Scleredema

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Scleredema
Other namesBuschke disease, Scleredema of Buschke, and Scleredema adultorum [1] [2]
Specialty Rheumatology, Pediatrics   Blue pencil.svg

Scleredema, is a rare, self-limiting skin condition defined by progressive thickening and hardening of the skin, usually on the areas of the upper back, neck, shoulders and face. [3] The skin may also change color to red or orange. The disease was discovered by Abraham Buschke. Although the cause of scleredema is unknown, it is usually associated with a disease, usually diabetes, [4] a viral illness or strep throat. [5] It is usually not fatal, but it may cause death if the disease spreads to the internal organs. [3] It may also cause an infection. [5]

In biology, a self-limiting organism or colony of organisms limits its own growth by its actions. For example, a single organism may have a maximum size determined by genetics, or a colony of organisms may release waste which is ultimately toxic to the colony once it exceeds a certain population. In some cases, the self-limiting nature of a colony may be advantageous to the continued survival of the colony, such as in the case of parasites. If their numbers became too high, they would kill the host, and thus themselves. In other cases, self-limitation restricts the viability of predators, thus ensuring the long-term survival of rare species.

Skin soft outer covering organ of vertebrates

Skin is the soft outer tissue covering of vertebrates with three main functions: protection, regulation, and sensation.

Neck part of the body, on many terrestrial or secondarily aquatic vertebrates, that distinguishes the head from the torso or trunk

The neck is the part of the body, on many vertebrates, that separates the head from the torso. It contains blood vessels and nerves that supply structures in the head to the body. These in humans include part of the esophagus, the larynx, trachea, and thyroid gland, major blood vessels including the carotid arteries and jugular veins, and the top part of the spinal cord.

Contents

Diagnosis

The scleredema is usually proposed as a diagnosis based on the appearance of the skin and the patient's medical history. To confirm the diagnosis, the doctor performs a skin biopsy, in which hematoxylin and eosin staining will show a thick reticular dermis with thick collagen bundles separated by clear spaces. [6] The patient's blood may be examined for diseases that may appear after the onset of symptoms, such as multiple myeloma. [5]

Skin biopsy is a biopsy technique in which a skin lesion is removed to be sent to a pathologist to render a microscopic diagnosis. It is usually done under local anesthetic in a physician's office, and results are often available in 4 to 10 days. It is commonly performed by dermatologists. Skin biopsies are also done by family physicians, internists, surgeons, and other specialties. However, performed incorrectly, and without appropriate clinical information, a pathologist's interpretation of a skin biopsy can be severely limited, and therefore doctors and patients may forgo traditional biopsy techniques and instead choose Mohs surgery. There are four main types of skin biopsies: shave biopsy, punch biopsy, excisional biopsy, and incisional biopsy. The choice of the different skin biopsies is dependent on the suspected diagnosis of the skin lesion. Like most biopsies, patient consent and anesthesia are prerequisites.

Collagen is the main structural protein in the extracellular space in the various connective tissues in the body. As the main component of connective tissue, it is the most abundant protein in mammals, making 25% to 35% of the whole-body protein content. Collagen consists of amino acids wound together to form triple-helices of elongated fibrils. It is mostly found in fibrous tissues such as tendons, ligaments, and skin.

Blood specialized bodily fluid in animals

Blood is a body fluid in humans and other animals that delivers necessary substances such as nutrients and oxygen to the cells and transports metabolic waste products away from those same cells.

Treatment

Although many types of medications have been tried as treatments, none of them have been proven effective in treating scleredema. Those treatments, such as corticosteroids, may benefit the patient, but will not cure their condition. If the affected area is infected, it is usually treated immediately. The symptoms of the condition usually resolve within six months to two years after onset. However, patients whose condition was associated to diabetes may suffer for longer periods of time. [5]

A cure is a substance or procedure that ends a medical condition, such as a medication, a surgical operation, a change in lifestyle or even a philosophical mindset that helps end a person's sufferings; or the state of being healed, or cured.

Myocarditis resulting as a complication from the disease has been successfully treated with penicillin and steroids. [7]

Myocarditis extrinsic cardiomyopathy that is characterized as an inflammation of the heart muscle

Myocarditis, also known as inflammatory cardiomyopathy, is inflammation of the heart muscle. Symptoms can include shortness of breath, chest pain, decreased ability to exercise, and an irregular heartbeat. The duration of problems can vary from hours to months. Complications may include heart failure due to dilated cardiomyopathy or cardiac arrest.

Penicillin group of antibiotics derived from Penicillium fungi

Penicillin is a group of antibiotics which include penicillin G, penicillin V, procaine penicillin, and benzathine penicillin. Penicillin antibiotics were among the first medications to be effective against many bacterial infections caused by staphylococci and streptococci. They are still widely used today, though many types of bacteria have developed resistance following extensive use.

See also

Related Research Articles

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Hyperglycemia, is a condition in which an excessive amount of glucose circulates in the blood plasma. This is generally a blood sugar level higher than 11.1 mmol/l (200 mg/dl), but symptoms may not start to become noticeable until even higher values such as 15–20 mmol/l (~250–300 mg/dl). A subject with a consistent range between ~5.6 and ~7 mmol/l is considered slightly hyperglycemic, while above 7 mmol/l is generally held to have diabetes. For diabetics, glucose levels that are considered to be too hyperglycemic can vary from person to person, mainly due to the person's renal threshold of glucose and overall glucose tolerance. On average however, chronic levels above 10–12 mmol/L can produce noticeable organ damage over time.

