Scleredema: Difference between revisions
From WikiMD's Wellness Encyclopedia
Removed redirect to Sclerema edematosum Tags: Removed redirect visualeditor-wikitext |
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{{Infobox medical condition | |||
| name = Scleredema | |||
| synonyms = Scleredema adultorum | |||
| field = [[Dermatology]] | |||
| symptoms = [[Skin]] thickening, [[swelling]], [[stiffness]] | |||
| complications = [[Limited range of motion]], [[difficulty swallowing]] | |||
| onset = Usually [[adulthood]] | |||
| duration = [[Chronic (medicine)|Chronic]] | |||
| causes = Unknown, possibly related to [[diabetes mellitus]], [[streptococcal infection]] | |||
| risks = [[Diabetes]], [[obesity]], [[infection]] | |||
| diagnosis = [[Clinical diagnosis]], [[skin biopsy]] | |||
| differential = [[Scleroderma]], [[morphea]], [[eosinophilic fasciitis]] | |||
| treatment = [[Physical therapy]], [[corticosteroids]], [[immunosuppressive drugs]] | |||
| prognosis = Variable, can be [[self-limiting]] or [[progressive]] | |||
| frequency = Rare | |||
}} | |||
Sclerema edematosis, scleredema or Buschke disease is a rare pathological condition of [[connective tissue]]. | Sclerema edematosis, scleredema or Buschke disease is a rare pathological condition of [[connective tissue]]. | ||
== Other names == | == Other names == | ||
Scleredema adultorum; Scleredema adultorum of Buschke; Scleredema diabeticorum | Scleredema adultorum; Scleredema adultorum of Buschke; Scleredema diabeticorum | ||
== Pathophysiology == | == Pathophysiology == | ||
Scleredema is a form of cutaneous mucinosis, a diverse group of rare skin conditions that are characterized by an accumulation of mucin (a jelly-like complex carbohydrate substance) in the skin. | Scleredema is a form of cutaneous mucinosis, a diverse group of rare skin conditions that are characterized by an accumulation of mucin (a jelly-like complex carbohydrate substance) in the skin. | ||
== Cause == | == Cause == | ||
* Its pathogenesis is poorly understood. | * Its pathogenesis is poorly understood. | ||
* It occurs most commonly following an infectious episode. | * It occurs most commonly following an infectious episode. | ||
* It has also been linked to diabetes mellitus and to hematological disorders. | * It has also been linked to diabetes mellitus and to hematological disorders. | ||
== History == | == History == | ||
It was described by Curizo in 1752, and the disease was well defined afterward by Buschke in 1902. | It was described by Curizo in 1752, and the disease was well defined afterward by Buschke in 1902. | ||
== Classification == | == Classification == | ||
It belongs to the spectrum of scleroderma-like disorders. | It belongs to the spectrum of scleroderma-like disorders. | ||
== Clinical features == | == Clinical features == | ||
* It causes fibro-mucinous progressive induration of the skin, involving the neck, shoulders and proximal upper members and eventually the face. | * It causes fibro-mucinous progressive induration of the skin, involving the neck, shoulders and proximal upper members and eventually the face. | ||
* Signs and symptoms of this condition include hardening and thickening of the skin which may restrict movement. | * Signs and symptoms of this condition include hardening and thickening of the skin which may restrict movement. | ||
* Skin in affected areas may be red or brown and often has an 'orange-skin' appearance. | * Skin in affected areas may be red or brown and often has an 'orange-skin' appearance. | ||
== Types == | == Types == | ||
There are three forms of the condition which vary by disease course and long term outlook. | There are three forms of the condition which vary by disease course and long term outlook. | ||
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* In some cases, scleredema resolves spontaneously on its own, while in other affected people, the condition persists for long periods of time. | * In some cases, scleredema resolves spontaneously on its own, while in other affected people, the condition persists for long periods of time. | ||
* Due to the rarity of the condition, there is no standard treatment | * Due to the rarity of the condition, there is no standard treatment | ||
== Histology == | == Histology == | ||
Histological features commonly seen include dermal fibrosis with thickened collagen bundles and variable amounts of mucin deposits. | Histological features commonly seen include dermal fibrosis with thickened collagen bundles and variable amounts of mucin deposits. | ||
==Diagnosis== | ==Diagnosis== | ||
* The scleredema is considered as diagnosis based on the appearance of the skin and the patient's medical history. | * The scleredema is considered as diagnosis based on the appearance of the skin and the patient's medical history. | ||
