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A skin and connective tissue condition characterized by nonpitting woody induration of the skin typically involving the nape of the neck, interscapular region and upper and mid back and sometimes the face, upper chest, and upper arms.
The distal extremities are usually spared.
Scleredema
Scleredema, also known as Buschke disease, scleredema of Buschke, and scleredema adultorum,
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.
The skin may also change color to red or orange.
Usually associated with diabetes, a viral illness or strep throat.
It is usually not fatal, but it may cause death if it spreads to the internal organs.
May cause infection.
Diagnosis based on the appearance of the skin and medical history.
Confirmation of the diagnosis requires a skin biopsy, in which hematoxylin and eosin staining will show a thick reticular dermis with thick collagen bundles separated by clear spaces.
No treatment has been proven effective in treating scleredema.
Corticosteroids, may be beneficial, but is not curable.
Symptoms usually resolve within six months to two years after onset.
In patients with diabetes symptoms may persist for longer periods of time.
Marked facial involvement can lead to periorbital edema.
Associated with four clinical patterns: after certain infections infection is known as scleredema of Buschke, associated with circulating paraproteins, an idiopathic form, and associated with diabetes mellitus, referred to as scleredema diabeticorum.
All subtypes have similar histopathology.
Cultured dermal fibroblasts show increase collagen production and elevated type I procollagen messenger RNA levels.
Prevalence of scleredema diabeticorum ranges from 2 1/2-14% among diabetics,but it is und2242ecognized.
Sclerederma diabeticorum is generally poorly controlled and complicated by retinopathy and neuropathy.
Starts insidiously and progresses gradually.
Mean age of onset of scleredema diabeticorum is 54 years and mean duration of diabetes is 13 years.
Skin biopsy shows thickening of the reticular dermis, due to increased deposition of collagen which extends into the subcutis.