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Morbus Dupuytren/Morbus Ledderhose
Abstract
The digitopalmar contracture named after Guillaume Dupuytren (1832, 1834) [36, 37] and the corresponding digito-plantar contracture named after Georg Ledderhose (1897) [91] are fibrous, proliferative hyperplastic disorders of pre-existing connective tissue structures of the fascia of the fingers
and palm or the toes and sole, respectively. However, in Morbus Dupuytren (MD) and Morbus Ledderhose (ML), the digitopalmar
and digitoplantar changes are not just singular afflictions, but part of a systemic connective tissue disorder [41], which is confirmed by subtle biochemical changes and obvious ectopic fibrous deposits, which are located, for example,
above the dorsal proximal interphalangeal joints (= knuckle pads), on the auricular helix, the hand wrist, the elbow and inside
the penis (= Morbus Peyronie). These tissue changes are histologically identical, but all efforts to identify a single cause
of this generalized disorder have failed, and numerous hypotheses about the disease onset and progression have been published,
but a simple and straightforward explana tion is still missing.
The abnormal proliferation of the fibrous tissue in the plantar fascia, Morbus Ledderhose is a non-malignant thickening of the deep connective tissue of the foot, a relatively rare pa-thology that intensely affects patients through marked pain and functional impotence. Re-habilitation treatments are poorly presented in the literature. To apply a non-invasive physiotherapeutic treatment which leads to consistent results in reducing pain, reducing the size of nodules or even their disappearance, the functional resumption of the foot, an increase in the life quality of affected patients. In the study, pa-tients were divided into 2 groups: G1 received treatment consisting of shockwave therapy complemented with SIS therapy, G2 received treatment consisting of ultrasound, laser therapy, kinesiotherapy, massage, excluding the application of shockwave therapy and SIS. Some of the patients associate comorbidities such as type II diabetes, Dupuytren’s disease or, more rarely, with Peyronie’s disease (penile fibromatosis). Important positive results have been obtained: a considerable decrease in the pain level of patients, the dissi-pation of consistency and decrease in the size of plantar nodules, an increase in the life quality of the patients with the resumption of productive activities. The treat-ment with shock waves and super inductive system has an increased efficiency both folowing the first cure and after various consecutive cures applied consistently at 6 months or maybe even faster, at 4-5 months, if necessary, if the pathology advances faster by intensifying fibrosis.
Ledderhose’s disease, or plantar fibromatosis, is a rare benign condition characterized by the excessive proliferation of connective tissue in the plantar aponeurosis, leading to slow-growing nodules. Although it usually presents unilaterally, bilateral involvement occurs in about 25% of cases. The exact cause is unknown, but associations with diabetes mellitus, liver disease, and repeated trauma have been noted. Treatment options are diverse, with surgery often being the primary approach, but radiation therapy has emerged as a promising alternative. We present the case of a 70-year-old Moroccan man with a painful plantar nodule, diagnosed with Ledderhose’s disease. Initial measures were insufficient, leading to the use of radiation therapy. The patient received Three-Dimensional Conformal Radiotherapy (3D-CRT) at a dose of 30.0 Gy, administered in two courses. Following treatment, the patient experienced significant symptom relief and a notable reduction in nodule size. Although surgery was eventually required due to disease progression, it is noteworthy that radiation therapy effectively delayed the need for surgical intervention.
Background and purpose:
Radiotherapy is considered a treatment option for Ledderhose disease. However, its benefits have never been confirmed in a randomised controlled trial. Therefore, the LedRad-study was conducted.
Materials and methods:
The LedRad-study is a prospective multicentre randomised double-blind phase three trial. Patients were randomised to sham-radiotherapy (placebo) or radiotherapy. The primary endpoint was pain reduction at 12 months after treatment, measured with the Numeric Rating Scale (NRS). Secondary endpoints were pain reduction at 6 and 18 months after treatment, quality of life (QoL), walking abilities and toxicity.
Results:
A total of 84 patients were enrolled. At 12 and 18 months, patients in the radiotherapy group had a lower mean pain score compared to patients in the sham-radiotherapy group (2.5 versus 3.6 (p=0.03) and 2.1 versus 3.4 (p=0.008), respectively). Pain relief at 12 months was 74% in the radiotherapy group and 56% in the sham-radiotherapy group (p=0.002). Multilevel testing for QoL scores showed higher QoL scores in the radiotherapy group compared to the sham-radiotherapy group (p<0.001). Moreover, patients in the radiotherapy group had a higher mean walking speed and step rate with barefoot speed walking (p=0.02). Erythema, skin dryness, burning sensations and increased pain were the most frequently reported side effects. These side effects were generally graded as mild (95%) and the majority (87%) were resolved at 18 months follow-up.
Conclusion:
Radiotherapy for symptomatic Ledderhose disease is an effective treatment resulting in a significant pain reduction, improvement of QoL scores and bare feet walking abilities, in comparison to sham-radiotherapy.
