SCLEROMYXEDEMA ARNDT-GOTTRON - A CASE-REP ORT OVER A 2-YEAR TREATMENTPERIOD

The case of a 53-year-old patient with a 2-year history of Arndt-Gottr on scleromyxoedema is reported. Typical lichenoid papules were found c linically, along with infiltration of acid mucopolysaccharides into th e skin, which induces extensive elephantine-like thickening and harden ing of the skin. The presence of abnormal paraproteins is to be rated as the criterion for the diagnosis of scleromyxoedema. This type of pa raproteinaemia is described as a monoclonal gammopathy of undetermined significance. We found no paraproteinaemia in our patient; bone marro w histology and urinalysis were normal. The oetiopathogenesis of the d isease is unknown. Pathohisto-logical examination corroborates the pre sumption that fibro-blasts are producing acid mucopolysaccharides and that collagen fibres play an important role in inducing the infiltrati on of mucinous material into the skin. We treated our patient for 6 mo nths with cyclosporin A and observed regression of the extensively thi ckened skin; it appeared smooth, especially over the region of the del toid muscles and shoulders. The flexibility of the finger and mandible joints showed an improvement, but the neck was still stiff and painfu l. The patient complained of ''burning'' pain in the neck and back. Se rum factors able to stimulate fibroblasts in scleromyxoedema are the c ytokines IL-l and TNF-alpha. Cyclosporin A inhibits both these substan ces and also inhibits the activation of helper T-cells, which express lymphokines.