Plantar Fibromatosis

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Search Timeline: Februari, 2013 - April, 2013

Definition/Description

Plantar fibromatosis, also known as the Ledderhose disease or Morbus Ledderhose, is a (mostly) small slow-growing thickening of the superficial fibromatoses wich is actually a soft tissue tumor of the plantar aponeurosis similar in appearance to the disease which occurs in the palm of the hand (disease of Dupuytren). Basically it can be described as a benign fibroblastic proliferative disorder in which fibrous nodules may develop in the plantar aponeurosis, more specifically on the medial plantar side of the foot arch and on the fore foot region. The symptoms are swelling, pain is not usual and also a contraction is not applicable in the first stage. [4],[6],[9],[10]

Clinical Relevant Anatomy

The plantar fascia, or aponeurosis is synonymous with the deep fascia of the sole of the foot. The plantar fascia is a strong connective tissue structure that consists of pearly white longitudinally organized fibers. It begins at the medial tuberosity of the calcaneus where it is thinner and extends into a thicker center portion. This thicker portion is flanked by thinner lateral and medial portions. The thicker central portion of the plantar fascia then extends, into five different bands surrounding the digital tendons, to the plantar plates of the metatarsophalangeal joints and the bases of the proximal phalanges. [1], [9], [13]

Epidemiology/ etiology

Ledderhose’s disease, is named after a German surgeon, Dr. Georg Ledderhose. He described the condition first in 1894 as an uncommon hyperproliferative plantar aponeurosis [2],[6]

Ledderhose’s disease is listed as a “rare disease” by the Office of Rare Diseas-es (ORD) of the National Institutes of Health (NIH), which means that it affects less than 200,000 people in the United State’s population. [2]

Plantar Fibromatosis occurs less frequently than the palmar disease, with a prevalence of 0.23% and usually more frequently in middle aged male individuals (30 – 50 years). So men are affected twice as often as females and incidence increases with advancing age. Bilateral involvement is seen in 25 % of patiënts.[5],[10]

Due to the lack of information about the formation of this condition, the etiology is still controversial. But the plantar disease seems to have a multifactorial etiology, for example diabetis mellitus, genetic and traumatic causes (like a puncture wound or a micro-tear), family history and cancer incidence. [5], [10], [12]

Patients with the contracture of Dupuytren, diabetes mellitus, epilepsy, alcoholics with liver disease, stressfull work and keloids have more risk to develop the disease of Ledderhose and/or a the disease of Peyronie's. [5],[6], [9],[10]

Characteristics/Clinical Presentation

There will be a visible bulge, a soft- tissue mass composed of one or more subcutaneous nodules, on the medial (60%) or central (40%) plantar area of the foot same as a reduced capacity of bending the foot. The nodules may be multiple in 33% of cases and are typically slow growing. [2],[4], [7], [8], [10]
Not all of the patients do have symptomatic complains. Complains such as pain can occur after standing or walking for a long time, or when those nodules happen to grow and stiffen the affected structures of the foot (due to a lack of space) such as neurovascular bundles, muscles or tendons. Nevertheless this disease typically do not cause symptoms such as contractures and patients do frequently have normal radiographs.[5], [10]

Plantar fibromatosis is thought to have three phases:

1) Proliferative phase: with nodular fibroblastic proliferation
2) Active phase: with collagen synthesis and deposition
3) Mature phase: with reduced fibroblastic activity and collagen maturation [9]

Differential Diagnosis

Ledderhose’s disease is sometimes associated with other forms of fibromatosis, such as:
• Dupuytren’s disease
• Peyronie's disease
• knuckle pads [2]

Some others main differential diagnoses are:
• plantar fasciitis
• chronic rupture of the plantar fascia [9]

Diagnostic Procedures

The identification of characteristics of plantar fibromatosis on imaging can give several important information for the clinical diagnosis.
Cross sectional imaging (ultrasonography, compute tomography, MRI) reveals the lesions location, extension and involvement of neighboring structures.
Evaluation is therefor most commonly performed with ultrasound and MRI. Sonographic imaging demonstrates a well-defined (64%) or poorly defined (36%) fusiform mass in the soft tissues adjacent to the plantar aponeurosis.
Plantar fibroma may be heterogeneous and hypoechoic (76%) or isoechoic (24%) relative to the plantar fascia . Posterior acoustic enhancement (20%), cystic components, and intratumoral hypervascularity (8%) have also been described. [5], [9],[10], [11]