Scleroedema Preceding Monoclonal Gammopathy by 30 Years.
- 1The Faculty of Medicine, Hebrew University and Hadassah Medical School, Jerusalem, Israel.
Abstract
Three decades after the diagnosis of scleroedema (Buschke's disease), a benign chronic sclerotic skin condition of unknown pathogenesis without systemic involvement, a patient developed secondary gout and normocytic anaemia. Investigations revealed monoclonal gammopathy of undetermined significance, with 1.3 g/dl immunoglobulin G-lambda in the gamma region, a clinically important albeit rare scleroedema-related manifestation, with associated low complement component 4 levels and Coomb's positivity, but no myeloma or amyloidosis.Learning points
Scleroedema (of Buschke) is an unusual sclerotic skin condition grouped together with localised scleroderma, systemic sclerosis, and scleromyoedema.Scleroedema has three important systemic associations: a preceding streptococcal infection, type 2 diabetes, and monoclonal gammopathy of undetermined significance (MGUS).Scleroedema can precede the appearance of monoclonal gammopathy by decades, and these patients need to be monitored for the development of plasma cell disorder, from MGUS to multiple myeloma.
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BibTeX
@article{schattner2026scleroedema,
title = {{Scleroedema Preceding Monoclonal Gammopathy by 30 Years.}},
author = {Ami Schattner},
journal = {European journal of case reports in internal medicine},
year = {2026},
month = oct,
doi = {10.12890/2026_007288},
url = {https://europepmc.org/article/MED/43012021},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Scleroedema Preceding Monoclonal Gammopathy by 30 Years.
AU - Ami Schattner
PY - 2026
DA - 2026/10/16
JO - European journal of case reports in internal medicine
DO - 10.12890/2026_007288
UR - https://europepmc.org/article/MED/43012021
AB - Three decades after the diagnosis of scleroedema (Buschke's disease), a benign chronic sclerotic skin condition of unknown pathogenesis without systemic involvement, a patient developed secondary gout and normocytic anaemia. Investigations revealed monoclonal gammopathy of undetermined significance, with 1.3 g/dl immunoglobulin G-lambda in the gamma region, a clinically important albeit rare scleroedema-related manifestation, with associated low complement component 4 levels and Coomb's positivity, but no myeloma or amyloidosis.Learning points Scleroedema (of Buschke) is an unusual sclerotic skin condition grouped together with localised scleroderma, systemic sclerosis, and scleromyoedema.Scleroedema has three important systemic associations: a preceding streptococcal infection, type 2 diabetes, and monoclonal gammopathy of undetermined significance (MGUS).Scleroedema can precede the appearance of monoclonal gammopathy by decades, and these patients need to be monitored for the development of plasma cell disorder, from MGUS to multiple myeloma.
SN - 2284-2594
ER -