Introduction
Ledderhose disease (LD), also known as plantar fibromatosis, is a rare hyperproliferative disorder of the plantar aponeurosis, meaning that cell proliferation occurs much faster in cells present in the thick connective tissue that protects the underlying vital structures of the foot.1 This rare disease was first described by doctor Georg Ledderhose and is a benign condition that has been linked to Dupuytren disease, also known as palmar fibromatosis, which is a disease that consists of the abnormal thickening of the fascia (a layer of tissue found under the skin) on the palms of your hands and at the base of the fingers.1
The nodules that form due to Ledderhose disease on the plantar fascia can harden and become an indurated mass.1 In patients with LD, the plantar aponeurosis (the normal connective tissue found at the bottom of the feet) is replaced by fibrous tissue.2 Additionally, the nodes found in the central medial plantar fascia can cause problems for the rest of the plantar fascia as it could potentially lead to sclerosis of the whole plantar fascia.3 Similar to Dupuytren's disease, fibromatosis formation can be affected by the increased production of certain cell growth factors (as these would lead to hyperproliferation) and therefore can lead to toe contractures in the plantar fascia which are painful.4
Causes and risk factors
Developing LD may be due to repeated trauma, long-term alcohol consumption, chronic liver disease, diabetes, and epilepsy.5,6 The exact cause remains unknown, although hereditary factors have been considered a common cause.1
Signs and symptoms
Small, hard nodules develop slowly on the bottom of your feet (late-stage symptoms)
Other symptoms include:
- Joint pains
- Lack of skin elasticity
- Tingling of the feet
- Swelling and pain in the feet
- Walking disability
- Local pressure and distension (early stage)
Risk factors
People assigned male at birth are more affected than people assigned female at birth. The onset is very rare in children, and more common in adults. Caucasians are the ethnic group most affected by LD.
Diagnosis
Ultrasonography
An ultrasound can be used in order to diagnose Ledderhose disease. If ultrasound results show hypoechoic and homogeneous nodules present in the plantar fascia (tissue at the bottom of the foot) that have increased colour Doppler, these could suggest Ledderhose disease. This technique is more accessible to patients because it is easier to find and less costly.3
Computer tomography
Computer tomography (CT) scans can be used to image parts of the body using X-rays and can be used in order to confirm results presented by an ultrasound. In a CT scan, the bones, soft tissues, and blood vessels can be observed and therefore, could be used to see any potential issues with the bones since an MRI will be better to observe the extent of the tumor and determine whether excision surgery will be helpful.
Magnetic Resonance Imaging (MRI)
An MRI is used to image a part of the body using radio waves. This scan can be used to corroborate the results from an ultrasound. It will show images of proton density in the target area. The image shows hyperintense nodules on the plantar fascia by displaying a darker area of mass gathered at the plantar fascia.6 An MRI scan can therefore be used in order to observe the extent of LD by showing nodules that are contiguous with the plantar fascia as well as being effective for LD diagnosis. By showing the extent of the tumor/nodules, an excision of these nodules can be organized with a sufficient margin to prevent the recurrence of the benign tumor.3
Treatments
Treatments can include non-surgical treatments as well as surgery. Non-surgical treatment examples include intralesional cortisone injections, stretching, orthotics, nonsteroidal antirheumatic drugs, and physiotherapy, which should be performed when clinical symptoms begin to show.1,6 The treatments for LD are all aimed toward reducing the pain as the nodules cannot be cured completely yet.
Home treatment
In order to reduce tension in the foot caused by the nodules, stretching exercises can be done. An example of an effective stretch is the arch stretch. In order to conduct this stretch:
- Cross the affected foot across the opposite knee
- Grab your big toe with your thumb and index finger
- Pull the toe back gently towards your shin until you feel a gentle stretch in the arch, hold the stretch for 30 seconds and then relax
- Repeat 3 times.
Radiation therapy
Low-dose radiotherapy can provide pain relief and prevent the progression of LD. Radiation can be used in order to target the nodules; these lumps can be softened and shrunk which prevents the development of contractures. Radiotherapy can also be used post-surgery in order to prevent the recurrence of LD.7
Steroid injections
In order to treat LD non-surgically, steroid injections can be used. Steroid hormones can be injected into the nodules in the plantar fascia in order to limit the growth of the nodules and also limit the pain.3 Intralesional steroid injections may represent an alternative to surgery in patients with plantar fibromatosis or Dupuytren's contractures as some case studies have shown an improvement in the nodules and symptoms in patients with LD after 3 or 4 months of the treatment.8
Surgery
When the clinical treatments have been tried but the pain persists and the nodules continue to grow and there is a danger of toe contractures, in other words, when the other treatments have been ineffective, excision surgery can be done. The excision surgery can be conducted in a variety of ways; local excision, wide excision, and complete plantar fasciectomy are some of the options.4 A wide excisional approach or subtotal plantar fasciectomy seem to be the most effective treatment in preventing the recurrence of LD and consist of creating an excision on the side of the heel, and having the surgeon detach the ligament from the bone as this will relieve the inflammation of the plantar fascia.3
Summary
As with many diseases, a great advantage for LD patients, in order for treatment to be successful, is an early and correct diagnosis of the disease. LD can be confused with other diseases due to the mass on the sole of the foot being a common symptom in other diseases such as plantar fasciitis (the most frequent lesion of swelling of the plantar fascia), leiomyoma, rhabdomyosarcoma, neurofibroma, and liposarcoma.3 Using other diagnostic tools such as MRI or ultrasound can confirm the diagnosis and create a higher probability for treatment to be effective.3 Surgery and radiation therapy can be used in the advanced stages of the disease in order to treat the nodules, remove the benign tumor and then prevent the recurrence.
References
- Fausto de Souza D, Micaelo L, Cuzzi T, Ramos-E-Silva M. Ledderhose disease: an unusual presentation. The Journal of clinical and aesthetic dermatology. 2010 Sep;3(9):45.
- Thygarajan U, Raj DG, Susruthan M. Ledderhose disease: pathophysiology diagnosis and management. Journal of Orthopaedic Case Reports. 2019;9(2):84.
- Omor Y, Dhaene B, Grijseels S, Alard S. Ledderhose disease: clinical, radiological (ultrasound and MRI), and anatomopathological findings. Case Reports in Orthopedics. 2015 Sep 6;2015.
- Motolese A, Mola F, Cherubino M, Giaccone M, Pellegatta I, Valdatta L. Squamous cell carcinoma and ledderhose disease: a case report. The International Journal of Lower Extremity Wounds. 2013 Dec;12(4):297-300.
- Van Der Bauwhede J. Ledderhose disease: plantar fibromatosis. Wheeless’ textbook of orthopaedics. 2007.
- Elhadd TA, Ghosh S, Malik MI, Collier A. Plantar fibromatosis and Dupuytren's disease: an association to remember in patients with diabetes. Diabetic Medicine. 2007 Nov 1;24(11):1305.
- Connelly TJ. Development of Peyronie’s and Dupuytren’s diseases in an individual after single episodes of trauma: a case report and review of the literature. Journal of the American Academy of Dermatology. 1999 Jul 1;41(1):106-8.
- Pentland AP, Anderson TF. Plantar fibromatosis responds to intralesional steroids. Journal of the American Academy of Dermatology. 1985 Jan 1;12(1 Pt 2):212-4.
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