Dupuytren-Like Contracture of the Foot: Ledderhose Disease.
ABSTRACT: Introduction Plantar fibromatosis is a rare hyperproliferative disease of plantar aponeurosis and is also called Ledderhose disease. Case properties and treatment are discussed in this report. Case Report A 30-year-old man presented with painful bilateral plantar nodules. He had multiple and bilateral fixed and solid nodules on the plantar and medial side of his feet measuring 1 cm each. Ultrasound was performed and hypoechoic homogeneous nodules were detected. The patient underwent surgery, and the nodes were removed via a plantar incision with 2-cm safety distance. Discussion Ledderhose disease is a rare, hyperproliferative disorder of the plantar aponeurosis. The nodules are slow growing and found in the medial part of the plantar fascia. The precise etiology remains unknown. The treatment options are conservative management, steroid injections, radiotherapy, and surgery. Conclusion The main cause of this disease remains uncertain. Related conditions should be evaluated, and a patient who presents with Dupuytren or Peyronie disease should also be investigated for Ledderhose disease.
Project description:Palmar fibromatosis (Dupuytren disease/contracture) is the most common type of fibromatosis, defined as a benign proliferation of fibroblasts and myofibroblasts. The disease process is most common in white, middle-aged and older men occurring at the distal palmar crease leading to nodules and contracture, which in many cases recur after surgical treatment. In a similar process, plantar fibromatosis (Ledderhose disease) is a proliferation of fibroblasts and myofibroblasts on the plantar aponeurosis of mostly middle-aged patients that may lead to painful nodules but usually does not lead to contracture. Both processes are histologically similar, composed of a bland cellular proliferation of spindle cells with a bluish appearance and with a variable amount of background collagen, depending on the age of the lesion. The etiology of both lesions is still uncertain, while treatment ranges from observation to surgery, with some pharmacologic agents being investigated with mixed success. In this paper we provide an overview of both processes with regards to clinical and radiologic findings, pathophysiology, diagnosis, treatment, and prognosis.
Project description:To determine the safety and efficacy of collagenase clostridium histolyticum (CCH) injection for the treatment of palmar Dupuytren disease nodules.In this 8-week, double-blind trial, palpable palmar nodules on one hand of adults with Dupuytren disease were selected for treatment. Patients were randomly assigned using an interactive web response system to receive a dose of 0.25 mg, 0.40 mg, or 0.60 mg (1:1:1 ratio) and then allocated to active treatment (CCH) or placebo (4:1 ratio). All patients and investigators were blinded to treatment. One injection was made in the selected nodule on Day 1. Caliper measurements of nodule length and width were performed at screening and at Weeks 4 and 8. Investigator-reported nodular consistency and hardness were evaluated at baseline and Weeks 1, 4, and 8. Investigator-rated patient improvement (1 [very much improved] to 7 [very much worse]) and patient satisfaction were assessed at study end.In the efficacy population (n = 74), percentage changes in area were significantly greater with CCH 0.40 mg (-80.1%, P = 0.0002) and CCH 0.60 mg (-78.2%, P = 0.0003), but not CCH 0.25 mg (-58.3%, P = 0.079), versus placebo (-42.2%) at post-treatment Week 8. Mean change in nodular consistency and hardness were significantly improved with CCH versus placebo at Weeks 4 and 8 (P ? 0.0139 for all). At Week 8, investigator global assessment of improvement was significantly greater with CCH 0.40 mg and 0.60 mg (P ? 0.0014) but not statistically significant with CCH 0.25 mg versus placebo (P = 0.13). Most patients were "very satisfied" or "quite satisfied" with CCH 0.40 mg and 0.60 mg. Contusion/bruising (50.0% to 59.1%) was the most common adverse event with CCH treatment.In patients with Dupuytren disease, a single CCH injection significantly improved palmar nodule size and hardness. The safety of CCH was similar to that observed previously in patients with Dupuytren contracture.ClinicalTrials.gov identifier: NCT02193828 . Date of trial registration: July 2, 2014 to December 5, 2014.
