Buschke adult scleroderma as a rare differential stiff-man syndrome diagnosis

Abstract

Stiff-man syndrome is a rare disorder of the central nervous system characterized by muscular rigidity and superimposed spasms. The etiology is still unknown and an autoimmune cause is discussed. Many differential diagnoses of 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. He 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 beginning disorder of swallowing and respiration were observed. Clinical investigation, EMG, cerebrospinal fluid and laboratory findings were all normal, thus excluding a stiff-man syndrome and other neuromuscular diseases. Skin biopsy showed typical changes of Buschke's scleredema adultorum, a rare connective 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 the stiff man syndrome.