Avtor/Urednik		Krajnc, Ivan
Naslov		Skleromyxoedema - Arndt-Gottron: Uebersicht eineer 2jaehrigen Behandlung
Prevedeni naslov		Scleromyxoedema Arndt-Gottron: a case report over 2-year treatment period
Tip		članek
Vir		Wien Klin Wochenschr
Vol. in št.		Letnik 109, št. 24
Leto izdaje		1997
Obseg		str. 960-3
Jezik		ger
Abstrakt		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 ifiltration 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 to 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 ininducing the infiltration of mucinous material into the skin. We treated our patient fo 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 schoulders. 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.
Deskriptorji		LICHENOID ERUPTIONS MYXEDEMA PARAPROTEINS MIDDLE AGE PARAPROTEINEMIAS CYCLOSPORINE TREATMENT OUTCOME