Scleredema, also known as scleredema adultorum, is a rare connective tissue disorder characterized by thickening and hardening of the skin. It belongs to a group of conditions called scleroderma spectrum disorders, including systemic sclerosis.

• Scleredema is classified into two main types based on its association with another condition:

Type I: It is the most common form and is often preceded by an upper respiratory tract infection, usually caused by Streptococcus species.

Type II: This type is less common and is associated with underlying conditions such as monoclonal gammopathy (abnormal production of monoclonal immunoglobulins) or diabetes mellitus.

• Prevalence: The prevalence of scleredema is challenging to determine due to its rarity and variability in diagnostic criteria. Estimates suggest a prevalence ranging from 1 to 4 cases per 100,000 individuals in the general population.
• Age and gender distribution: Scleredema can affect individuals of all ages but primarily occurs in adults. Most cases are diagnosed between 30 and 60, with a peak incidence in the sixth decade of life. It affects both males and females, but some studies suggest a slight male predominance.
• Associated conditions: Scleredema can be associated with certain underlying conditions. Type II scleredema, which is less common, is often linked to diabetes mellitus or monoclonal gammopathy. Type I scleredema may have a preceding upper respiratory tract infection caused by Streptococcus species.
• Geographic distribution: Scleredema has been reported worldwide, but available data on its geographic distribution are limited. It appears to occur in different populations without significant regional variations.  The prevalence of scleredema is challenging to determine due to its rarity and variability in diagnostic criteria. Estimates suggest a prevalence ranging from 1 to 4 cases per 100,000 individuals in the general population.
• Age and gender distribution: Scleredema can affect individuals of all ages but primarily occurs in adults. Most cases are diagnosed between 30 and 60, with a peak incidence in the sixth decade of life. It affects both males and females, but some studies suggest a slight male predominance.
• Associated conditions: Scleredema can be associated with certain underlying conditions. Type II scleredema, which is less common, is often linked to diabetes mellitus or monoclonal gammopathy. Type I scleredema may have a preceding upper respiratory tract infection caused by Streptococcus species.
• Geographic distribution: Scleredema has been reported worldwide, but available data on its geographic distribution are limited. It appears to occur in different populations without significant regional variations.

• Dermal collagen accumulation: Scleredema is characterized by excessive collagen deposition in the dermis, which leads to thickening and hardening of the skin. This collagen accumulation is believed to result from increased collagen production by fibroblasts and impaired breakdown or clearance of collagen in the affected tissues.
• Inflammation and immune dysregulation: Inflammatory processes and immune dysregulation have been suggested to contribute to the pathogenesis of scleredema. Infiltration of immune cells, such as macrophages and T cells, has been observed in affected skin areas. This immune activation may trigger the production of cytokines and other inflammatory mediators, leading to tissue remodeling and fibrosis.
• Glycosaminoglycan (GAG) abnormalities: Abnormalities in glycosaminoglycan metabolism, specifically increased hyaluronic acid, have been proposed to contribute to scleredema. Hyaluronic acid is a component of the extracellular matrix and plays a role in maintaining tissue structure. Elevated levels of hyaluronic acid may contribute to the thickening and hardening of the skin seen in scleredema.
• Endothelial dysfunction: Dysfunction of endothelial cells, which line the blood vessels, has also been implicated in the pathophysiology of scleredema. Endothelial dysfunction may lead to altered blood flow and impaired nutrient and oxygen delivery to the affected skin, further contributing to tissue damage and fibrosis.

• Infection: In some cases, scleredema has been preceded by an upper respiratory tract infection, most caused by Streptococcus species. This association suggests a potential role of infection in triggering an immune response that leads to the development of scleredema.
• Diabetes mellitus: Scleredema has been reported to occur more frequently in individuals with diabetes, particularly those with poorly controlled blood glucose levels. The association between diabetes and scleredema suggests a potential relationship between metabolic abnormalities and the development of scleredema.
• Monoclonal gammopathy: Scleredema has been associated with monoclonal gammopathy, a condition characterized by the abnormal production of monoclonal immunoglobulins. This association suggests a potential link between immune dysregulation and the development of scleredema.

• Age at onset: The age at which scleredema develops may influence its prognosis. Scleredema in childhood or adolescence tends to have a more favorable prognosis, with a higher likelihood of spontaneous improvement or resolution than adult-onset cases.
• Disease severity: The extent and severity of skin involvement are considered important prognostic factors. Patients with more extensive skin thickening and induration are more likely to have a chronic and persistent disease course, while those with limited involvement may have a better prognosis.
• Systemic involvement: Scleredema primarily affects the skin, but in rare cases, it can be associated with systemic manifestations. Systemic involvement, such as respiratory or cardiovascular complications, may indicate a more severe disease course and impact the prognosis.
• Underlying conditions: Scleredema can be associated with other conditions, such as diabetes mellitus or monoclonal gammopathy. The presence of these underlying conditions may influence the prognosis and response to treatment.
• Treatment response: The response to treatment can also be a prognostic factor. Patients who demonstrate an excellent response to therapy, such as immunosuppressive agents or physical therapy, may have a more favorable prognosis than those who do not respond well.

