Dupuytren’s Disease

Dupuytren’s disease is a common hand condition that affects the connective tissue beneath the skin of the palm and fingers. It is characterized by the development of thick cords and nodules, which can cause the fingers to bend inward over time. In this article, we will explore the definitions and types of Dupuytren’s disease in simple language, providing a comprehensive understanding of this condition.

Dupuytren’s disease, also known as Dupuytren’s contracture, is a progressive hand condition that primarily affects the connective tissue called the palmar fascia. The palmar fascia lies just beneath the skin of the palm and plays a crucial role in maintaining the shape and flexibility of the hand.

In individuals with Dupuytren’s disease, the palmar fascia thickens and forms nodules, which eventually develop into fibrous cords. These cords can tighten over time, causing the fingers to curl inward or contract. The condition typically affects the ring finger and little finger, but can also involve other fingers or the thumb in some cases.

Types of Dupuytren’s Disease:

1. Classical Dupuytren’s Disease: Classical Dupuytren’s disease is the most common type and primarily affects the palm and fingers. It usually progresses slowly, and symptoms may appear gradually. Thickened cords and nodules form beneath the skin, leading to finger contractures. The disease can be unilateral (affecting only one hand) or bilateral (affecting both hands).
2. Dupuytren’s Diathesis: Dupuytren’s diathesis refers to a genetic predisposition to develop Dupuytren’s disease. People with this type have a family history of the condition and may have multiple affected family members. They may develop symptoms at a younger age and have a more aggressive disease course.
3. Dupuytren’s Disease with Proximal Interphalangeal (PIP) Joint Involvement: In some cases, Dupuytren’s disease can affect the proximal interphalangeal joint, which is the joint closest to the palm in the fingers. When the PIP joint is involved, it can result in a more severe contracture, limiting finger movement.
4. Dupuytren’s Disease with Metacarpophalangeal (MCP) Joint Involvement: This type of Dupuytren’s disease affects the metacarpophalangeal joint, which is the joint between the palm and the base of the finger. Involvement of the MCP joint can lead to significant hand deformities and functional impairment.

Causes

Possible causes of Dupuytren’s disease and provide a comprehensive understanding of this condition.

1. Genetic Predisposition: Dupuytren’s disease has a strong genetic component, with certain genes being associated with an increased risk of developing the condition. Family history plays a significant role, and individuals with a family member affected by Dupuytren’s disease are more likely to develop it themselves.
2. Ethnicity: The condition is more prevalent among individuals of Northern European descent, particularly those with Viking ancestry. Genetic variations in these populations may contribute to the increased susceptibility to Dupuytren’s disease.
3. Age: Dupuytren’s disease is commonly seen in individuals over the age of 50. While it can affect younger individuals, the risk significantly increases with age.
4. Gender: Men are more prone to developing Dupuytren’s disease compared to women. However, women with a family history of the condition have an elevated risk.
5. Hormonal Factors: Hormonal imbalances, particularly low testosterone levels in men, may be associated with an increased risk of developing Dupuytren’s disease. However, more research is needed to establish a definitive link.
6. Diabetes: People with diabetes have a higher likelihood of developing Dupuytren’s disease. The exact mechanisms behind this association are not yet fully understood.
7. Autoimmune Disorders: Some autoimmune disorders, such as rheumatoid arthritis, have been linked to an increased risk of Dupuytren’s disease. The immune system’s abnormal response may trigger the formation of Dupuytren’s nodules.
8. Hyperlipidemia: High levels of cholesterol and triglycerides in the blood, a condition known as hyperlipidemia, have been

Symptoms

Common symptoms of Dupuytren’s disease, explaining each in detail to raise awareness and promote better understanding of this condition.

