Dupuytren's Contracture (Irish Hand Disease): Causes, Symptoms, Diagnosis, and Treatment

What is the Irish hand disease?

The term "Irish hand disease" is a colloquial name for Dupuytren's contracture, a progressive fibrotic condition that causes one or more fingers to permanently bend into the palm, often affecting individuals of Northern European descent.

Understanding Dupuytren's Contracture (The "Irish Hand Disease")

Dupuytren's contracture, often referred to as "Irish hand disease" or "Viking disease" due to its higher prevalence in populations with Northern European ancestry, is a chronic, progressive condition affecting the hand. It is characterized by the abnormal thickening and shortening of the fascia, the fibrous tissue located just beneath the skin in the palm of the hand. This thickening leads to the formation of nodules (lumps) and cords that can eventually pull the fingers into a flexed, or bent, position, making it difficult or impossible to fully straighten them. While the condition can affect any finger, the ring and little fingers are most commonly involved. It can occur in one or both hands and may also manifest in other areas, such as the soles of the feet (Ledderhose disease) or the penis (Peyronie's disease).

Causes and Risk Factors

The exact cause of Dupuytren's contracture remains unknown, but it is understood to be a complex interplay of genetic predisposition and environmental factors.

• Genetics and Ancestry: This is the strongest known risk factor. The condition is significantly more common in people of Northern European descent, particularly those with Irish, Scottish, English, or Scandinavian ancestry, hence the colloquial names. It often runs in families, suggesting a strong genetic component.
• Age: Dupuytren's contracture typically develops in middle-aged and older adults, with the risk increasing with age.
• Gender: Men are more likely to develop Dupuytren's contracture than women, and they often experience more severe contractures at an earlier age.
• Medical Conditions: Certain health conditions are associated with an increased risk:
  • Diabetes: Individuals with diabetes have a higher incidence and often more severe forms of the disease.
  • Epilepsy: Especially in those taking anti-seizure medications like phenytoin.
  • Alcoholism and Liver Disease: Chronic alcohol consumption and liver cirrhosis are linked to an increased risk.
• Smoking: Tobacco use has been identified as a risk factor.
• Hand Trauma or Repetitive Vibration: While less definitively proven than genetic factors, some studies suggest a possible link between hand injuries, repetitive hand use, or exposure to vibrating tools and the development or progression of Dupuytren's.

Signs and Symptoms

Dupuytren's contracture typically progresses slowly over years. The signs and symptoms can vary in severity and presentation:

• Nodules: The first sign is often the appearance of one or more small, firm lumps or nodules under the skin in the palm of the hand. These are usually painless but can sometimes be tender to the touch.
• Cords: As the condition progresses, these nodules may thicken and extend into fibrous cords that run from the palm into the fingers. These cords are visible and palpable.
• Contracture: The most defining symptom is the gradual bending of the fingers towards the palm, making it difficult or impossible to fully straighten them. The ring and little fingers are most commonly affected, followed by the middle finger. The thumb and index finger are less frequently involved.
• Functional Limitations: The inability to fully straighten the fingers can significantly impair daily activities such as washing hands, putting on gloves, gripping objects, shaking hands, or placing a hand flat on a surface.
• Pitting and Grooves: In some cases, the skin over the affected area may appear puckered or dimpled.

Diagnosis

Diagnosing Dupuytren's contracture typically involves a thorough physical examination by a hand specialist or orthopedic surgeon.

• Medical History: The doctor will inquire about the patient's symptoms, family history of the condition, and any associated medical conditions.
• Physical Examination: The physician will visually inspect and palpate the hand to identify nodules, cords, and measure the degree of contracture in the fingers.
  • Tabletop Test: A common test involves asking the patient to place their palm flat on a table. If the hand cannot lie completely flat due to finger contracture, it indicates a significant degree of Dupuytren's contracture.
• Imaging (Rarely Needed): X-rays or other imaging studies are generally not required to diagnose Dupuytren's contracture, as it is a soft tissue condition. However, they may be used to rule out other conditions affecting the bones or joints if symptoms are atypical.

