Buschke's scleredema and concomitant diseases: report of five cases and literature review

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.