Ledderhose disease (plantar fibromas) is histologically related to Dupuytren disease, which has been successfully treated for years with radiotherapy. Many conservative treatments have been advanced for plantar fibromas, including accommodative orthotic devices, which help but do not cure the disease. Surgery is considered the mainstay of treatment for this malady, but the failure rate has been as high as 100%, depending on the type of fasciectomy. Radiotherapy is a new, exciting modality that has shown promising results for treating plantar fibromas.
The aim of the study was the comparative analysis of treatment for gonarthritis of 0–2 stages by symptomatic therapy (Symptomatic slow acting drugs for osteoarthritis — SYSADOA) and in combination with orthovoltage x-ray therapy (OVRT) within an open randomized study.
Material and methods. The study included patients with clinically confirmed gonarthritis. Patients were randomly divided into 2 groups, each of them was administered a combined SYSADOA glucosamine (500 mg) and chondroitin (400 mg) sulfate, OVRT was prescribed to patients of second group. OVRT was performed on X-ray machine Xstrahl-200, a single dose was 0,45 Gy, in 10 sessions to a total dose of 4,5 Gy. The pain syndrome was evaluated by VAS, WOMAC scales, efficiency of the treatment by OARSI/OMERACT (reduction of VAS and WOMAC by more than 20% of the baseline). Disability and the number of endoprosthesis replacement were also analyzed. Evaluation was performed before, after treatment, 6, 12 and 36 months.
Results. There were selected 300 patients, 150 in each group, eight people were lost. By the end of the treatment there were no statistically significant differences in the reduction of pain syndrome evaluating by VAS and WOMAC A. After 6–36 months degree of pain reduction was significantly higher in the OVRT group. After three years in the OVRT group, WOMAC A levels, VAS, VAS 20%, WOMAC A 20% were 21,9, 21,2, 69%, 78%, in the SYSADOA group 53,5, 54,9, 25%, 32%, p<0,0001. In the SYSADOA group, by the end of follow-up, 48 (32,9%) patients had a disability group, 42 (28,8%) underwent endoprosthesis replacement, in the combined treatment group — four and four cases (2,7%), respectively, p<0,0001.
Conclusion: there was stable reduction of pain syndrome level among patients underwent OVRT, and improvement of the functional state of the joint, which allows to reduce the frequency of disability and knee endoprosthesis replacement.
Plantar fibromatosis (morbus Ledderhose), an extra-abdominal desmoid tumor of the plantar foot, is a rare benign hyperproliferative disorder of the plantar fascia with an unknown etiology. The main clinical characteristics include slow growing nodules on the medial and central bands of the plantar fascia, which may become painful and negatively affect ambulation. Most established conservative therapies today target symptomatic relief. As symptoms progress, therapies such as injections, shockwave ablation, radiation, and/or surgery may be required. This review aims to provide insight into the pathophysiology of this condition in addition to detailing current and investigational therapies for this disorder. Many therapies have been proven in similar conditions, which could lead to promising treatment options for plantar fibromatosis.
Levels of evidence:
Level V: Expert opinion.
This chapter reviews plantar fibromatosis. It defines the condition and shows its origins and genetics. It reviews the histology and possible differential diagnoses. The symptoms are reviewed and causes explained. The medical treatments and surgical interventions are discussed. The history and physiology of cryotherapy is reviewed. It will show why cryosurgery provides an excellent alternative to traditional surgical or conservative treatments. Research has shown about an 80% success rate. Patients have been very receptive to having this procedure done in comparison to traditional surgical intervention.
While the modern use and indication for radiotherapy (RT) of malignant skin disorders is a well-established strategy which includes the treatment of basal cell and squamous cell carcinoma, melanoma, Merkel cell tumors Kaposi's sarcoma, and cutaneous lymphomas (all, see previous chapters of this book), the use of ionizing radiation for nonmalignant or benign skin disorders is less recognized, is not represented in actual interdisciplinary treatment guidelines, and has clearly decreased as many new and better systemic and local noninvasive and invasive treatment options have become available in the past two decades. Moreover, in many dermatological departments throughout the world, the actual training and knowledge of medical specialists about the possible implementation of ionizing radiation to treat nonmalignant skin disorders has slowly faded away and is dismissed in modern dermatology textbooks. Local surgical methods and/or systemic medication options dominate the actually recommended treatment strategies for most nonmalignant skin disorders.
Radiation therapy (RT) is an established and effective treatment modality in the management of a large variety of hyperproliferative disorders and benign neoplasms. Objective of this article is to summarize the updated DEGRO consensus S2e guideline recommendations.
This report comprises an overview of the relevant aspects of the updated guidelines with regard to treatment decision, dose prescription, and RT technique for a selected group of disorders including Morbus Dupuytren (MD)/Morbus Ledderhose (ML), keloids, Peyronie's disease (induratio penis plastica, IPP), desmoid tumors, pigmented villonodular synovitis (PVNS), symptomatic vertebral hemangiomas (sVH), and Gorham-Stout syndrome (GSS). On the basis of results in the literature, we attempted to classify the level of evidence (LoE) and the grade of recommendation (GR) according to the Oxford criteria.
