Scleroedema adultorum Buschke as a rare differential diagnosis of the Stiff-man syndrome

Stiff-man syndrome is a rare disorder of the central nervous system charact erized by muscular rigidity and superimposed spasms. The etiology is still unknown and an autoimmune cause is discussed. Many differential diagnoses o f muscular rigidity and of induration of the skin have to be considered. A 22 year old patient was admitted under suspicion of a stiff-man syndrome. H e has had developed a painful rigidity of the trunk, starting in the neck. Massive stiffness of the trunk with pale appearance of the skin and a begin ning disorder of swallowing and respiration were observed. Clinical investi gation, EMG, cerebrospinal fluid and laboratory findings were all normal,th us excluding a stiff-man syndrome and other neuromuscular diseases. Skin bi opsy showed typical changes of Buschke's scleredema adultorum, a rare conne ctive tissue disorder of unknown etiology. Therapy with corticosteroide and ciclosporin led to a mild improvement of symptoms. Although rare, Buschke' s scleredema adultorum should be regarded as a differential diagnosis of th e stiff man syndrome.