eMedicine Specialties > Dermatology > Benign Neoplasms

Plantar Fibromatosis

Author: Firas G Hougeir, MD, Staff Physician, Department of Dermatology, Mayo Clinic Scottsdale
Coauthor(s): Jose M Mascaro, MD, MS, Chairman, Professor, Department of Dermatology, Hospital Clinic, University of Barcelona, Spain
Contributor Information and Disclosures

Updated: Jun 30, 2009

Introduction

Background

The fibromatoses represents a wide spectrum of locally infiltrative clinicopathologic processes characterized by the proliferation of generally mature fibroblasts associated with mature collagen. Some of these entities are present at birth or develop in early childhood (eg, juvenile fibromatosis [JF]). Others may appear in adulthood.¹

The term plantar fibromatosis (PF) is used for different conditions, as follows: (1) a relatively common plantar equivalent of Dupuytren palmar contracture named Ledderhose disease (LD); (2) a more uncommon plantar superficial fibromatosis that, unlike deep fibromatosis (eg, abdominal, extra-abdominal, and visceral fibromatosis) generally has a less aggressive and recurrent tendency; and (3) an extremely rare, benign cerebriform mesodermal hamartomatous proliferation that, in a plantar location, appears to be a clinicopathologic marker of Proteus syndrome (PS).

Juvenile aponeurotic fibroma (JAF) and aggressive infantile fibromatosis (AIF) can also be considered to be in the plantar fibromatosis group when lesions are present on the sole of the foot.

Pathophysiology

Plantar fibromatosis represents not a single entity, but rather, a heterogeneous group of conditions with the common characteristics of plantar location and histologic features of mature collagen and fibroblasts with no malignant cytologic features.

In Ledderhose disease (described in 1897), as in Dupuytren contracture (DC) (first reported in 1831), repeated trauma, long-term alcohol consumption, chronic liver disease, diabetes, and epilepsy have been reported in association with the development of the lesions in middle-aged or elderly people. Often, patients with Ledderhose disease also have other fibrosing conditions such as Dupuytren contracture, knuckle pads, or induratio penis plastica (ie, Peyronie disease, first reported in 1743 by François de la Peyronie, physician of Louis XV of France). Heredity is also a clear factor in many patients.

Superficial fibromatosis (SF) in a plantar location includes a variety of soft-tissue tumoral proliferations of fibroblasts. However, it has been shown that some forms are not due to fibroblast overgrowth but to myofibroblast proliferation; superficial fibromatosis is more common in children and young adults than in older people.

Cerebriform mesodermic hamartomas on the soles represent a kind of mesodermal nevus and are usually associated with Proteus syndrome. This syndrome was named after the Greek god Proteus, the "Old Man of the Sea" and son of Poseidon who was able to change his shape to protect himself. Proteus syndrome is a complex malformative or asymmetric hypertrophic syndrome associated with multiple cutaneous and musculoskeletal manifestations such as epidermal verrucous nevus, vascular hamartomas, and exophytic cerebriform fibrolipomata and scoliosis, kyphosis, and exostosis, respectively.² Hamartomatous cerebriform plantar fibromatosis may develop on the soles before other manifestations of Proteus syndrome appear, and it is considered a marker for Proteus syndrome.

Fibromas and desmoid tumors (eg, intestinal polyps, osteomas, soft-tissue tumors, epidermal cysts) are common in Gardner syndrome, which was described in 1950. These tumors often arise over previous surgical scars. By means of direct DNA sequencing, recent studies show that somatic beta-catenin or adenomatous polyposis coli (APC) gene mutations are present in virtually 100% of cases of Gardner syndrome–associated fibromatosis (GAF), as well as most cases of deep fibromatosis (DF). On the other hand, no somatic mutations were identified in beta-catenin or APC genes in superficial fibromatosis. Therefore, the divergent behaviors of superficial fibromatosis in relation to DF and GAF, despite their similar clinical and histologic morphologic features, are based on genetic differences.³

Frequency

United States

Ledderhose disease is relatively common, and plantar contracture develops in approximately 25% of middle-aged or elderly individuals (1 of every 4 with Dupuytren contracture). Superficial plantar fibromatosis (SPF) is uncommon, and the hamartomatous form associated with Proteus syndrome is rare. The exact incidences of superficial plantar fibromatosis and the hamartomatous form associated with Proteus syndrome are unknown.

