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Plantar Fibromatosis

  • Author: Firas G Hougeir, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 03, 2016
 

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 Lederhose 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.

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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 Lederhose 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 Lederhose 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 deep fibromatosis and Gardner syndrome–associated fibromatosis, despite their similar clinical and histologic morphologic features, are based on genetic differences.[3]

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Epidemiology

Frequency

Lederhose 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.

Race

Whites are affected more often than other groups.

Sex

Lederhose 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

Lederhose 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.

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Prognosis

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.

Lederhose disease has a favorable prognosis, although slow progression is not uncommon. Patients who undergo plantar fasciectomy have been shown to have a lower recurrence rate.

Superficial plantar fibromatosis is usually benign and may regress spontaneously. Rare cases that are relatively progressive and recurrent occur. The hamartomatous form is also benign, and problems are related to difficulties in standing or walking or to associated Proteus syndrome.

Juvenile aponeurotic fibroma is benign, but recurrences are common.

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Contributor Information and Disclosures
Author

Firas G Hougeir, MD Staff Dermatologist, Private Practice, Georgia

Firas G Hougeir, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: XOMA; Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Neil Shear, MD Professor and Chief of Dermatology, Professor of Medicine, Pediatrics and Pharmacology, University of Toronto Faculty of Medicine; Head of Dermatology, Sunnybrook Women's College Health Sciences Center and Women's College Hospital, Canada

Neil Shear, MD is a member of the following medical societies: Canadian Medical Association, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada, Canadian Dermatology Association, American Academy of Dermatology, American Society for Clinical Pharmacology and Therapeutics

Disclosure: Nothing to disclose.

Acknowledgements

Jose M Mascaro, MD, MS Chairman, Professor, Department of Dermatology, Hospital Clinic, University of Barcelona, Spain

Jose M Mascaro, MD, MS is a member of the following medical societies: American Dermatological Association, American Society of Dermatopathology, and International Academy of Pathology

Disclosure: Nothing to disclose.

References
  1. McPoil TG, Martin RL, Cornwall MW, Wukich DK, Irrgang JJ, Godges JJ. Heel pain--plantar fasciitis: clinical practice guildelines linked to the international classification of function, disability, and health from the orthopaedic section of the American Physical Therapy Association. J Orthop Sports Phys Ther. 2008 Apr. 38(4):A1-A18. [Medline].

  2. Wiedemann HR, Burgio GR, Aldenhoff P, Kunze J, Kaufmann HJ, Schirg E. The proteus syndrome. Partial gigantism of the hands and/or feet, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies and possible accelerated growth and visceral affections. Eur J Pediatr. 1983 Mar. 140(1):5-12. [Medline].

  3. Montgomery E, Lee JH, Abraham SC, Wu TT. Superficial fibromatoses are genetically distinct from deep fibromatoses. Mod Pathol. 2001 Jul. 14(7):695-701. [Medline].

  4. Morrison WB, Schweitzer ME, Wapner KL, Lackman RD. Plantar fibromatosis: a benign aggressive neoplasm with a characteristic appearance on MR images. Radiology. 1994 Dec. 193(3):841-5. [Medline].

  5. Bancroft LW, Peterson JJ, Kransdorf MJ. Imaging of soft tissue lesions of the foot and ankle. Radiol Clin North Am. 2008 Nov. 46(6):1093-103, vii. [Medline].

  6. Scheler J, Rehani B, Percy T, et al. Increased F-18 FDG uptake on positron emission tomography/computed tomography imaging caused by plantar fibromatosis. Clin Nucl Med. 2008 Apr. 33(4):280-1. [Medline].

  7. Alman BA, Naber SP, Terek RM, Jiranek WA, Goldberg MJ, Wolfe HJ. Platelet-derived growth factor in fibrous musculoskeletal disorders: a study of pathologic tissue sections and in vitro primary cell cultures. J Orthop Res. 1995 Jan. 13(1):67-77. [Medline].

  8. DeBrule MB, Mott RC, Funk C, Nixon BP, Armstrong DG. Osseous metaplasia in plantar fibromatosis: a case report. J Foot Ankle Surg. 2004 Nov-Dec. 43(6):430-2. [Medline].

  9. Evans HL. Multinucleated giant cells in plantar fibromatosis. Am J Surg Pathol. 2002 Feb. 26(2):244-8. [Medline].

  10. van der Veer WM, Hamburg SM, de Gast A, Niessen FB. Recurrence of plantar fibromatosis after plantar fasciectomy: single-center long-term results. Plast Reconstr Surg. 2008 Aug. 122(2):486-91. [Medline].

  11. Wapner KL, Ververeli PA, Moore JH Jr, Hecht PJ, Becker CE, Lackman RD. Plantar fibromatosis: a review of primary and recurrent surgical treatment. Foot Ankle Int. 1995 Sep. 16(9):548-51. [Medline].

 
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Superficial fibromatosis of the heel.
Juvenile aponeurotic fibroma.
Hamartomatous fibromatosis.
 
 
 
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