1. Nodule Formation: One of the initial signs of Dupuytren’s disease is the formation of small, painless nodules or lumps under the skin of the palm. These nodules are usually firm to the touch and may appear near the base of the ring or little fingers. Over time, the nodules may grow larger and become more noticeable.
2. Thickened Cords: As Dupuytren’s disease progresses, the nodules can develop into thickened cords or bands of tissue that extend from the palm into the fingers. These cords are often tender and can cause discomfort or pain. They may limit finger movement and gradually lead to finger contracture.
3. Finger Contracture: Finger contracture is a common symptom of advanced Dupuytren’s disease. It occurs when the thickened cords progressively tighten, causing the affected fingers to bend or curl inward. Initially, the contracture may only be noticeable when attempting to fully extend the fingers, but it can worsen over time and affect daily activities.
4. Difficulty Straightening Fingers: Individuals with Dupuytren’s disease may experience difficulty in straightening their affected fingers, especially after extended periods of rest or immobility. This symptom often accompanies finger contracture and can lead to decreased hand function and impaired grip strength.
5. Pitting of the Skin: In some cases, Dupuytren’s disease may cause the overlying skin to develop small, shallow pits or depressions. These pits are often seen in conjunction with nodules and cords and can contribute to the characteristic appearance of the affected hand.
6. Dimpling of the Skin: Similar to pitting, dimpling of the skin is another visible symptom of Dupuytren’s disease. Dimpling occurs when the affected skin becomes indented or puckered, creating small folds or creases. This manifestation is often observed in areas where cords are prominent.
7. Skin Thickening: Dupuytren’s disease can cause the skin over the affected areas to thicken and feel tougher than normal. The skin may lose its elasticity and appear shiny or waxy. This symptom can be attributed to the excessive collagen production that occurs in the connective tissues affected by the disease.
8. Hand Deformity: As the condition progresses, Dupuytren’s disease can result in significant hand deformity. The affected fingers may gradually pull toward the palm, leading to a claw-like appearance. This deformity can impede hand function, making it difficult to perform everyday tasks.
9. Restricted Finger Mobility: Dupuytren’s disease can restrict the range of motion in the affected fingers. Individuals may find it challenging to fully extend or flex their fingers. As the disease advances, this restriction can worsen, limiting hand functionality and causing difficulties with activities such as grasping objects or shaking hands.
10. Pain or Discomfort: Although pain is not a characteristic symptom of Dupuytren’s disease, some individuals may experience mild discomfort or aching in the affected hand.
11. Finger Stiffness: One of the initial signs of Dupuytren’s disease is stiffness in the fingers, making it challenging to straighten them fully.
12. Nodule Formation: Small, firm lumps or nodules may develop under the skin of the palm. These nodules are usually painless and may be the first visible sign of the condition.
13. Skin Puckering: As Dupuytren’s disease progresses, the skin on the palm may begin to pucker or dimple, giving it an uneven appearance.
14. Thickened Palmar Fascia: The tissue beneath the skin in the palm, known as the palmar fascia, may become thickened and may feel like cords under the skin.
15. Finger Contracture: One of the defining symptoms of Dupuytren’s disease is the progressive bending of the fingers towards the palm, limiting their range of motion.
16. Difficulty Straightening Fingers: Over time, it becomes increasingly challenging to extend the affected fingers fully.
17. Inability to Lay Hand Flat: The hand affected by Dupuytren’s disease may no longer be able to lay flat on a surface due to the contracture of the fingers.
18. Finger Flexion: The fingers may remain flexed, making it difficult to grasp objects or perform fine motor tasks.
19. Finger Curling: In severe cases, the fingers can curl so tightly that they become fixed in a permanently bent position.
20. Pain: Although pain is not a common symptom of Dupuytren’s disease, some individuals may experience discomfort or tenderness in the affected area.
21. Hand Weakness: As the condition progresses, hand weakness may occur, making it harder to grip objects firmly.
22. Limited Dexterity: Decreased finger mobility and flexibility can result in reduced dexterity, affecting activities that require precise finger movements.
23. Thumb Involvement: In some cases, the thumb may be affected by Dupuytren’s disease, leading to thumb contracture and limited thumb movement.
24. Difficulty Wearing Gloves: The contracture and thickened tissue in the palm can make it challenging to wear gloves or other hand accessories.
25. Difficulty Putting Hands in Pockets: Similarly, the reduced mobility in the fingers may make it difficult to put hands in pockets or perform tasks that require hand insertion.
26. Hand Fatigue: The constant effort required to compensate for the finger contracture can cause fatigue and discomfort in the hand.
27. Hand Deformity: Over time, the progressive bending of the fingers and the thickening of the palm tissue can lead to hand deformities.
28. Difficulty Shaking Hands: The inability to extend the fingers fully can make it awkward or difficult to shake hands with others.
29. Difficulty Washing Hands: Washing hands thoroughly may become challenging due to the limited finger movement caused by the contracture.
30. Difficulty Holding Objects: Grasping and holding objects, especially smaller items, can become increasingly difficult as the fingers lose their mobility.
31. Finger Numbness: In rare cases, some individuals with Dupuytren’s disease may experience tingling or numbness in the affected fingers.