There is comprehensive evidence in the literature that RT is a reasonable and effective treatment modality for the treatment of all the above-mentioned disorders. The LoE varies from 2c to 4, and GR varies from A to C.
The use of RT can be recommended for the interdisciplinary management of most of the reported disorders. It can be used in the primary treatment approach and as an effective adjunct to other treatment modalities or in some indications as a valuable alternative treatment option. We hope that the updated DEGRO S2e consensus guideline recommendations are a helpful tool for radiation oncologists in the clinical decision-making process.
Background
Up to present no curative treatment is known for Dupuytren's disease (DD). Surgery remains the most common treatment but lack of long-term efficacy and complications limit this therapeutic option.Objective
In a retrospective analysis, the results of radiotherapy with soft X-rays in the treatment of DD were evaluated.MethodsA total of 206 patients (297 affected hands) with DD were included. Radiation therapy was carried out with soft X-rays. A structured questionnaire considering patient and disease characteristics and effects of radiotherapy was evaluated after a median follow-up time of 40 months.ResultsNinety-three (45%) of the 206 treated patients were reported on a regression of symptoms after radiation. No further disease progression (including patients with regression) was present in 165 patients (80%). Satisfaction with the therapy was expressed with an average score of 7.9 points (visual analogue scale, 0 = not satisfied, 10 = extremely satisfied). Subjective therapeutic effects for 426 nodules and/or cords showed a reduction of 92 nodules and/or cords.Conclusion
In 206 DD patients further disease progression was stopped in most patients. Radiotherapy proved to be well-tolerated, successful and satisfying for the patients.
Background:
Dupuytren disease (DD) is a connective tissue disorder that consists in fibromatosis of the palmar and digital fascia (in form of nodules or flanges) that leads to the development of flexion contractures of the palm and fingers. The little and ring finger are particularly affected. The disease can limit hand function, reducing the quality of life. The disease can have a traumatic origin and is also associated with conditions such as diabetes mellitus, alcoholism, dyslipidemia, epilepsy and AIDS, among others. However, none of these conditions can fully explain the genesis of DD. A hereditary component is described in 40% of patients and is attributed to an autosomal dominant gene of variable penetrance, probably related to collagen synthesis. However there are also spontaneous and recessive inheritance cases. The diagnosis is clinical and based on physical examination. Treatment ranges from observation or use of injectable collagenase to the surgical option in cases with significant functional limitations.
The major unexplained phenomenon in fibrotic conditions is an increase in replicating fibroblasts. In this report we present evidence that oxygen free radicals can both stimulate and inhibit proliferation of cultured human fibroblasts, and that fibroblasts themselves release superoxide (O2.-) free radicals. Fibroblasts released O2.- in concentrations which stimulated proliferation, a finding confirmed by a dose-dependent inhibition of proliferation by free radical scavengers. Oxygen free radicals released by a host of agents may thus provide a very fast, specific and sensitive trigger for fibroblast proliferation. Prolonged stimulation may result in fibrosis, and agents which inhibit free radical release may have a role in the prevention of fibrosis.
Es werden zwei Dosiskonzepte bei Bestrahlung des Morbus Dupuytren verglichen, um die Progression der Erkrankung bzw. eine Handoperation zu verhindern.
Patienten und Methode: 129 Patienten (67 Männer, 62 Frauen) wurden prospektiv in zwei Gruppen behandelt und für mindestens ein Jahr nachbeobachtet. 69 Patienten waren an beiden Händen, 60 an einer Hand erkrankt (gesamt: 198 bestrahlte Hände). Nach Tubiana befanden sich 73 Hände im Stadium N, 61 im Stadium N/I (≤ 10° Beugekontraktur), 59 im Stadium I (11 bis 45°) und fünf im Stadium II (46 bis 90°). Bestrahlt wurden am Orthovoltgerät (120 kV) mit 40 cm Abstand und individuellen Bleigummi-Blenden. Gruppe A (63 Patienten/95 Hände) erhielt 10-mal 3 Gy (gesamt: 30 Gy; 5-mal 3 Gy täglich in zwei Serien, acht Wochen Pause zwischen den Serien; Gruppe B (66 Patienten/103 Hände) erhielt 7-mal 3 Gy (gesamt: 21 Gy) in zwei Wochen. Die Patienten- und Krankheitsparameter waren in beiden Gruppen gleich verteilt. Die Auswertung (Toxizität, Effektivität) erfolgte drei und zwölf Monate nach Bestrahlung. Subjektive (Patient) und objektive Parameter (Palpation, Messungen, Photovergleich – Arzt) wurden bewertet.