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Peripheral neuropathy disease

Peripheral neuropathy, often shortened to neuropathy, is a general term describing disease affecting the peripheral nerves, meaning nerves beyond the brain and spinal cord. Damage to peripheral nerves may impair sensation, movement, gland or organ function depending on which nerves are affected; in other words, neuropathy affecting motor, sensory, or autonomic nerves result in different symptoms. More than one type of nerve may be affected simultaneously. Peripheral neuropathy may be acute or chronic, and may be reversible or permanent.

Ichthyosis skin disease in which dry, scaly skin accumulates

Ichthyosis is a family of rare genetic skin disorders characterized by dry, thickened, scaly skin.

Pagets disease of the breast type of cancer that outwardly may have the appearance of eczema, with skin changes involving the nipple of the breast.

Paget's disease of the breast is a type of cancer that outwardly may have the appearance of eczema, with skin changes involving the nipple of the breast. The condition is an uncommon disease accounting for 1 to 4.3% of all breast cancers and was first described by Sir James Paget in 1874. The condition in itself often appears innocuous, limited to a surface appearance and it is sometimes dismissed, although actually indicative of underlying breast cancer.

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Diabetic nephropathy disease

Diabetic nephropathy (DN), also known as diabetic kidney disease, is the chronic loss of kidney function occurring in those with diabetes mellitus. Protein loss in the urine due to damage to the glomeruli may become massive, and cause a low serum albumin with resulting generalized body swelling (edema) and result in the nephrotic syndrome. Likewise, the estimated glomerular filtration rate (eGFR) may progressively fall from a normal of over 90 ml/min/1.73m2 to less than 15, at which point the patient is said to have end-stage kidney disease (ESKD). It usually is slowly progressive over years.

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Urticaria pigmentosa is the most common form of cutaneous mastocytosis. It is a rare disease caused by excessive numbers of mast cells in the skin that produce hives or lesions on the skin when irritated.

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Amyloid light-chain (AL) amyloidosis, also known as primary amyloidosis, is the most common form of systemic amyloidosis in the US. The disease is caused when a person's antibody-producing cells do not function properly and produce abnormal protein fibers made of components of antibodies called light chains. These light chains come together to form amyloid deposits which can cause serious damage to different organs. Abnormal light chains in urine are sometimes referred to as "Bence Jones protein".

Dermatitis herpetiformis chronic blistering skin condition, characterised by blisters filled with a watery fluid

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Acquired generalized lipodystrophy is a rare skin condition that appears during childhood or adolescence, characterized by fat loss affecting large areas of the body, particularly the face, arms, and legs. There are 4 types of lipodystrophy based on its onset and areas affected: acquired or inherited, and generalized or partial. Both acquired or inherited lipodystrophy present as loss of adipose tissues. The near-total loss of subcutaneous adipose tissue is termed generalized lipodystrophy while the selective loss of adipose tissues is denoted as partial lipodystrophy. Thus, as the name suggests, AGL is a near-total deficiency of adipose tissues in the body that is developed later in life. It is an extremely rare disease that only about 100 cases are reported worldwide. There are three main etiologies of AGL suspected: autoimmune, panniculitis-associated, or idiopathic. After its onset, the disease progresses over a few days, weeks, months, or even in years. Clinical presentations of AGL are similar to other lipodystrophies, including metabolic complications and hypoleptinemia. Treatments are also similar and mainly supportive for symptomatic alleviation. Although HIV- or drug-induced lipodystrophy are a type of acquired lipodystrophy, its origin is very specific and distinct hence is usually not discussed with AGL.

African tick bite fever spotted fever that has material basis in Rickettsia africae, which is transmitted by ticks

African tick bite fever (ATBF) is a bacterial infection spread by the bite of a tick. Symptoms may include fever, headache, muscles pains, and a rash. At the site of the bite there is typically a red skin sore with a dark center. Onset usually occur 4–10 days after the bite. Complications are rare, however may include joint inflammation. Some people do not develop symptoms.

Lymphocytic esophagitis

Lymphocytic esophagitis is a rare and poorly understood medical disorder involving inflammation in the esophagus. The disease is named from the primary inflammatory process, wherein lymphocytes are seen within the esophageal mucosa. Symptoms of the condition include difficulty swallowing, heartburn and food bolus obstruction. The condition was first described in 2006 by Rubio and colleagues. Initial reports questioned whether this was a true medical disorder, or whether the inflammation was secondary to another condition, such as gastroesophageal reflux disease.

References

  1. Turchin I, Adams SP, Enta T (September 2003). "Dermacase. Scleredema adultorum, or Bushke disease". Can Fam Physician. 49: 1089, 1093. PMC   2214291 . PMID   14526859.
  2. Pegum JS (June 1972). "Scleredema of Buschke". Proc. R. Soc. Med. 65 (6): 528. PMC   1643957 . PMID   5044976.
  3. 1 2 "Scleredema" . Retrieved 2009-05-18.
  4. Meguerditchian, C; Jacquet P; Béliard S; et al. (November 2006). "Scleredema adultorum of Buschke: an under recognized skin complication of diabetes". Diabetes and Metabolism. 32 (5): 481–484. doi:10.1016/S1262-3636(07)70307-5. PMID   17110904.
  5. 1 2 3 4 "Scleredema" . Retrieved 2009-05-18.
  6. Schmults CA (October 2003). "Scleredema". Dermatol. Online J. 9 (4): 11. PMID   14594584.
  7. Erlichman, Matityahu; Glaser, Joram (1983). "Buschke's Sclerema with right-sided heart failure". Cardiology. 70 (6): 344–348. doi:10.1159/000173618. PMID   6673828.
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