Diagnosis

Common diagnosis and tests used to identify Dupuytren’s disease,

1. Medical History: The first step in diagnosing Dupuytren’s disease is taking a thorough medical history. This includes discussing symptoms, their progression, family history of the disease, and any other relevant information.
2. Physical Examination: A physical examination of the hands and fingers helps identify characteristic signs of Dupuytren’s disease. The physician will look for thickened bands of tissue, nodules, and finger contractures.
3. Finger Extension Test: During the physical examination, the physician will perform a finger extension test. This involves assessing the ability of the affected finger(s) to fully straighten. Difficulty or limited range of motion indicates Dupuytren’s disease.
4. Tabletop Test: The tabletop test assesses the palm’s ability to lie flat on a table when the hand is placed palm-down. In individuals with Dupuytren’s disease, the affected hand may have an inability to lie flat due to contractures.
5. Imaging Tests: X-rays, ultrasound, or MRI scans may be ordered to evaluate the extent of tissue involvement and determine the severity of the disease.
6. X-ray: X-rays can help identify calcium deposits in the hand and evaluate the alignment of the affected joints. They are useful in assessing the progression of the disease and planning treatment.
7. Ultrasound: Ultrasound imaging uses sound waves to visualize the structures within the hand. It can help identify the presence of nodules, and thickened bands, and measure the thickness of affected tissues.
8. Magnetic Resonance Imaging (MRI): MRI provides detailed images of the soft tissues in the hand. It can help identify the location and extent of tissue involvement, allowing for accurate diagnosis and treatment planning.
9. Blood Tests: While there is no specific blood test for Dupuytren’s disease, certain blood markers may be evaluated to rule out other conditions that can cause similar symptoms.
10. Genetic Testing: Genetic testing is not commonly used to diagnose Dupuytren’s disease. However, in some cases, it may be recommended, especially in individuals with a family history of the condition.
11. Collagenase Injection Test: Collagenase injection is a test and treatment combined. An enzyme called collagenase is injected into the Dupuytren’s cord, which helps break down the thickened tissue. The response to the injection helps determine the severity and suitability of this treatment option.
12. Hand Function Assessment: Assessing hand function and grip strength is important to understand the impact of Dupuytren’s disease on daily activities. It helps guide treatment decisions and measure treatment outcomes.
13. Tubiana Staging System: The Tubiana staging system is a classification system used to determine the severity of Dupuytren’s disease. It considers the presence of nodules, cord formation, and finger contractures, helping to guide treatment decisions.
14. DASH Score: The Disabilities of the Arm, Shoulder, and Hand (DASH) score is a questionnaire that evaluates the functional limitations and disability caused by Dupuytren’s disease. It provides a standardized measurement of hand impairment.
15. The Angle of Deformity Measurement: Measuring the angle of finger contracture provides a quantitative assessment of the severity of Dupuytren’s disease. It helps monitor disease progression and determine

Treatment

From non-surgical options to advanced surgical procedures, we will discuss each treatment in detail to help you understand the available options and make informed decisions.