• Age group: Scleredema can occur at any age, but it most commonly affects adults. Most cases are seen in middle-aged individuals, typically between 30 and 60. However, there have been reported cases in children and adolescents as well.
• Childhood and adolescence: Scleredema in this age group is rare but can occur. The clinical presentation may be similar to adult-onset scleredema, including skin thickening and hardening. However, the severity and extent of skin involvement may vary.
• Adulthood: Scleredema most commonly presents in adults. The age of onset in adults can vary, but it typically occurs in middle-aged individuals. The characteristic skin changes of thickening and hardening typically affect the neck, upper back, and shoulder regions.

Skin examination:

• Skin texture: The provider will assess the skin’s texture, looking for areas of thickening and hardening. The affected skin may have a woody or indurated appearance.
• Skin color: They will observe the color of the skin, which may be normal or slightly erythematous (red) in the early stages. As the condition progresses, the skin can become pale or dusky.
• Distribution of skin involvement: The provider will examine the distribution of skin changes, which typically affect the neck, upper back, and shoulders. The face, chest, and upper arms may also be involved in some cases.
• Skin mobility: They will assess the mobility and flexibility of the affected skin. Scleredema can cause tethering or limited movement of the skin.
• Skin temperature: The provider may evaluate the temperature of the affected skin, which can be normal or slightly cool to the touch.

Joint examination:

• Limited joint mobility: In some cases, scleredema can affect joint mobility. The provider will assess the range of motion of joints, particularly in the affected areas, such as the neck and shoulders.
• Associated findings:
• Associated comorbidities: The provider may inquire about or evaluate for associated conditions such as diabetes mellitus or monoclonal gammopathy, which can be seen in conjunction with scleredema.
• Systemic examination:

In most cases, scleredema primarily affects the skin and does not involve internal organs. However, in rare instances, systemic involvement may occur. If systemic manifestations are suspected, the provider may perform additional evaluations, such as respiratory or cardiovascular assessments.

Scleredema is often idiopathic, meaning it occurs without an identifiable cause.

• Diabetes mellitus: There is an increased prevalence of scleredema in individuals with diabetes, particularly those with poorly controlled blood glucose levels.
• Monoclonal gammopathy: Scleredema has been associated with monoclonal gammopathy, a condition characterized by the abnormal production of monoclonal immunoglobulins.

The acuity of scleredema presentation can vary among individuals.

• It may manifest in different ways:
• Gradual onset: Scleredema is a gradual onset, with skin thickening and hardening progressing slowly over weeks or months. This gradual progression may make it challenging to identify the exact onset time.
• Insidious development: In some cases, scleredema may have an insidious development, meaning it appears slowly over time without any specific triggering event.
• Subacute presentation: In certain instances, scleredema may have a subacute presentation, with a more rapid onset of skin changes occurring over weeks.

The treatment of scleredema focuses on managing symptoms and improving quality of life.

• Physical and Occupational Therapy: Physical and occupational therapy can help maintain joint mobility, improve range of motion, and minimize muscle stiffness. These therapies may include stretching exercises, heat therapy, and techniques to improve daily functioning.
• Topical Treatments: Various topical treatments may be prescribed to alleviate symptoms and improve skin condition. These can include moisturizers, emollients, and mild corticosteroid creams to reduce itching and inflammation.
• Systemic Therapy: In some cases, systemic medications may be prescribed to manage symptoms and slow down the progression of scleredema. These medications may include corticosteroids, immunosuppressive drugs (such as methotrexate or mycophenolate mofetil), or immunomodulatory agents (such as intravenous immunoglobulin).
• Phototherapy: Light therapy, specifically ultraviolet A1 (UVA1) phototherapy, has improved skin thickness and flexibility in scleredema. This treatment involves exposing the affected skin to UVA1 light in controlled settings.
• Compression Garments: Wearing compression garments or bandages may help improve skin mobility and reduce swelling in the affected areas. These garments can help prevent skin complications and improve comfort.
• Symptom Management: Additional treatments may be recommended to manage specific symptoms associated with scleredema. For example, non-steroidal anti-inflammatory drugs (NSAIDs) or analgesics may be prescribed if there is joint pain.