Ergebnisse: Die Akuttoxizität war gering: 76 (38%) Hände hatten Hautreaktionen CTC-Grad 1 und zwölf (6%) CTC-Grad 2. Chronische Folgen (Trockenheit, Atrophie, Sensibilitätsstörungen, LENT-Grad 1) traten bei neun (5%) Händen auf. Drei und zwölf Monate nach Radiotherapie wurde subjektiv und objektiv eine Rückbildung der Symtome, Knoten und Stränge in beiden Gruppen festgestellt (p < 0,01): Insgesamt sprachen 110 (56%) Hände an (Gruppe A: 55; B: 55), 74 (37%) blieben stabil (A: 35; B: 39). Die Zahl der Knoten, Stränge und Hautveränderungen nahm nach drei und zwölf Monaten signifikant ab (p < 0,01). Nach einem Jahr waren die Symptome an 16 (8%) Händen progredient (“Therapieversager”: A = 7; B = 9). Sieben von 60 Patienten mit einseitigem Befall wurden wegen Progredienz der zunächst nicht betroffenen Hand bestrahlt.
Schlussfolgerung: Die Radiotherapie verhindert erfolgreich eine Progression beim Morbus Dupuytren. Beide Dosiskonzepte zeigen nach einem Jahr gleiche Wirksamkeit; die Akuttoxizität ist bei Therapiekonzept B (7-mal 3 Gy) geringfügig verstärkt, bei der Spättoxizität besteht kein Unterschied. Langzeitbeobachtungen von mehr als fünf Jahren sind abzuwarten, bevor ein bestimmtes Dosiskonzept empfohlen werden kann.
Aim
Radiotherapy was applied in our clinic to prevent the disease progression in early stage Dupuytren’s contracture. Initial response, long-term outcome, acute and late toxicity of the treatment were evaluated in a retrospective analysis.
Patients and Method
Between 1982 and 1993, 96 patients (142 hands) received orthovoltage radiotherapy, which consisted of 2 radiotherapy courses with daily fractionation of 5×3 Gy (total dose: 30 Gy) separated by a 6 weeks interval. The Dupuytren’s contracture was staged according to the classification of Tubiana et al. [38]. The initial evaluation was performed 3 months after completion of radiotherapy, while long-term outcome was analysed at last follow-up between February and April 1994. The mean follow-up was 6±2 (range 1 to 12) years. Fifty-seven patients with a follow-up of ≥5 (median 7,5; mean 9,5 to 12) years were separately evaluated for long-term outcome, i. e. prevention of disease progression. Acute and late treatment toxicity was assessed using the RTOG/EORTC criteria.
Results
According to stage, 130 (92%) cases remained stable at 3 months follow-up, 10 (7%) improved and 2 (1%) progressed. An objective reduction of symptomatic cords and nodules was achieved in 107 (75%) cases at 3 months follow-up. Moreover, 87% of the patients reported a subjective relief of symptoms. In long-term follow-up, only 16 of 142 (11%) cases had progressed according to stage. In the group with a minimum follow-up of 5 years (n=57), 44 (77%) patients experienced no progression, while 13 (23%) progressed inside (8 cases) or outside (5 cases) of the radiotherapy field. Many “failures” could have been avoided with appropriate choice of larger safety margins included in the treated portals, however, most failures were successfully managed by a second radiotherapy or hand surgery.
Conclusion
Radiotherapy prevents disease progression for early stage Dupuytren’s contracture. Thus, an otherwise necessary surgical procedure in adavanced stages of Dupuytren’s contracture can be avoided. Moreover, in case of disease progression despite radiotherapy a second radiotherapy or salvage operation is still feasible.
Dupuytren’s contracture nodules, but not cords, contain myofibroblasts. These cells, which combine many electron microscopic, physiologic, and immunohistochemical characteristics of fibroblasts and smooth muscle cells, are probably the active force of contraction. Prominent myofibroblasts and intracellular microtubules correlate with increased likelihood of clinical recurrence after surgery. Tissue culture of cells derived from Dupuytren’s contracture myofibroblasts show consistently slower cell replication than from fibroblasts and show persistence of electron microscopic characteristics in early passages.
Research in Dupuytren’s contracture myofibroblasts has been done on human tissue and so has clinical correlation. Myofibroblast presence may help to predict recurrence of disease and suggests that palmar skin should be excised when adherent to disease nodules. The theory of myofibroblasts helps explain why the open technique often succeeds, and why full thickness skin grafts inhibit recurrent contracture.
The systematic examination and recording, ray by ray, using the author’s method of assessment of Dupuytren’s disease have revealed that there are more Dupuytren’s disease cases affecting the radial side of the hand than usually believed.
The aponeurotic structures of the thumb and first web space are described. The location of diseased tissue coincides with the normal aponeurotic structures. Radial involvement alone is rare; most often, radial-side involvement is associated with ulnar-side involvement. Radial-sided lesions in the older patient progress slowly and rarely require surgery. In the younger patient, however, progress can be rapid, can lead to significant contractures, and recurrence after partial fasciectomy is frequent. Surgical management of these aggressive forms requires a large approach and an extensive excision. These cases are the best indications for dermofasciectomies.
1. Surveys of a working community, of a group of elderly people, and of an urban population show an incidence of Dupuytren's contracture among men varying from 0·1 per cent in the age group fifteen to twenty-four, to 18·1 per cent in those aged seventy-five and over; and among women from 0·5 per cent in the age group forty-five to fifty-four, to 9 per cent over seventy-five. It is estimated that in the population aged fifteen and over in Lancashire and Cheshire there will be 4·2 per cent of the men and 1·4 per cent of the women with some degree of palmar contracture.
