We report on a 54-year-old patient with an 11-year history of Arndt-Go
ttron scleromyxoedema. We found typical lichenoid papules, diffuse ski
n thickening and skin hardening. Clinical and laboratory investigation
s revealed a monoclonal gammopathy of the IgG1 type, normal histology
of the bone marrow and normal urine. Therefore, a diagnosis of monoclo
nal gammopathy of undetermined significance was recorded. Phimosis and
stenosis of the urethra were the only other pathologic findings. Syst
emic treatment with chlorambucil and PUVA had little beneficial effect
on the skin thickening after 9 months.