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adiposis dolorosa
ICD-10 Codes
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Description
Adiposis Dolorosa: A Rare Condition Characterized by Painful Fatty Tissue Growth
Adiposis dolorosa, also known as Dercum's disease, is a rare and complex condition characterized by the growth of multiple, painful fatty tumors called lipomas. These lipomas can occur anywhere on the body, but are most commonly found in the abdomen, extremities, and subcutaneous tissue.
Key Features:
- Painful Fatty Tumors: Adiposis dolorosa is marked by the development of multiple, painful lipomas that can cause severe discomfort and pain.
- Obesity and Weight Gain: The condition often occurs in individuals who are overweight or obese, particularly women between the ages of 35 and 50.
- Weakness, Fatigue, and Mental Disturbances: Other symptoms may include weakness, fatigue, and a range of mental disturbances such as instability, depression, confusion, dementia, and epilepsy.
Types and Classification:
Adiposis dolorosa can be classified into four types:
- Generalized diffuse form with diffusely widespread painful adipose tissue without clear lipomas
- Generalized localized form with multiple painful lipomas
- Focal form with a single large painful lipoma
- Mixed form with a combination of the above features
Causes and Diagnosis:
The exact cause of adiposis dolorosa is unknown, but it is believed to be a combination of neurologic and endocrine disorders. The condition can be diagnosed through a combination of clinical evaluation, imaging studies (such as MRI or CT
Additional Characteristics
- Painful Fatty Tumors
- Obesity and Weight Gain
- Weakness, Fatigue, and Mental Disturbances
- Generalized diffuse form with diffusely widespread painful adipose tissue without clear lipomas
- Generalized localized form with multiple painful lipomas
- Focal form with a single large painful lipoma
- Mixed form with a combination of the above features
Signs and Symptoms
Diagnostic Tests
Diagnostic Tests for Adiposis Dolorosa
Adiposis dolorosa, also known as Dercum disease, is a rare disorder characterized by the growth of multiple, painful lipomas (benign, fatty tumors). While there are no specific diagnostic tests for this condition, several investigations may be considered to aid in diagnosis.
- Blood Tests: Blood tests may reveal an increase in cholesterol levels and ESR (erythrocyte sedimentation rate) [1].
- Biopsy: A biopsy involves taking a small tissue sample from the affected area and examining it under a microscope. This can help confirm the presence of lipomas [4][9].
- Imaging Studies: Imaging studies such as CT scans or MRI scans may be used to help diagnose adiposis dolorosa. These tests can provide detailed images of the affected areas, which can aid in diagnosis [3][6].
- Clinical Examination: A thorough clinical examination is essential for diagnosing adiposis dolorosa. This involves a systematic physical examination and exclusion of other differential diagnoses [2][10].
It's worth noting that the diagnosis of adiposis dolorosa is primarily based on clinical criteria, including chronic pain of the subcutaneous tissue for over 3 months in overweight patients or patients with obesity [12]. However, these criteria still need to be validated.
References:
[1] Context result 1 [2] Context result 2 [3] Context result 6 [4] Context result 4 [9] Context result 9 [10] Context result 11 [12] Context result 12
Additional Diagnostic Tests
- Blood Tests
- Biopsy
- Imaging Studies
- Clinical Examination
Treatment
Dercum Disease: A Rare Condition Characterized by Benign Painful Subcutaneous Lipomas
Dercum disease, also known as adiposis dolorosa or Dercum syndrome, is a rare condition characterized by the gradual and progressive development of benign painful subcutaneous lipomas and angiolipomas that vary in size, number, and location [4]. This condition causes significant morbidity due to chronic, often treatment-resistant pain, which greatly impairs the quality of life for those affected.
Symptoms and Treatment
The symptoms of Dercum disease include:
- Gradual development of benign painful subcutaneous lipomas and angiolipomas
- Chronic, often treatment-resistant pain
- Significant morbidity due to impaired quality of life
Treatment options for Dercum disease are limited and focus on managing the symptoms. Some methods that have been described for pain relief include:
- Liposuction or surgical resection of lipomas
- Traditional analgesics (NSAIDs)
- Intravenous lidocaine or peroral mexiletine [6]
- Transdermal lidocaine 5% patches [8]
Current Treatment Approaches
While current approaches to treatment focus on surgery, opiates, and other medications, health care providers may also consider ketamine infusion as a potential treatment option [3]. However, caution should be used when considering prednisone for pain relief due to the risk of adverse effects outweighing the benefits [5].