2. There appears to be no relationship between the type of occupation and the incidence or severity of contracture in men, except that among those engaged in light manual work the proportion of mildly affected hands is higher, and of bilateral contracture lower, than among either non-manual or heavy manual workers.
3. Evidence is provided that rheumatoid arthritis, past polyarthritis, osteoarthritis, cervical spondylosis and Paget's disease occur no more often in those with Dupuytren's contracture than in other members of the community.
4. Examination of the patients in an epileptic colony confirms a strong association between Dupuytren's contracture and epilepsy. Knuckle-pads, plantar nodules and periarthritis of the shoulder are all more frequent in epileptic than in non-epileptic patients with Dupuytren's contracture. Epileptics also show a higher proportion with bilateral contractures and a greater tendency to a symmetrical pattern of contracture in the two hands. A strong constitutional factor, probably genetic, thus operates in persons with both diseases.
Nevertheless, the frequency of a positive family history of contracture is lower in the epileptic cases, and reasons for this are discussed.
5. From the limited material available in the literature there would appear to be an inverse relationship between the population of certain countries and the prevalence in them of Dupuytren's contracture. The possible significance of this is briefly discussed.
Außer der Beschreibung eines Patienten mit Dupuytrenscher Kontraktur (D.K.) des Ring- und Kleinfingers der linken Hand durch den Baseler Anatomen FELIX PLATER im Jahre 1614 findet man lange Zeit hindurch keine Angaben über diese Erkrankung in der medizinischen Literatur. Erst zu Beginn des vergangenen Jahrhunderts wird die Krankheit mehrfach erwähnt und als Crispatura tendinum (BOYER. 1826) bzw. als Paraprimma palmare (ALIBERT 1832) bezeichnet. Bereits 1822 beschrieb Sir A. COOPER die Palmaraponeurose als Sitz des pathologischen Geschehens und schlug die quere Durchtrennung der Kontrakturstränge als Behandlung vor. Er unterschied die Kontrakturen aus anderen Ursachen scharf von denen, die durch Schrumpfung der Palmaraponeurose bedingt waren. Zehn Jahre später erfolgte die klassische Beschreibung durch DUPUYTREN, dessen Name seither mit der Erkrankung untrennbar verbunden ist. DUPUYTREN verfügte über ein größeres Krankengut. Einige seiner Beobachtungen haben noch heute volle Gültigkeit. Nach der Veröffentlichung der Beobachtungen von DUPUYTREN wies WINDSOR (1834) darauf hin, daß HENRY CLINE bereits 1808 eine ausführliche Darstellung der Erkrankung in seinen Vorlesungen gegeben habe.
When we survey the more than 2000 cases of Dupuytren’s contracture treated in our clinic since 1956, we find that the severity of disease is reduced compared to 30 years ago. Considering the slow progress of the disease, there is reason to believe that the decreased severity is due to the patients’ earlier request for treatment.
• This prospective study was undertaken to assess the prevalence of Dupuytren's contracture (DC) and its relationship with possible causes, especially alcohol consumption and chronic liver disease. Four hundred thirty-two consecutively hospitalized patients were examined for evidence of DC. They were divided into five groups based on the following clinical, biologic, and histologic criteria: alcoholic cirrhosis (89 patients), noncirrhotic alcoholic liver disease (55 patients), chronic alcoholism without liver disease (46 patients), nonalcoholic chronic liver disease (68 patients), and a control group (174 patients). The prevalence of DC in these five groups of patients was 32.5%, 22%, 28%, 6%, and 12%, respectively; the prevalence of DC was higher in patients with cirrhotic or noncirrhotic alcoholic liver disease (25.5%) than it was in patients with nonalcoholic liver disease (6%), but it was not significantly different in alcoholic patients with or without liver disease. The relationship between DC and age, sex, manual labor, previous hand injuries, diabetes mellitus, alcohol consumption, and cigarette smoking was assessed by univariate and logistic regression methods. Nine variables were significantly different in patients with or without DC: age, sex, manual labor, previous hand injuries, diabetes mellitus, daily alcohol consumption, duration of alcohol consumption, total alcohol consumption, and duration of cigarette smoking. In our patients, variables that could explain DC were, in decreasing order, age, total alcohol consumption, sex (male), and previous hand injuries. In alcoholic patients, these variables were age and previous hand injuries; in nonalcoholic patients, these variables were age and cigarette smoking. These results emphasize the high prevalence of DC in alcoholic patients and the absence of a correlation between DC and chronic liver disease. Age and alcohol consumption are the best explanatory variables of DC in hospitalized patients.
