Scleroedema preceding monoclonal gammopathy by 30 years

Three decades after the diagnosis of scleroedema (Buschke’s disease), a benign chronic sclerotic skin condition of unknown pathogenesis without systemic involvement, a patient developed secondary gout and normocytic anaemia. Investigations revealed monoclonal gammopathy of undetermined significance, with 1.3 g/dl immunoglobulin G-lambda in the gamma region, a clinically important albeit rare scleroedema-related manifestation, with associated low complement component 4 levels and Coomb’s positivity, but no myeloma or amyloidosis.