* A [[skin biopsy]], in which hematoxylin and eosin staining will show a thick [[reticular dermis]] with thick [[collagen]] bundles separated by clear spaces. | * A [[skin biopsy]], in which hematoxylin and eosin staining will show a thick [[reticular dermis]] with thick [[collagen]] bundles separated by clear spaces. | ||
==Treatment== | ==Treatment== | ||
Treatment is empiric and include [[corticosteroids]] | Treatment is empiric and include [[corticosteroids]] | ||
== Prognosis == | == Prognosis == | ||
* The symptoms of the condition usually resolve within six months to two years after onset. | * The symptoms of the condition usually resolve within six months to two years after onset. | ||
* However, patients with diabetes may suffer for longer periods of time. | * However, patients with diabetes may suffer for longer periods of time. | ||
* [[Myocarditis]] resulting as a complication from the disease has been successfully treated with [[penicillin]] and [[steroids]] | * [[Myocarditis]] resulting as a complication from the disease has been successfully treated with [[penicillin]] and [[steroids]] | ||
{{rarediseases}} | {{rarediseases}} | ||
{{Medicine}} | {{Medicine}} | ||
[[Category:Connective tissue diseases]] | [[Category:Connective tissue diseases]] | ||
Latest revision as of 00:02, 4 April 2025
| Scleredema | |
|---|---|
| Synonyms | Scleredema adultorum |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Skin thickening, swelling, stiffness |
| Complications | Limited range of motion, difficulty swallowing |
| Onset | Usually adulthood |
| Duration | Chronic |
| Types | N/A |
| Causes | Unknown, possibly related to diabetes mellitus, streptococcal infection |
| Risks | Diabetes, obesity, infection |
| Diagnosis | Clinical diagnosis, skin biopsy |
| Differential diagnosis | Scleroderma, morphea, eosinophilic fasciitis |
| Prevention | N/A |
| Treatment | Physical therapy, corticosteroids, immunosuppressive drugs |
| Medication | N/A |
| Prognosis | Variable, can be self-limiting or progressive |
| Frequency | Rare |
| Deaths | N/A |
Sclerema edematosis, scleredema or Buschke disease is a rare pathological condition of connective tissue.
Other names[edit]
Scleredema adultorum; Scleredema adultorum of Buschke; Scleredema diabeticorum
Pathophysiology[edit]
Scleredema is a form of cutaneous mucinosis, a diverse group of rare skin conditions that are characterized by an accumulation of mucin (a jelly-like complex carbohydrate substance) in the skin.
Cause[edit]
- Its pathogenesis is poorly understood.
- It occurs most commonly following an infectious episode.
- It has also been linked to diabetes mellitus and to hematological disorders.
History[edit]
It was described by Curizo in 1752, and the disease was well defined afterward by Buschke in 1902.
Classification[edit]
It belongs to the spectrum of scleroderma-like disorders.
Clinical features[edit]
- It causes fibro-mucinous progressive induration of the skin, involving the neck, shoulders and proximal upper members and eventually the face.
- Signs and symptoms of this condition include hardening and thickening of the skin which may restrict movement.
- Skin in affected areas may be red or brown and often has an 'orange-skin' appearance.
Types[edit]
There are three forms of the condition which vary by disease course and long term outlook.
- Although the underlying cause is currently unknown, each form is associated with a different condition: infection (type 1), blood abnormalities (type 2), and diabetes (type 3).
- In some cases, scleredema resolves spontaneously on its own, while in other affected people, the condition persists for long periods of time.
- Due to the rarity of the condition, there is no standard treatment
Histology[edit]
Histological features commonly seen include dermal fibrosis with thickened collagen bundles and variable amounts of mucin deposits.
Diagnosis[edit]
- The scleredema is considered as diagnosis based on the appearance of the skin and the patient's medical history.
- A skin biopsy, in which hematoxylin and eosin staining will show a thick reticular dermis with thick collagen bundles separated by clear spaces.
Treatment[edit]
Treatment is empiric and include corticosteroids
Prognosis[edit]
- The symptoms of the condition usually resolve within six months to two years after onset.
- However, patients with diabetes may suffer for longer periods of time.
- Myocarditis resulting as a complication from the disease has been successfully treated with penicillin and steroids
NIH genetic and rare disease info[edit]
Scleredema is a rare disease.
| Rare and genetic diseases | ||||||
|---|---|---|---|---|---|---|
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Rare diseases - Scleredema
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