(Arch Intern Med 1987;147:1065-1067)
The fibromatoses are distinctive lesions best defined as a group of nonmetastasizing fibrous tumors which tend to invade locally and recur after surgical excision. They may be multiple and familial, their growth rate often decreases after an initial period of rapid growth, and they may even regress spontaneously. They may be complicated by contractures and are sometimes associated with diseases of other organs. Different types of fibromatoses are not found in the same patient, and recurrences are more frequent in the young. As fibromatoses do not metastasize, they should never be called low-grade fibrosarcomas. The fibromatoses can be subdivided into the "adult" fibromatoses, which occur predominantly but not exclusively in people older than 20 years, and the "juvenile" fibromatoses, which usually affect patients aged less than 20 years. The adult fibromatoses comprise the Dupuytren-type fibromatoses, and the desmoid fibromatoses. Dupuytren-type fibromatoses are further subclassified into palmar fibromatosis, plantar fibromatosis, knuckle pads, and Peyronie's disease. Desmoid fibromatoses are divided into extra-abdominal desmoids, abdominal wall desmoids, intra-abdominal desmoids, multiple desmoids, multiple familial desmoids, and desmoids in Gardner's syndrome. The juvenile fibromatoses include congenital fibrosarcoma-like fibromatosis, congenital generalized fibromatosis, congenital localized fibromatosis, fibromatosis colli, diffuse infantile fibromatosis, juvenile aponeurotic fibroma, fibrous hamartoma of infancy, recurring digital fibrous tumor of childhood, juvenile nasopharyngeal angiofibroma, hereditary gingival fibromatosis, and fibromatosis hyalinica multiplex juvenilis. A total of 140 fibromatoses seen at the Armed Forces Institute of Pathology, (U.S.A) the Australian Soft Tissue Tumor Registry, and the Institute of Medical and Veterinary Science, Adelaide, are classified in this way and their clinicopathological characteristics are described. Two conclusions are drawn from the study: 1. Surgery is generally the only effective way of treating the fibromatoses, but operations may sometimes cause more morbidity than the tumors. 2. Amputation of a limb is rarely justifiable in the treatment of the fibromatoses.
Im Frühstadium des Morbus Dupuytren wird die externe Radiotherapie mit dem Ziel eingesetzt, den progressiven Verlauf der Erkrankung zu verhindern. Eine aktuelle Langzeitverlaufskontrolle soll die Ergebnisse und Nebenwirkungen der Radiotherapie darstellen.
Patienten und Methode: Wir untersuchten 99 Patienten (176 Hände), welche sich von 1982–1994 einer Radiotherapie an unserer Klinik unterzogen. Jeder Patient erhielt zwei Serien einer Radiotherapie mit jeweils 5 × 3 Gy (Gesamtdosis 30 Gy, 120 kV, 4 mm Al, Bestrahlungspause von 6–8 Wochen nach 15 Gy). Die Beugekontraktur wurde nach Tubiana et al. eingeteilt. Von Juli bis November 1999 erfolgte nach einer medianen Nachbeobachtungszeit von 10 Jahren (7–18 Jahre) eine Kontrolluntersuchung. Die Nebenwirkungen wurden nach den LENT-SOMA-Kriterien eingestuft.
Ergebnisse: Im Stadium N blieben 84% und im Stadium N/I 67% stabil oder zeigten eine Regression. Dagegen stieg ab dem Stadium I die Progressionsrate auf 65% und im Stadium II auf 83% an. Eine “Salvage”-Operation erfolgte bei 29 Patienten, ohne dass Wundheilungsstörungen beobachtet wurden.
Schlussfolgerung: Die Radiotherapie stellt in den Frühstadien (Stadium N, N/I) der Dupuytren'schen Kontraktur auch langfristig eine effektive und sichere Methode dar. Bei Therapieversagern ist eine spätere Operation möglich.
DC is a slow fibrotic process of the palmar fascia usually beginning in middle age in the form of small nodules at the distal ulnar aspect of one or both palms, gradually resulting in a permanent contracture of one or more fingers. The incidence varies between 1% and 3% of whites, and is six to ten times more frequent in males than in females. The etiology is unknown. Heredity certainly plays an important role, while local or distant trauma, neurogenic, and metabolic factors may be of significance in the development of this condition. Alcoholism, diabetes mellitus, and epilepsy are often associated with this moderately disabling ailment. Similar conditions of the plantar fascia may be seen isolated or concomitantly with DC of the hand. The natural history of DC varies greatly from one individual to another. Successful conservative treatment is possible only at the very beginning of the disease. Subsequently, various surgical methods should be considered. Whatever the method, recurrences are possible, especially in young people.
Purpose: In early stage Dupuytren's contracture radiotherapy was applied to prevent disease progression. Long-term results and late toxicity of this treatment were evaluated in a retrospective analysis. Patients and Methods: Between 1982 and 1994, 99 patients (176 hands) received orthovoltage radiotherapy, which consisted of two courses with 5 × 3 Gy (total dose: 30 Gy, daily fractionated; 120 kV, 4 mm Al), separated by a 6 to 8-week pause. The Dupuytren's contracture was staged according to the classification of Tubiana et al. The long-term outcome was analyzed at last follow-up between July and November 1999. The median follow-up was 10 years (range 7-18 years). Late toxicity was assessed using the LENT-SOMA criteria. Results: In Stage N 84% and Stage N/I 67% of cases remained stable. 65% of the cases in Stage I and 83% in Stage II showed progressive nodules and cords. In case of progression we saw no complications after a second radiotherapy or salvage operation. Conclusion: Radiotherapy effectively prevents disease progression for early stage Dupuytren's contracture (Stage N, N/I). Moreover, in case of disease progression despite radiotherapy salvage surgery is still feasible.
