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scleredema adultorum
Description
Scleredema Adultorum: A Rare Connective Tissue Disorder
Scleredema adultorum, also known as Buschke disease or scleredema diabeticorum, is a rare connective tissue disorder characterized by the progressive thickening and hardening of the skin. This condition typically affects adults and is often associated with underlying diseases such as diabetes mellitus.
Clinical Presentation
The clinical presentation of scleredema adultorum includes:
- Woody induration: The affected areas become firm and woody, sometimes slightly red or brown.
- Skin thickening: The skin becomes thickened and hardened, leading to a characteristic "woody" texture.
- Symmetrical hardening: The condition typically presents with symmetrical hardening and thickening of the skin.
Types of Scleredema Adultorum
There are three types of scleredema adultorum, distinguished by their association with pre-existing or underlying diseases:
- Scleredema in association with infections: This type is often associated with streptococcal infections.
- Scleredema in association with diabetes mellitus: This type is the most common and is characterized by thickening and hardening of the skin, usually localized to the nape, back, and shoulder areas.
- Scleredema in association with other diseases: Other conditions such as primary hyperparathyroidism, rheumatoid arthritis, ankylosing spondylitis, Sjogren syndrome, dermatomyositis, Waldenstrom macroglobulinemia, anaphylactoid purpura, primary biliary cirrhosis, and IgA deficiency have been associated with scleredema adultorum.
Treatment
While there is no specific treatment for scleredema adultorum, some cases may respond to radiation therapy. However, the condition is usually self-limiting and resolves on its own over time.
References:
- [1] Scleredema of Buschke is a rare connective tissue disorder. Its pathogenesis is poorly understood.
- [3] The skin may also change color to red or orange.
- [4] Although the cause of scleredema is unknown, it is usually associated with a disease, usually diabetes.
- [14] Scleredema adultorum (SA) is a rare scleromucinous disease of unknown etiology that characteristically leads to wodden induration located on the neck and upper trunk.
Additional Characteristics
- Skin thickening
- Woody induration
- Symmetrical hardening
Signs and Symptoms
Scleredema adultorum, also known as scleredema, is a rare skin condition characterized by firm and non-pitting edema of the skin. The signs and symptoms of this condition include:
- Hardening and thickening of the skin: This is one of the most common symptoms of scleredema, which can lead to restricted movement in affected areas [10].
- Skin induration: The skin becomes firm and woody, with a characteristic "woody" or "firm" texture [1].
- Redness and brown discoloration: Affected skin areas may appear red or brown, giving them an "orange-skin" appearance [10].
- Stiffness and heaviness: Patients may experience stiffness and heaviness in affected limbs, making it difficult to move them [5].
- Pruritus and pain: Some people with scleredema may experience pruritus (itching) or pain in the affected areas [3].
It's worth noting that the symptoms of scleredema can vary from person to person, and not everyone will experience all of these symptoms. In some cases, the condition may resolve spontaneously on its own, while in other cases it may persist for long periods of time [10].
Additional Symptoms
- Hardening and thickening of the skin
- Skin induration
- Redness and brown discoloration
- Stiffness and heaviness
- pain
Diagnostic Tests
Diagnostic Tests for Scleredema Adultorum
Scleredema adultorum, also known as Buschke's disease, is a rare connective tissue disorder that requires accurate diagnosis to rule out other conditions with similar symptoms. The following diagnostic tests are commonly used to diagnose scleredema adultorum:
- Skin Biopsy: A punch biopsy or incisional biopsy of lesional skin that extends to include subcutaneous fat is recommended [1]. This test helps confirm the presence of mucin deposits and grossly increased dermal width, which are characteristic features of scleredema adultorum.
- Blood Tests: While not always necessary, blood tests may be performed to rule out associated conditions such as diabetes mellitus (DM) and monoclonal gammopathy [11]. Fasting blood sugar, glucose tolerance test, and hemoglobin A1C test may be included in the diagnostic workup.
- Clinical Evaluation: A thorough clinical evaluation is essential to diagnose scleredema adultorum. The condition typically presents with symmetrical hardening and thickening of the skin, firm and woody plaques, sometimes slightly red or brown [8]. The affected areas are usually located on the neck, shoulders, and upper thorax.
Other Diagnostic Considerations
It is essential to consider other conditions that may present with similar symptoms, such as morphea, systemic sclerosis, nephrogenic fibrosing dermatopathy, and scleroderma-like disorders [11]. A comprehensive diagnostic workup should include a detailed medical history and physical examination to rule out these conditions.
References
[1] Lorincz AL. Scleredema adultorum of Buschke and ... (search result 1) [8] Scleroedema presents with symmetrical hardening and thickening of the skin. (search result 8) [11] Clinical differential diagnosis of scleredema includes other clinical entities with indurated skin, namely morphea, systemic sclerosis, nephrogenic ... (search result 11)
Additional Diagnostic Tests
- Blood Tests
- Clinical Evaluation
- Skin Biopsy
Treatment
Differential Diagnosis
Scleredema adultorum, also known as Buschke's scleredema, is a rare skin disease that can be challenging to diagnose due to its similarity with other conditions. The differential diagnosis for scleredema adultorum includes several diseases that share similar clinical and histological features.
Key Differential Diagnoses:
- Scleromyxedema: A rare skin disorder characterized by thickening of the skin, mucin deposits, and a distinctive "woody" texture.
- Systemic sclerosis (scleroderma): A chronic autoimmune disease that affects the skin and internal organs, causing thickening and hardening of the skin.
- Nephrogenic systemic fibrosis: A rare condition characterized by progressive thickening and hardening of the skin, often associated with kidney disease.
- Eosinophilic fasciitis: A rare inflammatory disorder that affects the skin and subcutaneous tissue, causing thickening and hardening of the skin.
- Morphea: A type of localized scleroderma characterized by patches of hardened skin.
Other Conditions to Consider:
- Graft-versus-host disease: A complication of bone marrow transplantation that can cause skin thickening and hardening.
- Eosinophilia-myalgia syndrome: A rare condition characterized by muscle pain, eosinophilia, and skin thickening.
- Lichen sclerosus and atrophicus: A chronic inflammatory disorder that affects the skin, causing thinning and hardening of the skin.
Diagnostic Considerations:
To diagnose scleredema adultorum accurately, it is essential to consider these differential diagnoses and perform a thorough clinical evaluation, including:
- Histological examination of skin biopsies
- Clinical presentation, including non-pitting induration of the skin
- Laboratory tests to rule out other conditions
References:
[1] Scleredema adultorum associated with type 2 diabetes mellitus: a report of three cases. [5] [3] DIFFERENTIAL DIAGNOSIS; TREATMENT. [6] Scleredema ยท Definition / general. Nonpitting, woody induration of upper back skin; associated with diabetes [10] Cases of concomitant lichen sclerosus and atrophicus and scleredema adultorum of Buschke had been reported, [13] Scleredema adultorum of Buschke is an important differential diagnosis in sclerosing diseases.
Additional Differential Diagnoses
- Scleromyxedema
- Morphea
- Eosinophilic fasciitis
- Nephrogenic systemic fibrosis
- Systemic sclerosis (scleroderma)
- graft-versus-host disease
- lichen disease
- eosinophilia-myalgia syndrome
Additional Information
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- A skin disease that is characterized by induration of the skin, especially of the neck and upper trunk, has_symptom skin hardening or stiffening, and is associated with infections (especially streptococcal), hematologic malignancies, diabetes, and adverse drug reactions.
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