Scleredema adultorum or
Buschke's scleredema is a rare disorder that belongs to the group of
mucinoses. Diffuse, sudden swelling, hardening and induration of the skin can occur in children and younger women as well as in older men. The dermis is thickened because of the increased
collagen glycosylation, like that in diabetic
stiff skin syndrome. The face is most often involved. In older persons the trunk is usually first involved. There is relative sparing of the extremities, with no distal or Raynaud's phenomenon.
Eosinophilic fasciitis,
scleromyxedema, associated gammopathy, and other forms of
edema and
mucin deposition must be excluded on differential diagnosis.
Antibiotics, sometimes high doses of intravenous
penicillin, systemic
corticosteroids, systemic PUVA and PUVA bath
therapy seem most promising for the management of the disorder. Our patients were aged 27, 60, 64, 69 and 72 years, with typical skin lesions, thus that term "adultorum" does not appear to fit well.