Treatment and Management Options

The choice of treatment depends on the severity of the contracture, the rate of progression, and the impact on hand function. Mild cases that do not interfere with daily activities may only require observation.

• Non-Surgical Treatments: These are typically considered for contractures that are interfering with function but are not yet severe enough for surgery.
  • Needle Aponeurotomy (NA) / Percutaneous Needle Fasciotomy: This minimally invasive procedure involves using a fine needle to puncture and sever the Dupuytren's cord, allowing the finger to be straightened. It is performed under local anesthesia and has a shorter recovery time than surgery, but recurrence rates can be higher.
  • Collagenase Clostridium Histolyticum (Xiaflex) Injection: This involves injecting an enzyme into the Dupuytren's cord, which helps break down the collagen that forms the cord. After the injection, the finger is manipulated a day or two later to break the cord and straighten the finger.
• Surgical Treatments: Surgery is typically recommended for more advanced contractures where non-surgical options are not effective or when the contracture significantly impairs hand function (e.g., inability to perform the tabletop test).
  • Partial Fasciectomy: This is the most common surgical procedure, involving the removal of the diseased fascia and cords. It is performed under regional or general anesthesia.
  • Dermofasciectomy: In cases of severe or recurrent Dupuytren's, particularly when the skin is involved, the diseased fascia and overlying skin may be removed, requiring a skin graft.
  • Amputation: In very rare, severe, and recurrent cases that significantly impair function and cannot be otherwise managed, amputation of the affected finger may be considered as a last resort.

Role of Physical Therapy and Exercise

Physical therapy and hand exercises play a crucial role, particularly in the post-treatment phase, to optimize recovery and function.

• Post-Procedure Rehabilitation: Following needle aponeurotomy, collagenase injection, or surgery, physical therapy is essential to help regain range of motion, reduce stiffness, and minimize scar tissue formation.
• Stretching and Splinting: Therapists guide patients through specific stretching exercises designed to maintain the straightened position of the fingers and improve flexibility. Custom splints may be prescribed to be worn during the day or night to prevent the contracture from returning.
• Hand Exercises: Exercises are used to improve grip strength, dexterity, and overall hand function. These may include:
  • Finger Extension Exercises: Gently extending the affected fingers.
  • Grip Strengthening: Using soft balls or putty.
  • Fine Motor Coordination: Exercises involving small objects to improve dexterity.
• Scar Management: After surgery, therapists can provide techniques for scar massage and management to prevent excessive scarring and improve tissue pliability.
• Education: Patients receive education on activity modification, ergonomic principles, and strategies to prevent recurrence.

It is important to understand that while physical therapy and exercise are vital for rehabilitation and maintaining function, they do not cure Dupuytren's contracture or prevent its progression in the absence of other medical intervention once contracture has begun.

Prognosis and Living with Dupuytren's

Dupuytren's contracture is a progressive condition, meaning it tends to worsen over time, though the rate of progression varies greatly among individuals. While treatments can effectively straighten the fingers and restore function, recurrence of the contracture is possible, especially after non-surgical interventions. Regular follow-up with a hand specialist is recommended to monitor the condition. Living with Dupuytren's often involves adapting to functional limitations, but with appropriate management and rehabilitation, most individuals can maintain a good quality of life and hand function.

Conclusion

The "Irish hand disease," scientifically known as Dupuytren's contracture, is a genetically influenced, progressive condition characterized by the bending of fingers into the palm. While its exact cause remains elusive, its strong association with Northern European ancestry and various risk factors highlights the importance of understanding its origins. Early recognition of symptoms, timely diagnosis, and appropriate intervention—ranging from observation to non-surgical or surgical treatments, complemented by dedicated physical therapy—are crucial for managing the condition, preserving hand function, and improving quality of life for those affected.