Mortality/Morbidity

• The different varieties of plantar fibromatosis may be asymptomatic. However, the feeling of a mass in the foot, difficulty fitting in shoes, and pain with weight bearing often affect patients' ability to stand or walk.
• Only aggressive infantile fibromatosis has an invasive course, as does fibrosarcoma; however, it does not metastasize.
• Recurrent forms are not uncommon, although plantar fasciectomy has a relatively reduced rate of recurrence in Ledderhose disease.

Race

• Whites are affected more often than other groups.

Sex

• Ledderhose disease affects men approximately 10 times more often than it affects women.
• Juvenile aponeurotic fibroma is more common in boys than in girls.
• No sex predilection is evident for the other forms of plantar fibromatosis.

Age

• Ledderhose disease is seen in middle-aged and elderly people.
• Superficial plantar fibromatosis and juvenile aponeurotic fibroma are most common in children and youths than in adults.
• The exceptional aggressive infantile fibromatosis begins in an infant's first year of life. The rare hamartomatous variety also develops in infants.

Clinical

History

• Patients with Ledderhose disease are not often aware of their disease because it is usually not painful.
• Likewise, patients with other forms of plantar fibromatosis may not be aware of their disease; however, they may notice difficulty in standing, walking, or wearing shoes when nodules or bumps become big enough.
• Ledderhose disease is typically bilateral and progresses slowly but not indefinitely.
• Superficial plantar fibromatosis may grow gradually, and, in most cases, self-involution occurs. In some cases, superficial plantar fibromatosis lesions enlarge and persist; if excised, they recur iteratively.
• Juvenile aponeurotic fibroma can spontaneously regress or persist. Recurrences after excision are common.

Physical

• Ledderhose disease consists of one or more small, asymptomatic, round or flattened, hard nodules that are generally located on the medial side of the sole. Flexion deformities usually do not occur in opposition to Dupuytren contracture.
• Superficial plantar fibromatosis appears as one or more asymptomatic, bad limited, flat nodules of fibrous consistency and variable size. They are most commonly located on the plantar aspect of the anteromedial portion of the heel pad (see Media File 1).
  
  

  

  Superficial fibromatosis of the heel.

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  Superficial fibromatosis of the heel.

• Juvenile aponeurotic fibroma may appear as a localized form affecting adults or a diffuse variety observed in children. It is more common in males than in females, and the hard nodules grow slowly and adhere to deep structures (see Media File 2).
  
  

  

  Juvenile aponeurotic fibroma.

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  Juvenile aponeurotic fibroma.

• Aggressive infantile fibromatosis is rare and ordinarily begins in the patient's first year of life. It grows rapidly and infiltrates the subcutaneous tissue, aponeurosis, and muscles with an expansive or infiltrative course like a fibrosarcoma. However, metastasis does not occur.
• Hamartomatous plantar fibromatosis lesions look like raised cerebriform soft-to-firm exophytic masses on any plantar area, where they are covered by pink, lightly dark, or normal-colored skin (see Media File 3). They can become large enough to cause disability.
  
  

  

  Hamartomatous fibromatosis.

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  Hamartomatous fibromatosis.

• In patients with Ledderhose disease, the presence of other fibrosing conditions (eg, Dupuytren contracture, knuckle pads, Peyronie disease in men) must be checked.
• In superficial plantar fibromatosis, Gardner syndrome must be ruled out.
• In the presence of cerebriform fibrous exophytic plantar lesions, Proteus syndrome must be considered.

Causes

As with many tumors, the causes are not known.

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