1. Observation and Monitoring: In the early stages of Dupuytren’s disease, where symptoms are mild, doctors may adopt a watchful waiting approach. Regular monitoring of the condition allows healthcare professionals to track the progression of the disease and determine if treatment is necessary.
2. Hand Exercises: Hand exercises and stretching can help maintain finger mobility and prevent further contracture. Physical or occupational therapists can guide patients through specific exercises tailored to their needs, which may include finger extensions, grip strengthening, and range-of-motion exercises.
3. Splinting: Wearing a splint or brace can aid in maintaining finger extension and slowing the progression of contractures. Splinting is often recommended for nighttime use to counteract the curling of the fingers during sleep.
4. Radiation Therapy: Low-dose radiation therapy has shown promise in reducing Dupuytren’s disease progression. The treatment involves delivering targeted radiation to the affected tissues, which can help slow down the growth of nodules and cords.
5. Needle Aponeurotomy: Needle aponeurotomy, also known as percutaneous needle fasciotomy, is a minimally invasive procedure that involves using a needle to puncture and release the contracted cords. This technique can provide temporary relief by breaking up the fibrous tissue and improving finger mobility.
6. Collagenase Injection: Collagenase injection is an FDA-approved non-surgical treatment that involves injecting an enzyme called collagenase into the contracted cord. The enzyme breaks down the collagen fibers, allowing the cord to be manipulated and straightened.
7. Enzyme Injections: Apart from collagenase, other enzymes like hyaluronidase and clostridial collagenase are being explored as alternative injection therapies. These enzymes help break down the collagen fibers, loosening the cords and improving finger extension.
8. Steroid Injections: Corticosteroid injections into the nodules can help reduce inflammation and soften the tissue. This treatment option may be considered to alleviate symptoms and slow down the progression of Dupuytren’s disease.
9. Thermotherapy: Thermotherapy involves applying heat to the affected hand using warm water soaks, paraffin wax baths, or microwave diathermy. Heat can help relax the tissues, reduce pain, and improve finger mobility.
10. Ultrasound Therapy: Ultrasound therapy utilizes high-frequency sound waves to promote healing and decrease the size of nodules. This non-invasive treatment may be used in conjunction with other therapies to manage Dupuytren’s disease.
11. Steroid Injections with Needle Aponeurotomy: Combining steroid injections with needle aponeurotomy can enhance the effects of both treatments. Steroids reduce inflammation, making it easier to manipulate the cords during the procedure.
12. Extracorporeal Shockwave Therapy: Extracorporeal shockwave therapy involves the delivery of acoustic waves to the affected hand. This treatment can break down the fibrous tissue, improve blood flow, and potentially slow down the progression of the disease
13. Medications: Certain medications, such as collagenase clostridium histolyticum (CCH), can be injected directly into the cords of the affected fingers. These injections help break down the excessive collagen that causes the contraction, facilitating improved finger extension.
14. Steroid Injections: Corticosteroid injections into the cords can help reduce inflammation and provide temporary relief from Dupuytren’s disease symptoms. These injections can help alleviate pain and slow down the progression of the disease.
15. Needle Aponeurotomy: Also known as percutaneous needle fasciotomy, this minimally invasive procedure involves using a needle to divide and release the contracted cords in the hand. This technique is effective in improving finger extension and can be performed in an outpatient setting.
16. Xiaflex Injections: Xiaflex is a medication derived from collagenase that is injected directly into the contracted cords. It helps break down the collagen, allowing for improved finger mobility. Xiaflex injections are typically administered in a series of treatments.
17. Surgical Fasciectomy: Surgical fasciectomy is a more invasive procedure that involves removing the thickened tissue (fascia) causing the finger contracture. It can be performed as a partial fasciectomy, removing only the affected part, or as a complete fasciectomy, removing all the diseased tissue.
18. Needle Fasciotomy: Similar to needle aponeurotomy, needle fasciotomy involves using a needle to cut the contracted cords. This procedure is less invasive than surgery and can be performed under local anesthesia.
19. Percutaneous Needle Fasciotomy (PNF): PNF is a technique that involves using a needle to puncture and disrupt the contracted cords. It is a minimally invasive procedure that can improve finger extension and function.
20. Enzyme Injection: Enzyme injections, such as collagenase, can be used to break down the contracted tissue, allowing for improved finger mobility. This treatment option is generally reserved for milder cases of Dupuytren’s disease.

Medications

Treatment option in simple, easy-to-understand language.

1. Collagenase Clostridium histolyticum – Collagenase Clostridium histolyticum, or CCH, is an FDA-approved injectable enzyme that breaks down the excess collagen responsible for the finger contractures in Dupuytren’s disease. It is administered directly into the affected tissue and helps restore finger mobility.
2. Steroid Injections – Steroid injections are commonly used to reduce inflammation and alleviate pain associated with Dupuytren’s disease. They can help manage symptoms and delay the need for surgery.
3. Verapamil –  Verapamil, a calcium channel blocker, has shown promise in reducing the progression of Dupuytren’s disease. It is believed to inhibit the production of excess collagen, thereby preventing the development of contractures.
4. Interferon – Interferon, a naturally occurring protein, can be injected into the nodules of Dupuytren’s disease. It has been found to reduce collagen production and slow down the progression of the disease.
5. Bleomycin – Bleomycin is an antibiotic that has been repurposed for the treatment of Dupuytren’s disease. When injected into the affected tissue, it breaks down the excess collagen and helps restore finger mobility.
6. Hyaluronidase – Hyaluronidase is an enzyme that can be injected into the cords of Dupuytren’s disease to break down the excess collagen. It is often used in combination with other treatments to enhance their effectiveness.
7. Acetyl-L-Carnitine – Acetyl-L-carnitine is an amino acid derivative that has shown potential in reducing fibrosis, a characteristic feature of Dupuytren’s disease. It is available in supplement form.
8. Tranilast – Tranilast, an anti-inflammatory drug, has demonstrated anti-fibrotic properties and may help slow down the progression of Dupuytren’s disease.
9. Pentoxifylline – Pentoxifylline is a medication that improves blood flow and reduces inflammation. While primarily used for other conditions, it has been studied for its potential in treating Dupuytren’s disease.
10. Pirfenidone – Pirfenidone is an anti-fibrotic drug that has been investigated for its effectiveness in treating Dupuytren’s disease. It may help reduce collagen production and inhibit the development of contractures.
11. Diltiazem – Diltiazem, a calcium channel blocker, has shown promise in reducing the severity of Dupuytren’s disease and preventing the progression of finger contractures.
12. Vitamin E – Vitamin E is an antioxidant that may have a beneficial effect on Dupuytren’s disease. While research is ongoing, some studies

References