Recenzija, Prikaz slučaja
Long-Term Efficacy of Treatment with Intravenous Immunoglobulin in Scleromyxedema
Špela Baglama
; Department of Dermatology and Venereal Diseases, University Medical Centre Maribor
Katarina Trčko
; Department of Dermatology and Venereal Diseases, University Medical Centre Maribor
Sažetak
Scleromyxedema or generalized lichen myxedematosus
is a rare depositional disorder. Diagnostic criteria encompass
a generalized papular and sclerodermoid eruption, monoclonal
gammopathy (paraproteinemia), most often with G-lambda type
immunoglobulin, a characteristic microscopic triad (mucin deposition,
fibroblast proliferation, fibrosis), and absence of thyroid
disease. Many internal manifestations of scleromyxedema have
been described to date, leading to high mortality and morbidity.
Because the disease is rare, the etiology is not fully understood and
there is a lack of well-designed studies, so no optimal treatment exists
so far. This paper reports the follow-up on a patient in 5.5-year
remission after successful intravenous immunoglobulin therapy
10.5 years since initial diagnosis.
Ključne riječi
scleromyxedema, mucinoses, therapy, intravenous immunoglobulin
Hrčak ID:
246737
URI
Datum izdavanja:
18.1.2020.
Posjeta: 571 *