The method for classification of Dupuytren's disease, established by R. Tubiana, J. Michon and J.M. Thomine, which translates ray by ray the localization and intensity of lesions, was partly modified and completed. These modifications give a more precise evaluation of the thumb and first web space deformations, indicate the skin's state, eventual post-operative stiffness, and provide numbers which can be used in computers. The pre-operative classification described in the first half of this article is very simple and practical, and may be used effectively by all physicians who are presented with Dupuytren's disease. Supplementary indicators in the second half of the paper provide further precision which permits objective judgement of operative results. They are especially useful to surgeons who desire to review their observations and to establish statistics.
The normal anatomy of the palmar fascia and histopathological changes occurring in Dupuytren's contracture are briefly described. The presenting signs are reviewed in 34 cases and correlated with these pathological and anatomical changes.
The results of treatment by surgery alone are given and reasons for overall unsatisfactory outcome discussed. Two techniques of radiation treatment are described as well as the combined orthopaedic-radiotherapy policy practised.
Roughly half the cases treated by radiotherapy alone obtain a satisfactory result. A combination of surgery and pre-operative radiotherapy is suggested for late cases, thereby lessening the chance of recurrence.
The proliferating cells in fibromatoses are myofibroblasts that produce abundant stromal collagen and contain intracellular native and widely spaced collagen fibers. To assess the clinical and cellular effects of colchicinein such tumors, this drug was administered to three patients, one with musculoaponeurotic desmoid fibromatosis, one with Dupuytren's palmar fibromatosis, and one with Peyronie's disease. All three patients had an excellent clinical response, with reduction of tumor size and improvement of contracture. Two cases were studied ultrastructurally; the main cellular changes detected were collapse of the rough endoplasmic reticulum cisternae, reduction of myofilaments, and disappearance of intracellular widely spaced collagen. The findings from this study indicate another probable application for colchicine and support the concept that collagen fibers can be formed intracellularly. Cancer 1992; 69:2478-2483.
Fifty-eight patients (52 males and 6 females) operated on for Dupuytren contracture were examined by the same author with a more than ten year follow-up. At time of surgery the average was 55 years old. 69 hands (169 fingers) rated 4.33 according to the simplified Tubiana's score were treated by the same operative procedure : Mac Indoe's incision, digital Z plasty (if needed), subtotal fasciectomy and physiotherapy beginning 8 days postoperatively. At long term, recurrence appears for 49 hands (71 %) one every two in the two first postoperative years, one out of five after five years. 24 of them were graded stage I. The recurrence appeared 14 times associated with an extension of the disease and the earlier, the higher was the initial stage. Some factors seem to be of a bad prognosis regarding recurrence : age (93 % of recurrence under 50 years old) Ledderhose or Lapeyronic (100 %) other associated diseases (Alcoholism, diabetus melitus, epilepsy) and severe preoperative stage. Subjective results are good : 45 patients are satisfied and only 3 underwent a second operation.
Dupuytren's contracture (DC) is a disease of the palmar fascia resulting in thickening and contracture of fibrous bands on the palmar surface of the hands and fingers. For decades, a controversy has existed regarding whether acute traumatic injury or cumulative biomechanical work exposure can contribute to the development of this disorder. To address this controversy, this review considers the following questions: Is there evidence that DC is associated with 1) frequent or repetitive manual work; and 2) hand vibration? The published literature was searched for studies meeting the following criteria: 1) in English or having an English abstract; 2) controlled studies; 3) DC an identified health outcome studied; and 4) the study group exposed to repetitive or frequent manual work, vibration, or acute traumatic injury. Relevant non-English articles identified through English abstracts were translated. The validity of studies meeting the selection criteria was assessed using a series of questions adapted from those of Stock [1991: Am J Ind Med 19:87–107]. Studies that met a priori minimum levels of methodologic quality were taken into account to reach conclusions with respect to the above questions. Odds ratios (ORs) and 95% confidence intervals (CIs) were calculated for each study. Ten studies met the initial selection criteria. Of these, four studies met the criteria for methodologic quality, one addressing the relationship between manual work and DC, and three studies of vibration and DC. No controlled studies of acute trauma and DC were identified. Bennett [1982: Br J Ind Med 39:98–100] found the prevalence of DC at a British PVC bagging and packing plant in which workers were exposed to repetitive manual work to be 5.5 times that at a local plant without packing, and twice the expected prevalence in a U.K. working population previously studied by Early [1962: J Bone Joint Surg 44B:602–613]. DC was observed more frequently among vibration white finger claimants than controls by Thomas and Clarke [1992: J Soc Occup Med 42:155–158] (OR. 2.1; 95% CI, 1.1–3.9), and more frequently among vibration-exposed workers than controls by Bovenzi et al. [1994: Occup Environ Med 51:603–611] (OR, 2.6; 95% CI, 1.2–5.5). Cocco et al. [1987: Med Lav 78:386–392] found that a history of vibration exposure occurred more frequently among cases of DC than among controls (OR, 2.3; 95% CI, 1.5–4.4). The latter two studies presented some evidence of a dose-response relationship. There is good support for an association between vibration exposure and DC. © 1996 Wiley-Liss, Inc.
