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Case Reports
. 2023 Sep 9;15(9):e44968.
doi: 10.7759/cureus.44968. eCollection 2023 Sep.

Scleredema in the Setting of Monoclonal Gammopathy of Unknown Significance With Progression to Multiple Myeloma: A Case Report

Austin L Nickell  1 , Megan Corn  2 , Devendranath Mannuru  3 , Alicia M Hinze  4
Affiliations
Case Reports

Scleredema in the Setting of Monoclonal Gammopathy of Unknown Significance With Progression to Multiple Myeloma: A Case Report

Austin L Nickell et al. Cureus. .

Abstract

Type 2 scleredema on the background of monoclonal gammopathy of undetermined significance (MGUS) is a rare and progressive connective tissue disorder with very few cases reported to date. It is characterized by chronic and diffuse induration of the skin that begins in the upper back and neck and progresses proximally to distally, involving the shoulders, trunk, and arms; the hands are usually spared. Here, we present an unusual case of long-standing scleredema that progressed to involve the hands and fingers. This case was further complicated by new-onset Raynaud's phenomenon, splenomegaly, lymphadenopathy, the development of a plasmacytoma, and eventual progression to multiple myeloma. We highlight the differential diagnoses for his complex presentation, the workup that was completed, and current treatment options.

Keywords: monoclonal gammopathy of undetermined significance (mgus); multiple myeloma; organomegaly; plasmacytoma; raynaud’s phenomenon; scleroderma.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Image of left hand showing thickness of the skin over the fingers, giving the appearance of sclerodactyly.
Figure 2
Figure 2. CT of the abdomen and pelvis showing an enlarged spleen measuring 16.1 cm in greatest dimension
Figure 3
Figure 3. CT of the lumbar spine demonstrating a lytic lesion within the L5 vertebra measuring 40 mm in greatest dimension
Figure 4
Figure 4. Axial PET-CT image showing FDG avidity of the lytic lesion in the L5 vertebral body (solid white arrow)
PET-CT: Positron emission tomography-computed tomography, FDG: F-fluorodeoxyglucose
Figure 5
Figure 5. CT of the abdomen and pelvis in axial view demonstrating an inguinal nodal conglomerate measuring 5.2 cm x 3.2 cm
Figure 6
Figure 6. Timeline of patient’s disease course
MGUS: Monoclonal gammopathy of undetermined significance, IgG: Immunoglobulin G, GERD: Gastroesophageal reflux disease, FNA: Fine-needle aspiration, PET-CT: Positron emission tomography-computed tomography
See this image and copyright information in PMC

References

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