Dermatology, General

Non-pharmacological approaches can be beneficial as adjunctive therapies for managing scleredema. Here are some non-pharmacological approaches that may be considered:

• Physical and Occupational Therapy: Physical and occupational therapy are crucial in managing scleredema. These therapies focus on maintaining joint mobility, improving range of motion, and minimizing muscle stiffness.
• Skin Care: Proper skin care is essential for individuals with scleredema. Regular moisturizing of the skin using emollients or moisturizers can help reduce dryness, itching, and discomfort.
• Exercise: Engaging in regular exercise can have several benefits for individuals with scleredema. It can help improve circulation, maintain muscle strength, and enhance joint flexibility. Low-impact exercises such as walking, swimming, or gentle stretching can be suitable options.
• Heat Therapy: Applying heat in the form of warm compresses or warm baths can help relieve stiffness, improve blood circulation, and reduce discomfort in affected areas. It is essential to use caution and not apply excessive heat, as it can lead to burns or other skin complications.
• Psychological Support: Living with scleredema can be emotionally challenging due to the impact on appearance and physical functioning. Seeking support from therapists, counselors, or support groups can provide emotional support, coping strategies, and a platform to connect with others facing similar challenges.
• Lifestyle Modifications: Certain lifestyle modifications can help manage symptoms and improve overall well-being. These may include maintaining a healthy diet, managing stress through relaxation techniques or meditation, quitting smoking, and avoiding excessive alcohol consumption.

Dermatology, General

Internal Medicine

Immunosuppressants play a role in treating scleredema, particularly in cases where the condition is severe or not responding to other treatment modalities. Scleredema involves immune system dysregulation, contributing to excessive collagen deposition and skin thickening. Immunosuppressive medications work by suppressing the immune system, thereby reducing inflammation and slowing down the progression of scleredema.

• Methotrexate: Methotrexate is an immunosuppressant drug that is commonly used in the treatment of autoimmune disorders. It works by inhibiting the proliferation of immune cells and reducing the production of inflammatory molecules. Methotrexate can help alleviate skin thickening and improve symptoms associated with scleredema.
• Mycophenolate mofetil: Mycophenolate mofetil is another immunosuppressant used to treat scleredema. It suppresses the activity of certain immune cells involved in the inflammatory response. Mycophenolate mofetil can help reduce skin tightness, improve mobility, and alleviate other symptoms of scleredema.
• Cyclosporine: Cyclosporine is an immunosuppressive medication that modulates the immune response by targeting specific immune cells. It can be used to treat severe scleredema cases that are unresponsive to other therapies. Cyclosporine may help reduce skin thickening and improve joint mobility.

Dermatology, General

Internal Medicine

Topical steroids play a role in treating scleredema by reducing inflammation and relieving symptoms associated with the condition. Scleredema involves an abnormal collagen accumulation in the skin, leading to thickening and tightening. Topical steroids, also known as topical corticosteroids, can be applied directly to the affected skin to reduce inflammation and alleviate symptoms such as itching, redness, and discomfort.

Here are some key aspects of the role of topical steroids in the treatment of scleredema:

• Anti-inflammatory Effects: Topical steroids work by suppressing the immune response and reducing inflammation in the skin. They act by inhibiting the production of inflammatory mediators and reducing the infiltration of immune cells into the affected area. By decreasing inflammation, topical steroids can help alleviate symptoms and improve the appearance of the skin in scleredema.
• Symptom Relief: Scleredema can be associated with itching, dryness, and discomfort. Topical steroids can provide relief by reducing itching and soothing the skin. They can also help moisturize the affected area and improve skin flexibility, making it easier to move and reducing tightness.
• Site-Specific Treatment: Topical steroids are applied directly to the affected areas of the skin, allowing for targeted treatment. This localized approach is beneficial when scleredema affects specific body regions, such as the face or limbs.
• Potency and Formulation: Topical steroids come in various potencies and formulations, ranging from mild to potent. The choice of the specific topical steroid and its strength will depend on the severity of symptoms, the location of the affected skin, and the individual patient’s characteristics.

Dermatology, General

Internal Medicine

Non-steroidal anti-inflammatory drugs (NSAIDs) are commonly used to treat scleredema to help manage pain, inflammation, and stiffness associated with the condition.

