Immunohistochemical investigations and introduction of new therapeutic strategies in scleromyxoedema: case report.
BACKGROUND
Scleromyxoedema is a rare chronic skin disease of obscure origin, which may often be associated with severe internal co-morbidity. Even though different casuistic treatment modalities have been described, to date, curing still seems to be impossible.


METHODS
We report a 44-year-old Caucasian female presenting with remarkable circumscribed, erythematous to skin-coloured, indurated skin eruptions at the forehead, arms, shoulders, legs and the gluteal region. Routine histology and Alcian blue labelling confirmed a massive deposition of acid mucopolysaccharides. Immunohistochemical investigations revealed proliferating fibroblasts and a discrete lymphocytic infiltration as well as increased dermal expression of MIB-1+ and anti-mastcell-tryptase+ cells. Bone marrow biopsies confirmed a monoclonal gammopathy of undetermined significance without morphological characteristics of plasmocytoma; immunofixation unveiled the presence of IgG-kappa paraproteins.


CONCLUSIONS
Taking all data into account, our patient exhibited a complex form of lichen mxyoedematosus, which could most likely be linked a variant of scleromyxoedema. Experimental treatment with methotrexate resulted in a stabilisation of clinical symptoms but no improvement after five months of therapy. A subsequent therapeutic attempt by the use of medium-dose ultraviolet A1 cold-light photomonotherapy led to a further stabilisation of clinical symptoms, but could not induce a sustained amelioration of skin condition.
Breuckmann
Frank
F
Department of Dermatology, Ruhr-University Bochum, Gudrunstrasse 56, D-44791 Bochum, Germany. Frank.Breuckmann@ruhr-uni-bochum.de.
Freitag
Marcus
M
Rotterdam
Sebastian
S
Stuecker
Markus
M
Altmeyer
Peter
P
Kreuter
Alexander
A
D000328:Adult; D002908:Chronic Disease; D004890:Erythema / Q000209:etiology; D005260:Female; D005492:Folic Acid / Q000008:administration & dosage; D006025:Glycosaminoglycans / Q000032:analysis*; D006801:Humans; D007150:Immunohistochemistry; D008727:Methotrexate / Q000008:administration & dosage; D009230:Myxedema / Q000473:pathology* / Q000628:therapy; D010789:Phototherapy; D012594:Scleroderma, Localized / Q000473:pathology* / Q000628:therapy; D012867:Skin / Q000737:chemistry*
D006025:Glycosaminoglycans; D005492:Folic Acid; D008727:Methotrexate