Dupuytren's disease

Medical Information

Synonyms

Dupuytren's contracture, palmar fibromatosis

Etiology

Genetic component through single nucleotide polymorphisms (SNPs) alterations, with autosomal dominant inheritance and variable penetrance.

Symptoms

Palmar fibromatosis with initial nodule formation, eventually followed by cords that can lead to a flexion contracture, especially when crossing a finger joint, which gives Dupuytren's contracture its name. The ring and little fingers in the palm and along the plane of the long fingers are most frequently affected. These nodules/cords can also cause pain, likely due to the growth of cutaneous nerve fibers within the fibrotic nodules, generating corresponding compression. In addition to clinical findings by palpation, imaging—particularly ultrasound—can help rule out benign or malignant tumors as a differential diagnosis.

Diagnostic Imaging

Ultrasonographically, Dupuytren's nodules located superficially in the subcutaneous tissue usually appear hypoechoic, although they are sometimes isoechoic compared to the surrounding subcutaneous fat (Knobloch, 2012). New ultra-high resolution power Doppler techniques suggest that vascularization could be a potential indicator of activity (Knobloch, 2022). The T2 signal in magnetic resonance imaging could indicate Dupuytren's nodule activity as a biomarker and hold prognostic value for the success of radiotherapy (Banks et al., 2018).

Therapy

From a therapeutic standpoint, a distinction must be made between the nodular stage (Tubiana N) and the cord stage with joint contracture of the finger.

In the nodular stage, for symptomatic painful nodules and the resulting discomfort, the following treatment options may be offered:

• High-energy focal shock wave therapy (typically three sessions separated by 1–2 weeks, with a 6-month follow-up for reinforcement; Knobloch et al., 2012; 2022).

• Shock wave therapy improves pain and patient satisfaction more effectively than stretching or laser therapy at 1, 2, and 3 months, with no side effects (Notarnicola, 2017).

• Radiotherapy to slow the progression of Dupuytren's disease (Banks, 2018; Rödel, 2017; Seegenschmiedt, 2015).

• A clinical case of a 79-year-old patient showed improvement in hand function with radial shock wave therapy (3 bar, 12 Hz, 1400 pulses) over four sessions to treat the contracture (Brunelli et al., 2020).

• In the largest randomized study on shock wave therapy for Dupuytren's disease to date (DupuyShock study, Knobloch et al., 2022), 52 patients with a mean age of 58±9 years with painful Dupuytren's nodules in the nodular stage (Tubiana N) were included. The intervention group received three sessions of high-energy electromagnetic focal shock wave therapy (Storz Ultra, 2000 pulses, 3 Hz, up to 0.35 mJ/mm², 49 mJ/mm² per hand), compared to a placebo group.

The pain was significantly reduced by 54% in the intervention group at 3, 6, and 12 months. Similarly, scores on patient-oriented scales, such as the DASH, the Michigan Hand Questionnaire, and the URAM scale, improved significantly in favor of the treated group. No side effects were observed.

Analogously, positive effects on pain reduction have been reported using focal ESWT in the nodular form of Ledderhose disease (Knobloch K, 2012; Hwang et al., 2020), which affects the sole of the foot, and in Peyronie's disease of the penis (Porst H, 2021; Krieger et al., 2019).

Radiotherapy in the nodular stage of Dupuytren's disease was evaluated in a cohort study with 135 patients and 208 symptomatic hands treated by orthovoltage irradiation at 30 Gy. With a 13-year follow-up, nodules remained stable in 59% of cases, improved in 10%, and progressed in 31%.

In the cord stage, with joint contracture exceeding 20°, classic therapeutic procedures for Dupuytren's disease include:

• Open surgery via selective fasciectomy

• Percutaneous needle fasciotomy (PNF)

• Enzymatic fasciotomy with collagenase (Xiapex) has no longer been available outside the United States since 2019.

• High-energy focal shock wave therapy may have additional positive effects on wound healing, edema reduction, and, if necessary, recurrence prophylaxis as a complementary treatment before and immediately after the aforementioned procedures.

Shock wave therapy for Dupuytren's disease

• Indication: Indication performed by a specialist physician.

• Contraindication: Malignant tumor in the treatment focus.

• Facility requirements: Certification criteria for a medical practice, e.g., hygiene plan, emergency management according to DIN standards.

• Patient preparation: Differentiated and documented information and education.

• Medical and assistant staff: ESWT must be performed personally by a physician with the corresponding qualifications.