Project description:<b>Background:</b> Indication for intervention in Dupuytren disease is influenced by many factors, including location and extent of disease, surgeon preference, and comfort level with different treatment techniques. The aim of this study was to determine current Dupuytren disease management trends. <b>Methods:</b> A questionnaire was sent through the American Society for Surgery of the Hand to all members. In addition to demographic data, questions focused on indications for different procedural interventions based on location of disease, age, and activity level of the patient. <b>Results:</b> Approximately 24% of respondents completed the survey. Respondents were mostly orthopedic surgeons in private practice who do not work with residents or fellows. Respondents preferred collagenase over needle aponeurotomy and limited fasciectomy for primary Dupuytren disease involving only the metacarpophalangeal (MCP) joint. Limited fasciectomy was the preferred treatment for primary Dupuytren disease involving the MCP and proximal interphalangeal joints. For a patient amenable to any treatment option, the majority would use collagenase, although 87.1% felt that fasciectomy offered the longest disease-free interval. Furthermore, given the option of a young, working patient, 42.7% would use collagenase, while plastic and general surgeons were more likely to treat this patient with limited fasciectomy. More plastic surgeons (vs orthopedic) believe that limited fasciectomy yields the longest disease-free interval. For a patient amenable to any surgical option, orthopedic surgeons prefer collagenase, whereas plastic hand surgeons prefer a limited fasciectomy. <b>Conclusion:</b> There are several procedural options for the treatment of Dupuytren disease. This study details current practice patterns among hand surgeons and reveals the increasingly prevalent use of collagenase.
Project description:BACKGROUND:The Dupuytren disease is a benign fibroproliferative disorder that leads to the formation of the collagen knots and fibres in the palmar fascia. The previous studies reveal different levels of Dupuytren's prevalence worldwide; hence, this study uses meta-analysis to approximate the prevalence of Dupuytren globally. METHODS:In this study, systematic review and meta-analysis have been conducted on the previous studies focused on the prevalence of the Dupuytren disease. The search keywords were Prevalence, Prevalent, Epidemiology, Dupuytren Contracture, Dupuytren and Incidence. Subsequently, SID, MagIran, ScienceDirect, Embase, Scopus, PubMed and Web of Science databases and Google Scholar search engine were searched without a lower time limit and until June 2020. In order to analyse reliable studies, the stochastic effects model was used and the I2 index was applied to test the heterogeneity of the selected studies. Data analysis was performed within the Comprehensive Meta-Analysis Software version 2.0. RESULTS:By evaluating 85 studies (10 in Asia, 56 in Europe, 2 in Africa and 17 studies in America) with a total sample size of 6628506 individuals, the prevalence of Dupuytren disease in the world is found as 8.2% (95% CI 5.7-11.7%). The highest prevalence rate is reported in Africa with 17.2% (95% CI 13-22.3%). According to the subgroup analysis, in terms of underlying diseases, the highest prevalence was obtained in patients with type 1 diabetes with 34.1% (95% CI 25-44.6%). The results of meta-regression revealed a decreasing trend in the prevalence of Dupuytren disease by increasing the sample size and the research year (P < 0.05). CONCLUSION:The results of this study show that the prevalence of Dupuytren disease is particularly higher in alcoholic patients with diabetes. Therefore, the officials of the World Health Organization should design measures for the prevention and treatment of this disease.
