Work

Scleroedema preceding monoclonal gammopathy by 30 years

Year: 2026

Type: article

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.

Source: European Journal of Case Reports in Internal Medicine

DOI: 10.12890/2026_007288

Author: Ami Schattner

Institutions: Hebrew University of Jerusalem, Hadassah Medical Center

Language: English

Topic: Systemic Sclerosis and Related Diseases

Subfield: Rheumatology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $323