos from Price etal. (2022) / John Wiley & Sons.
Reproduced with permission.
Tend to be supercial
Can be cystic, nodular or
keratotic (crusting)
Start as a red, dome-
shaped nodule with
visible capillaries that
may be pigmented
Can develop necrosis and
ulceration with a rolled
edge as they grow
If ulcerated– often have
well- dened borders
with a ‘pearly’ appearance to the wound edge
over years, minimally
invasive and rarely
metastasise
Firm, nodular,
crusted lesions
Can ulcerate as they grow
and have a propensity to
develop secondary
infections
Often have rolled and/or
raised edges and
hyper- granulation tissue
in the wound bed
Locally invasive, grow
noticeably in months
and with potential to
metastasise
Pigmented lesion/mole that has
changed in shape, size and/or
colour– can have uneven pigmentation, irregular borders and be
slightly raised from the skin surface
Most commonly presents on the legs
in women and trunk/back in men
Some types are associated with nail
beds and the soles of feet and are
more common with Asian skin
Can become amelanotic (non-
pigmented)– skin- coloured, pink,
red or purple
Some types are associated with
spontaneous bleeding, and if
ulcerate often have darkly pigmented borders and peri-
Aggressive development with high
risk of metastasising, particularly if
diagnosis is delayed
ulcer skin
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Other types of malignant leg ulcers or lesions are Marjolin’s
ulcers (which usually evolve from SCCs), Kaposi’s sarcoma and
cutaneous presentations of lymphoma that can result in leg ulcers
(Senet2014). Classic Kaposi’s sarcoma, which is unrelated to human
immunodeciency virus (HIV), usually presents as multiple dark
blue macules (at, distinct, discoloured areas of skin) that progress to
larger plaques and tumours/lesions (Senet2014). They are usually
conned to the lower limb and frequently appear on both legs.
Marjolin’s ulcers are malignant transformations that occur over
many years in chronic wounds of another aetiology, including chronic
leg ulcers. They were named after the French Physicist Jean-
Nicolas
Marjolin, who rst described them in 1827 (Senet,2014). Marjolin’s
ulcers are very rare, estimated to occur in 1.7% of chronic wounds,
and typically consisting of SCCs, although other types of skin cancers
have been identied more rarely (Trent and Kirsner 2003). These
ulcers have been shown to be more aggressive and metastasise more
often than if a new SCC was to develop directly on the skin
(Senet2014). Marjolin’s ulcers are often overlooked or misdiagnosed,
leading to a poor prognosis and mortality rate of around 21% (Saaiq
and Ashraf 2014). Their underlying cause is largely unknown,
although suggestions include occurrence due to constantly dividing
skin cells trying to resurface a chronic wound (Menendez and
Warriner 2006) and chronic inammation from chronic venous
insuciency (CVI) as contributing factors (Isoherranen etal.2019).
History
The incidence of primary skin cancer can relate to age, with rates
rising signicantly in the over 50s and peaking in the over 75s,
although a quarter of melanomas are diagnosed in the under 50 age
group (Jones etal.2020). Over 60 is also the age group when venous
insuciency and/or PAD are most frequent, hence the potential for
misdiagnosis. Table3.3 outlines some of the key risk factors to look
for in a patient’s history that can be indicative of BCC, SCC or MM,
of which sun exposure is common to all.
Marjolin’s ulcers can have a very long latent time over which
they transform, usually over 10 years and often much longer
(Combemale etal.2007). The long duration of a pre- existing ulcer is
one of the key factors for diagnosing this type of malignant leg
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150 ATYPICAL CAUSES OF LEG ULCERATION
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wound. They are also associated with sites of previously healed
burns, traumatic injury and osteomyelitis (Menendez and
Warriner2006). Suspicion should be raised if the patient is reporting
sudden or unexpected changes in a longnew pain, foul- smelling discharge, increased volume of exudate or
change in nodular appearance to the wound bed (Choa etal.2015).
Examination
Ulcers on the calf are unusual when of vascular origin or diabetes
related, so the location of a wound in this area is worth investigating
for an atypical cause, usually either malignancy, infection or vasculitis
(White1999).
The presence of apparent granulation tissue that is nodular (like
a cauliower), raised, budding, exuberant, translucent, shiny or
rolling over the wound edge should raise suspicion of malignancy
(Harris etal.1993; Poccia etal.2014; Tchanque- Fossuo etal.2018)
(Figures3.13 and3.14). Other key signs suggestive of malignancy
include (Harris etal.1993; Poccia etal.2014):
standing wound, such as
FIGURE3.13 Basal cell carcinoma presenting as a leg ulcer– note the
raised granulation tissue with raised borders. The ulcer was unresponsive to
standard treatment.
Source: Misciali etal. (2013) / John Wiley & Sons. Reproduced with permission.
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Atypical Causes of Leg Ulceration 151
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FIGURE3.14 Amelanotic malignant melanoma disguised as a diabetic
foot ulcer– note exuberant granulation tissue.
Source: Gregson and Allain (2004) / John Wiley & Sons. Reproduced with
permission.
Islands of epithelium (new skin produced within the wound bed)
that form but do not continue to produce healed tissue and often
break down again.
Firm indurated surrounding skin unrelated to venous
skin changes.
Unusual pain or bleeding.
Necrosis may be evident as the malignancy progresses, and large
volumes of exudate are associated with the invasion of the lymphatic
system by malignant cells (Adderley and Holt2014).
Marjolin’s ulcers may not always exhibit classical malignant
changes such as an abnormal wound edge, raised crusty growths or
bleeding, but are more likely to have an irregular wound bed and
hyper- granulation (Choa etal.2015) (Figure3.15). The key sign for
Marjolin’s ulcers along with their long duration is an increase in size
despite appropriate treatment.
A clinical knowledge summary from the National Institute for
Health and Care Excellence (NICE2022a) on melanoma recommends
that suspicious skin lesions should be assessed using a weighted
seven- point checklist (Table 3.7) (Mackie 1990), with any lesion
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