Project description:Importance:Owing to its tendency to recur, Dupuytren contracture often requires multiple treatments, which places additional economic burden on health care. The likelihood of contracture recurrence varies not only with treatment but also with disease characteristics, such as contracture severity and location, but prior cost-effectiveness analyses of Dupuytren contracture treatments have not considered these patient-specific disease characteristics. Objective:To identify the most cost-effective treatment regimen for patients with recurrent Dupuytren contracture. Design, Setting, and Participants:This economic evaluation was conducted with state-transition microsimulation modeling using data from published studies and Medicare. A simulated cohort of 10?000 individuals with Dupuytren contracture was created. Patients could transition yearly between the following health states: symptom-free, symptomatic, and death. Available treatments were collagenase clostridium histolyticum injection, percutaneous needle aponeurotomy (PNA), and limited fasciectomy (LF); individuals randomly chose any treatment when symptomatic. Patients were limited to 3 rounds of treatment for a contracture affecting 1 joint, totaling 27 unique combinations. If the contracture recurred after 3 treatments, patients lived with the disease for the remainder of life. Exposures:PNA, collagenase clostridium histolyticum injection, or LF. Main Outcomes and Measures:Quality-adjusted life-years (QALYs), total costs (in US dollars), and incremental cost-effectiveness ratios (ICERs). A willingness-to-pay threshold of $100?000 per quality-adjusted life-year was used to assess cost-effectiveness. Results:For the base case scenario of a patient aged 60 years with recurrent, low-severity metacarpophalangeal (MCP) joint contracture, repeated PNA treatment was the only cost-effective treatment (2 PNA treatments followed by LF vs 3 PNA treatments, ICER [Monte Carlo SE]: $212?647/QALY [$36?000/QALY]). For recurrent high-severity MCP joint contractures, treatment regimens composed of PNA and LF were cost-effective (ICER [Monte Carlo SE], $93?932/QALY [$16?500/QALY]). LF was cost-effective for high-severity MCP joint contracture (ICER [Monte Carlo SE], $98?624/QALY [$26?233/QALY]). For recurrent proximal interphalangeal (PIP) joint contractures, PNA was the only cost-effective treatment, regardless of severity (eg, 2 PNA treatments followed by LF vs 3 PNA treatments for low-severity PIP joint contracture, ICER [Monte Carlo SE]: $263?726/QALY [$29?000/QALY]). Any combination with collagenase clostridium histolyticum injection compared with 3 PNA treatments had an ICER greater than $100?000 per QALY. Probabilistic sensitivity analysis estimated a 44%, 15%, 41%, and 52% chance of a regimen consisting of only PNA being cost-effective in low-severity MCP, high-severity MCP, low-severity PIP, and high-severity PIP joint contractures, respectively. Conclusions and Relevance:The results of this study suggest that LF is a cost-effective intervention for recurrent high-severity MCP joint contractures. For recurrent low-severity MCP joint contractures and PIP joint contractures of all severity levels, PNA was the only cost-effective intervention. Collagenase clostridium histolyticum injections were not a cost-effective intervention for recurrent Dupuytren contracture and should not be preferred over PNA or LF.
Project description:Dupuytren disease is highly prevalent and the finger contractures can be very extensile, compromising the patients' hand function. To restore full function, contractures have been addressed by cutting the causative strands for nearly 200 years, ever since Baron Guillaume Dupuytren demonstrated his technique at the beginning of the nineteenth century. Surgery can be minimal (fasciotomy) or quite invasive (fasciectomy and even skin replacement). However, in the last decade translational research has introduced the non-surgical technique of enzymatic fasciotomy with collagenase injections. Now, finger contractures can be released with single injections on monthly intervals, to address one joint contracture at a time. However, in hands affected with Dupuytren contractures to the extent that the patient calls for treatment, most often more than one joint is involved. In surgical treatment options all contracted joints are addressed in a single procedure. Nevertheless, extensile surgery withholds inherent risks of complications and intense rehabilitation. Today, the minimally-invasive method with enzymatic fasciotomy by collagenase injection has demonstrated reliable outcomes with few morbidities and early recovery. However, single-site injection is todays' standard procedure and multiple joints are addressed in several sessions with monthly intervals. This triggers a longer recovery and treatment burden in severely affected hands even though surgery is avoided. Therefore, further treatment modalities of collagenase use are explored. Adjustments in the treatment regimes' flexibility and collagenase injections addressing more than one joint contracture simultaneously will improve the burden of multiple sessions and, therefore, enzymatic fasciotomy may become the preferred method in more extensile Dupuytren contractures. In this independent review, the challenge of Dupuytren disease affecting a single versus multiple joints is presented. The pros and cons of collagenase use are weighed, founded by the available scientific background. The demands and options for collagenase in future treatment regimens for extensile Dupuytren contractures are discussed.
