Plantar fascial fibromatosis, also known as Ledderhose's disease, Morbus
Ledderhose, and plantar fibromatosis, is a relatively uncommon non-malignant
thickening of the feet's deep connective tissue, or fascia. In the beginning,
where nodules or cords start growing along tendons of the foot, the disease
is minor. Eventually, however, the cords thicken, the toes stiffen and bend, and
walking becomes painful. The disease is named after Dr. Georg Ledderhose, a
German surgeon who described the condition for the first time in 1894. A
similar disease is Dupuytren's disease, which affects the hand and causes bent
hand or fingers. As in most forms of fibromatosis, it is
usually benign and its onset varies with each patient. The nodules are typically
slow growing and most often found in the central and medial portions of the
plantar fascia. Occasionally, the nodules may lie dormant for months to
years only to begin rapid and unexpected growth. Options for intervention include
radiation therapy, cryosurgery, Xiaflex surgical removal only if discomfort
hinders walking. Symptoms
Plantar fibromatosis is most frequently present on the medial border of the
sole, near the highest point of the arch. The lump is usually painless and
the only pain experienced is when the nodule rubs on the shoe or floor. The
overlying skin is freely movable, and contracture of the toes does not occur
in the initial stages. The typical appearance of plantar
fibromatosis on Magnetic resonance imaging is a poorly defined,
infiltrative mass in the aponeurosis next to the plantar muscles.
Only 25% of patients show symptoms on both feet. The disease may also
infiltrate the dermis or very rarely the flexor tendon sheath
Risk factors The histological and ultrastructural
features of Ledderhose and Dupuytren's disease are the same, which supports the
hypothesis that they have a common etiology and pathogenesis. As with
Dupuytren's disease, the root cause(s) of Ledderhose's disease are not yet
understood. It has been noted that it is an inherited disease and of variable
occurrence within families, i.e. the genes necessary for it may remain
dormant for a generation or more and then surface in an individual, or be
present in multiple individuals in the same generation with varying degree.
There are certain identified risk factors. The disease is more commonly
associated with - A family history of the disease
Higher incidence in males Palmar fibromatosis 10-65% of the time.
Peyronie's disease Epilepsy patients
Patients of diabetes mellitus There is also a suspected, although
unproven, link between incidence and alcoholism, smoking, liver diseases,
thyroid problems, and stressful work involving the feet.
Treatment Although the origin of the disease is
unknown, there is speculation that it is an aggressive healing response to small
tears in the plantar fascia, almost as if the fascia over-repairs itself
following an injury. There is also some evidence that it might be genetic.
In the early stages, when the nodule is single and/or smaller, it is recommended
to avoid direct pressure to the nodule(s). Soft inner soles on footwear
and padding may be helpful. MRI and sonogram are effective in
showing the extent of the lesion, but cannot reveal the tissue composition.
Even then, recognition of the imaging characteristics of plantar fibromatoses
can help in the clinical diagnosis. Surgery of Ledderhose's disease is
difficult because tendons, nerves, and muscles are located very closely to each
other. Additionally, feet have to carry heavy load, and surgery might have
unpleasant side effects. If surgery is performed, the biopsy is predominantly
cellular and frequently misdiagnosed as fibrosarcoma. Since the diseased area is
not encapsulated, clinical margins are difficult to define. As such, portions
of the diseased tissue may be left in the foot after surgery. Inadequate
excision is the leading cause of recurrence.
Radiotherapy has been shown to reduce the size of the nodules and reduce the
pain associated with them. It is approximately 80% effective, and the
side-effects are very minor. Post-surgical radiation treatment may
decrease recurrence. There has also been variable success in preventing
recurrence by administering gadolinium. Skin grafts have been shown to control
recurrence of the disease. In few cases shock waves also have been
reported to at least reduce pain and enable walking again. Currently in the
process of FDA approval is the injection of collagenase. Recently successful
treatment of Ledderhose with cryosurgery has been reported.
Cortisone injections, such as Triamcinolone, and clobetasol ointments
have been shown to stall the progression of the disease temporarily, although the
results are subjective and large-scale studies far from complete. Injections of
superoxide dismutase have proven to be unsuccessful in curing the disease
while radiotherapy has been used successfully on Ledderhose nodules.
See also Fibromatosis
Dupuytren's contracture Plantar fasciitis
Calcaneal fat pad atrophy List of cutaneous conditions
References External links
International Dupuytren Society Website Describes treatments for Ledderhose's
disease. Peer-reviewed write-up of the disease
and associated conditions Wheeless' textbook of orthopaedics:
Ledderhose disease