• Pain Relief: NSAIDs are effective in reducing pain associated with scleredema. The inflammatory process in scleredema can lead to discomfort and tenderness in affected areas. NSAIDs work by inhibiting the production of certain enzymes called cyclooxygenases (COX), which are responsible for synthesizing inflammatory mediators called prostaglandins.
• Anti-inflammatory Effects: Scleredema involves inflammation of the skin and underlying tissues. NSAIDs have anti-inflammatory properties and can help reduce localized inflammation in affected areas. NSAIDs reduce swelling, redness, and heat associated with inflammation by suppressing the production of inflammatory mediators.
• Improved Mobility: Stiffness and reduced joint mobility are common symptoms in scleredema. NSAIDs can help reduce inflammation around the joints, allowing for improved movement and flexibility. By relieving pain and reducing inflammation, NSAIDs may enhance mobility in individuals with scleredema.
• Adjunctive Therapy: NSAIDs are often used as adjunctive therapy with other treatment modalities for scleredema. They can be used with topical treatments, physical therapy, or systemic therapies to provide a comprehensive approach to symptom management.

Dermatology, General

• Physical Therapy: Physical therapy plays a crucial role in managing scleredema symptoms. A physical therapist can develop an individualized exercise program to improve joint flexibility, maintain muscle strength, and enhance overall mobility. They may also recommend manual techniques such as massage or stretching to relieve stiffness and promote blood circulation.
• Occupational Therapy: Occupational therapists can assist individuals with scleredema in adapting their daily activities to maximize independence and minimize discomfort. They can recommend assistive devices, ergonomic modifications, and energy conservation techniques. Additionally, occupational therapists can help manage hand involvement, which may include splinting or specialized exercises.
• Skin Care: Since scleredema can cause skin dryness and tightness, proper skin care is essential. This may involve regularly moisturizing the skin using emollients or prescribed creams to keep it hydrated and prevent cracking. Avoiding excessive exposure to cold and dry environments can also help maintain skin integrity.
• Medications: In some cases, medications may be prescribed to manage specific symptoms of scleredema. For example, non-steroidal anti-inflammatory drugs (NSAIDs) can help reduce inflammation and relieve pain. Corticosteroids may be used in severe cases to suppress the immune response and reduce skin thickening. Other immunosuppressive drugs like methotrexate or mycophenolate mofetil may also be considered in certain situations.
• Extracorporeal Photopheresis (ECP): ECP is a procedure that involves removing a patient’s blood, treating it with a photosensitizing agent, and then exposing it to ultraviolet light before returning it to the body. ECP has been used to treat various immune-related conditions, including scleredema, to modulate the immune response and improve skin symptoms.
• Laser Therapy: Laser treatment has shown promise in managing skin thickening and tightness associated with scleredema. Different types of lasers, such as pulsed dye laser or fractional carbon dioxide laser, may target and reduce collagen buildup in the affected skin, leading to improved skin flexibility.

Infectious Disease

The management of scleredema typically involves a multi-disciplinary approach and can be divided into different phases based on the severity and progression of the disease.

• Acute Phase: This phase represents the initial presentation of scleredema and is characterized by rapid progression of skin thickening. Treatment during this phase focuses on controlling symptoms and preventing complications. Therapeutic interventions may include:
• Physical measures: Maintaining proper skin hygiene, avoiding trauma or pressure on affected areas, and using emollients or moisturizers to prevent dryness and cracking.
• Symptomatic relief: Non-steroidal anti-inflammatory drugs (NSAIDs) or low-dose oral corticosteroids may be prescribed to reduce inflammation, pain, and stiffness.
• Physiotherapy: Range of motion exercises and physical therapy may help maintain joint mobility and prevent contractures.
• Monitoring: Regular follow-up appointments are essential to assess disease progression, adjust treatment, and monitor for complications.
• Subacute Phase: In this phase, the disease activity and skin involvement stabilize, although some residual skin thickening may persist. The management goals shift towards long-term care and prevention of complications. Critical interventions may include:
• Skincare: Continue with proper skin hygiene and moisturization to prevent dryness and cracking.
• Physiotherapy: Continue exercises and physical therapy to maintain joint mobility and prevent contractures.
• Treatment of complications: Address any complications that may arise, such as infections, pressure ulcers, or joint stiffness.
• Supportive therapies: Depending on individual needs, other treatments like occupational therapy, speech therapy, or psychological support may be beneficial.
• Chronic Phase: This phase refers to the long-term management of scleredema. The disease activity is usually minimal, mainly focusing on maintaining skin integrity, preventing complications, and optimizing the quality of life. Strategies in this phase may include:
• Skin care: Ongoing moisturization, skin protection, and regular assessment for skin breakdown or infection signs.
• Physiotherapy and rehabilitation: Continued exercises, joint mobilization, and occupational therapy to manage joint stiffness and prevent contractures.
• Symptomatic relief: Treatment of residual symptoms, such as pain or discomfort, with appropriate medications as needed.
• Psychosocial support: Addressing emotional and psychological well-being through counseling or support groups.
• Regular monitoring: Periodic follow-up visits to assess disease stability, manage any emerging issues, and adjust treatment if necessary.