[Arndt-Gottron scleromyxedema. Summary of 2 years treatment].
The case of a 53-year-old patient with a 2-year history of Arndt-Gottron scleromyxoedema is reported. Typical lichenoid papules were found clinically, along with infiltration of acid mucopolysaccharides into the skin, which induces extensive elephantine-like thickening and hardening of the skin. The presence of abnormal paraproteins is to be rated as the criterion for the diagnosis of scleromyxoedema. This type of paraproteinaemia is described as a monoclonal gammopathy of undetermined significance. We found no paraproteinaemia in our patient; bone marrow histology and urinalysis were normal. The oetiopathogenesis of the disease is unknown. Pathohisto-logical examination corroborates the presumption that fibro-blasts are producing acid mucopolysaccharides and that collagen fibres play an important role in inducing the infiltration of mucinous material into the skin. We treated our patient for 6 months with cyclosporin A and observed regression of the extensively thickened skin; it appeared smooth, especially over the region of the deltoid muscles and shoulders. The flexibility of the finger and mandible joints showed an improvement, but the neck was still stiff and painful. The patient complained of "burning" pain in the neck and back. Serum factors able to stimulate fibroblasts in scleromyxoedema are the cytokines IL-1 and TNF-alpha. Cyclosporin A inhibits both these substances and also inhibits the activation of helper T-cells, which express lymphokines.
Krajnc
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Abteilung für Innere Medizin-Rheumatologie, Lehrklinik Maribor, Slowenien.
D016572:Cyclosporine / Q000008:administration & dosage* / Q000009:adverse effects; D003937:Diagnosis, Differential; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage* / Q000009:adverse effects; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage* / Q000009:adverse effects; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology; D012867:Skin / Q000473:pathology
D007166:Immunosuppressive Agents; D016572:Cyclosporine; D008775:Methylprednisolone
Skleromyxoedema--Arndt-Gottron. Ubersicht einer 2jährigen Behandlung.