Banks JS, Wolfson AH, Subhawong TK. T2 signal intensity as an imaging biomarker for patients with superficial fibromatoses of the hands (Dupuytren's disease) and feet (Ledderhose disease) undergoing defninitive electron beam irradiation. Skeletal Radiol 2018;47(2):243-51.

Brunelli S, Bonanni C, Traballesi M, Foti C. Radial ESWT: a novel approach for the treatment of Dupuytren's contractures: a case report. Medicine (Baltimore) 2020;99(24):e20587.

Hwang JT, Yoon KJ, Park CH, Choi JH, Park HJ, Park YS, Lee YT. Follow-up of clinical and sonographic features after ESWT in painful plantar fibromatosis. PLoS One 2020;15(8):e0237447.

Knobloch K, Kühn M, Vogt PM. Focused high-energy shock wave therapy improves quality of life in Dupuytren's disease-a randomized trial (DupuyShock). 43rd Annual Meeting of the German Society of Plastic, Reconstructive and Aesthetic Surgery (DGPRÄC), 2012, Bremen. doi: 10.3205/12dgpraec191

Knobloch K, Kühn M, Vogt PM. Focused high-energy shock wave therapy for palmar nodularity in Dupuytren's disease-a randomized trial (DupuyShock). 53rd Congress of the German Society for Hand Surgery 2012 Lübeck. doi: 10.3205/12dgh05

Knobloch K, Kühn M, Sorg H, Vogt PM. German version of the Unite rhumatologique des affections de la main (URAM) scale in Dupuytren's disease: the need for a uniform definition of recurrence. Arthritis Care Res (Hoboken) 2012:64(5):793.

Knobloch K, Vogt PM. High-energy focused ESWT reduces pain in plantar fibromatosis (Ledderhose's disease) BMC Res Notes 2012;5:542.

Knobloch K, Kuehn M, Papst S, Kraemer R, Vogt PM. German standardized translation of the michigan hand outcomes questionnaire for patient-related outcome measurement in Dupuytren disease. Plast Reconstr Surg. 2011 Jul; 128 (1): 39e-40e. doi: 10.1097/PRS.0b013e318218fd70.

Knobloch K, Kuehn M, Vogt PM. Focused extracorporeal shockwave therapy in Dupuytren's disease--a hypothesis. Med Hypotheses. 2011 May;76(5):635-7. doi: 10.1016/j.mehy.2011.01.018. Epub 2011 Feb 1.

Knobloch K. From nodules to chords in Dupuytren's contracture. MMW Fortsch Med 2012;154(19):36.

Knobloch K, Redeker J, Vogt PM. Antifibrotic medication using a combination of N-acetyl- L-cysteine (NAC) and ACE inhibitors can prevent the recurrence of Dupuytren's disease. Med Hypotheses. 2009 Nov;73(5):659-61. doi: 10.1016/j.mehy.2009.08.011. epub 2009 Sep 1.                                   

Knobloch K, Hellweg M, Sorg H, Nedelka T. Focused electromagnetic high-energetic ESWT reduces pain levels in the nodular state of Dupuytren's disease - a randomized controlled trial (DupuyShock). Laser Med Sci 2022;37(1):323-333.

Krieger JR, Rizk PJ, Kohn TP, Pastuszak A. Shockwave therapy in the treatment of Peyronie's disease. Sex Med Rev 2019;7(3):499-507.

Notarnicola A, Maccagnano G, Rifino F, Pesce V, Gallone MF, Covelli I, Moretti B. Short- term effect of shockwave therapy, temperature controlled high energy adjustable multi- mode emission laser or stretching in Dupuytren's disease: a prospective randomized clinical trial. J Biol Regul Homeost Agents 2017;31(3):775-84.

Porst H. Review of the current status of low intensity ESWT in erectile dysfunction (ED), Peyronie's disease (PD), and sexual rehabilitation after radical prostatectomy with special focus on technical aspects of the different marketed ESWT devices including personal experiences in 350 patients. Sex Med Rev 2021;9(1):93-122.

Rödel F, Fournier C, Wiedemann J, Merz F, Gaipl US, Frey B, Keilholz L, Seegenschmiedt MH, Rödel C, Hehlgans S. Basis of radiation biology when treating hyperproliferative benign diseases. Front Immunol 2017;8:519.

Seegenschmiedt MH, Micke O, Niewald M, Mücke R, Eich HT, Kriz J, Heyd R, German Cooperative Group on Radiotherapy of benign diseases. DEGRO guidelines for the radiotherapy of non-malignant disorders: part III: hyperproliferative disorders. Strahlenther Onkol 2015;191(7):541-8.