Scleredema

Sclerema edematosis, scleredema or Buschke disease is a rare pathological condition of connective tissue.

Other names

Scleredema adultorum; Scleredema adultorum of Buschke; Scleredema diabeticorum

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.

Cause

• 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

It was described by Curizo in 1752, and the disease was well defined afterward by Buschke in 1902.

Classification

It belongs to the spectrum of scleroderma-like disorders.

Clinical features

• 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

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

Histological features commonly seen include dermal fibrosis with thickened collagen bundles and variable amounts of mucin deposits.

Diagnosis

• 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

Treatment is empiric and include corticosteroids

Prognosis

• 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

• NIH info on Scleredema

Scleredema is a rare disease.