Die klassische “Handerkrankung” des Nordens ist der Morbus Dupuytren: heimgesucht werden keltische oder Wikinger-Nachfahren in ganz Nordeuropa, während das Krankheitsbild im Mittelmeerraum unbekannt ist. Nur vordergründig scheint der Morbus Dupuytren ein extremitätenchirurgisches Problem zu sein. Bei unbekannter Ätiologie und Mangel an aktuellen, bevölkerungsspezifischen Daten für Norddeutschland zielt diese Studie auf die Erhellung gleichzeitig bestehender Grunderkrankungen ab, um darüber und gemeinsam mit der Literatur einen pathogenetischen Erklärungsversuch zu unternehmen. 566 Dupuytren-Patienten aus dem Raum um Hannover wurden retrospektiv hinsichtlich epidemiologischer Daten und des Tubiana-Kontrakturstadiums untersucht: 91,2% waren norddeutscher Abstammung, 12,5% hatten eine familäre Prädisposition. Mit einem Geschlechterverhältnis von 7:1 erkrankten bevorzugt Männer. Der Erkankungsgipfel lag im 6. Lebensjahrzehnt. Signifikant unterschieden sich die Tubiana-Werte für Intellektuelle gegenüber Schwerarbeitern. Ipsilaterale Extremitätenverletzungen boten 15%. Bei 55,1% bestand eine bilaterale Kontraktur. Ektope Bindegewebsdepots in Penis, Fußsohle oder als Fingerknöchelspolster zeigten 6,7%. Auf die Stadien I–IV entfielen absteigend 59,1–2,4% der 1808 erkrankten Fingerstrahlen. Mit 3,7–3,72 zeigten Trinker und Raucher signifikant schwerwiegendere, dagegen die 8,2% Diabetiker eine mildere Form der Dupuytren-Kontraktur. Unter den 1,3% Epileptikern, alle mit beidseitiger Flexionskontraktur, überstieg der Tubiana-Wert von 3,71 das mittlere händische Erkrankungsstadium von 3,63.
Unter Dupuytren-Kontraktur (DK; Morbus Dupuytren, “Dupuytren’s disease”) versteht man eine Erkrankung der kollagenen Faserbündel
des straffen Bindegewebssystems der Palmarseite der Hand. Es kommt zu einer Fibrose mit Knotenbildung und Umbauvorgängen,
in deren Verlauf sich eine Verkürzung der Faserbündel mit einer Kontraktur der betroffenen Gelenke entwickelt.
Fibrosarcomas make up a small percentage of son somatic tissue malignancies. Fibrosarcomas of the foot and ankle constitute an even smaller percentage of all reported cases with respect to anatomic locations. The postoperative local recurrence is great, and the diagnosis is often difficult to make because of the similar microscopic presentation of plantar fibromatosis and fibrosarcoma. A case presentation of a plantar fibrosarcoma, after two failed attempts at excision, with a review of the literature, is discussed.
On overview is present which briefly summarizes the incidence, pathology and natural progress of Dupuytren's Disease. The importance of recognizing the Dupuytren diathesis in planning the surgical treatment, the importance of conservatism in initiating surgery, and the principles of surgical management including dermofasciectomy are presented, and it is once again stressed that a distinction exists between recurrence and extension of Dupuytren's Disease post-operatively.RésuméUne vue d'ensemble de l'incidence, de l'anatomopathologie et de l'évolution spontanée de la maladie de Dupuytren est donnée. L'importance du diagnostic de la diathèse de Dupuytren pour établir le programme du traitement chirurgical, la nécessité d'une attitude chirurgicale conservatrice, et les principes du traitement chirurgical qui comprend la dermofasciectomie sont présentés. La différence qui existe entre récidive et extension post-opératoires de la maladie de Dupuytren, est à nouveau soulignée.
Limited indications for radiation treatment of benign dermatoses are presented in order to emphasize modern restrictions and techniques in the use of dermatologic radiation therapy and to promote measures of protection against stray radiation. The use of softer, less penetrating X rays, least effective doses and meticulous shielding are recommended. So done, radiation therapy offers an effective therapeutic alternative for conditions that do not respond to other forms of treatment.
The relative proportion of collagen type I and type III in the aponeurosis of twenty-four patients with Dupuytren's disease was determined and compared with the aponeurosis of normal persons. The presence of considerable amounts of type III collagen was found in the Dupuytren's disease patients. The sera of the patients were screened for circulating anti-collagen antibodies using a sensitive radioimmunoassay. In seven out of the twenty-four patients low concentrations of these antibodies were found.
There are at least 11 distinctive fibromatoses which occur predominantly or exclusively in patients less than 20 years of age. Each group is considered separately.