Project description:The rigidity of the human foot is often described as a feature of our evolution for upright walking and is bolstered by a thick plantar aponeurosis that connects the heel to the toes. Previous descriptions of human foot function consider stretch of the plantar aponeurosis via toe extension (windlass mechanism) to stiffen the foot as it is levered against the ground for push-off during walking. In this study, we applied controlled loading to human feet <i>in vivo</i>, and studied foot function during the push-off phase of walking, with the aim of carefully testing how the foot is tensioned during contact with the ground. Both experimental paradigms revealed that plantar aponeurosis strain via the 'windlass mechanism' could not explain the tensioning and stiffening of the foot that is observed with increased foot-ground contact forces and push-off effort. Instead, electromyographic recordings suggested that active contractions of ankle plantar flexors provide the source of tension in the plantar aponeurosis. Furthermore, plantar intrinsic foot muscles were also contributing to the developed tension along the plantar aspect of the foot. We conclude that active muscular contraction, not the passive windlass mechanism, is the foot's primary source of rigidity for push-off against the ground during bipedal walking.
Project description:A 46-year-old man presented with a six-month history of lumps in the sole of his left foot. Physical examination revealed two nodules, one tender and one firm, at the plantar left foot with no overlying skin changes. Although the initial radiographs were normal, magnetic resonance imaging of the left foot demonstrated two nodules along the medial band of the plantar fascia, characteristic of plantar fibromas. The patient opted for surgical excision. There was no further recurrence of symptoms after surgery. We describe the clinical and radiological features of plantar fibromatosis and briefly discuss other causes of lumps and pain in the sole of the foot.
Project description:For decades, low- and moderate-dose radiation therapy (RT) has been shown to exert a beneficial therapeutic effect in a multitude of non-malignant conditions including painful degenerative muscoloskeletal and hyperproliferative disorders. Dupuytren and Ledderhose diseases are benign fibroproliferative diseases of the hand/foot with fibrotic nodules and fascial cords, which determine debilitating contractures and deformities of fingers/toes, while keloids are exuberant scar formations following burn damage, surgery, and trauma. Although RT has become an established and effective option in the management of these diseases, experimental studies to illustrate cellular composites and factors involved remain to be elucidated. More recent findings, however, indicate the involvement of radiation-sensitive targets like mitotic fibroblasts/myofibroblasts as well as inflammatory cells. Radiation-related molecular mechanisms affecting these target cells include the production of free radicals to hamper proliferative activity and interference with growth factors and cytokines. Moreover, an impairment of activated immune cells involved in both myofibroblast proliferative and inflammatory processes may further contribute to the clinical effects. We here aim at briefly describing mechanisms contributing to a modulation of proliferative and inflammatory processes and to summarize current concepts of treating hyperproliferative diseases by low and moderate doses of ionizing radiation.
Project description:INTRODUCTION:Peyronie disease (PD) is a progressive fibrotic disorder of the penile tunica albuginea that results in fibrotic penile plaques and can lead to penile deformity. Characterized by aberrant fibrosis resulting in part from the persistence of myofibroblasts and altered gene expression, the molecular factors underpinning PD and other related fibrotic diatheses are just being elucidated. A genetic link to PD was first identified three decades ago using pedigree analyses. However, the specific genetic factors that predispose patients to aberrant fibrosis remain unknown, and the relations between these fibrotic conditions and other heritable diseases, including malignancy, are uncharacterized. AIM:To review the current landscape linking molecular and genetic factors to aberrant fibrosis in PD and related fibrotic diatheses, including Dupuytren disease. METHODS:Review and evaluation of the literature from 1970 to the present for genetic factors associated with PD were performed. MAIN OUTCOME MEASURES:Data describing the genetic factors associated with PD were obtained. RESULTS:We describe the known structural chromosomal abnormalities and single-nucleotide polymorphisms associated with fibrotic diatheses and discuss the spectrum of differential gene expression data comparing normal tissues with those derived from men with PD or Dupuytren disease. We discuss epigenetic mechanisms that might regulate gene expression and alter predisposition to fibrosis. CONCLUSION:Although the current understanding of the genetic factors associated with PD is limited, significant advances have been made during the past three decades. Further research is necessary to provide a more comprehensive understanding of the landscape of genetic factors responsible for the development of PD.