Conclusion
Dercum disease is a rare condition characterized by benign painful subcutaneous lipomas and angiolipomas. The symptoms are chronic and often treatment-resistant, causing significant morbidity and impairing quality of life. Treatment options are limited, but healthcare providers may consider various methods to manage the pain.
References:
[1] Kastrup J. Dercum's disease (adiposis dolorosa). Treatment of the severe pain with intravenous lidocaine. Pain. 1987 Jan; 28(1):77-80. [2] Ghazala S, Bilal J, Ross E, Riaz IB, Kalb B, Herbst KL. Low ... [3] by AH Eliason · 2019 · Cited by 3 — While current approaches to treatment focus on surgery, opiates, and other medications, health care providers may also consider ketamine infusion, ... [4] Dercum disease is a rare condition characterised by the gradual and progressive development of benign painful subcutaneous lipomas and angiolipomas that vary in size, number, and location. [5] Caution should be used when considering prednisone for pain relief due to the risk of adverse effects outweighing the benefits [5]. [6] Intravenous lidocaine or peroral mexiletine have been described as potential treatment options for Dercum disease [6]. [7] Transdermal lidocaine 5% patches have also been used to manage pain in patients with Dercum disease [8].
Recommended Medications
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Differential Diagnosis
Differential Diagnosis of Adiposis Dolorosa
Adiposis dolorosa, also known as Dercum disease, is a rare condition characterized by the development of multiple, painful subcutaneous lipomas and angiolipomas. When diagnosing this condition, it's essential to consider several differential diagnoses that may present with similar symptoms.
List of Differential Diagnoses:
- Fibromyalgia: A chronic condition characterized by widespread muscle pain, fatigue, and tender points.
- Lipoedema: A condition involving the abnormal growth of fat cells in the legs, hips, and buttocks.
- Panniculitis: An inflammation of the fatty tissue under the skin, which can cause painful nodules or lesions.
- Endocrine disorders: Certain hormonal imbalances, such as Cushing's syndrome or hypothyroidism, can lead to weight gain and pain in the subcutaneous tissues.
- Primary psychiatric disorders: Mental health conditions like depression or anxiety can manifest physically, leading to symptoms similar to adiposis dolorosa.
- Multiple symmetric lipomatosis (Madelung syndrome): A rare condition characterized by the growth of multiple, symmetrical lipomas on the body.
- Multiple familial lipomatosis: A genetic disorder that causes the growth of multiple lipomas throughout the body.
- Proteus syndrome: A rare genetic disorder that can cause the growth of unusual skin lesions and tumors.
- Benign adipose tissue tumors: Various types of non-cancerous tumors that grow in the fatty tissues.
Key Points to Consider:
When differentiating between these conditions, it's crucial to consider factors such as:
- The presence and location of painful subcutaneous lipomas or angiolipomas
- Generalized obesity and weight gain patterns
- Associated symptoms like fatigue, difficulty concentrating, and sleep disturbances
- Physical examination findings, including the size, number, and location of lipomas
References:
[4] Dercum's disease (DD) is a rare lipodystrophy syndrome characterized by obesity and tender fatty tissues. It is also known as adiposis dolorosa, ...
[7] Differential diagnoses include: fibromyalgia, lipoedema, panniculitis, endocrine disorders, primary psychiatric disorders, multiple symmetric lipomatosis, familial multiple lipomatosis, and adipose tissue tumours.
[10] The diagnosis of adiposis dolorosa should only be made when other diagnoses have been excluded. Differential diagnoses include fibromyalgia, panniculitis, endocrine disorders, primary psychiatric disorders, multiple symmetric lipomatosis (Madelung syndrome), multiple familial lipomatosis, Proteus syndrome, and benign adipose tissue tumors.
[13] The diagnosis of adiposis dolorosa is based on clinical history and the physical findings of lipomas and is made by exclusion. There is no current specific lab test.
Additional Differential Diagnoses
- Endocrine disorders
- Primary psychiatric disorders
- Lipoedema
- Benign adipose tissue tumors
- panniculitis
- Proteus syndrome
- multiple symmetric lipomatosis
- fibromyalgia
- familial multiple lipomatosis
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