Scleredema
Sclerema edematosis, scleredema or Buschke disease is a rare pathological condition of connective tissue.
Other names[edit | edit source]
Scleredema adultorum; Scleredema adultorum of Buschke; Scleredema diabeticorum
Pathophysiology[edit | edit source]
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 | edit source]
- 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 | edit source]
It was described by Curizo in 1752, and the disease was well defined afterward by Buschke in 1902.
Classification[edit | edit source]
It belongs to the spectrum of scleroderma-like disorders.
Clinical features[edit | edit source]
- 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 | edit source]
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 | edit source]
Histological features commonly seen include dermal fibrosis with thickened collagen bundles and variable amounts of mucin deposits.
Diagnosis[edit | edit source]
- 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 | edit source]
Treatment is empiric and include corticosteroids
Prognosis[edit | edit source]
- 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 source]
Scleredema is a rare disease.
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