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Review
. 2019 May 14;13(1):145.
doi: 10.1186/s13256-019-2072-1.

Scleredema associated with immunoglobulin A-κ smoldering myeloma: a case report and review of the literature

B S D P Keragala  1 , H M M T B Herath  2 , G H D C Janappriya  1 , B S Dissanayaka  1 , S C Shyamini  1 , D P Liyanagama  1 , Thanushah Balendran  1 , S R Constantine  1 , C N Gunasekera  1
Affiliations
Review

Scleredema associated with immunoglobulin A-κ smoldering myeloma: a case report and review of the literature

B S D P Keragala et al. J Med Case Rep. .

Abstract

Background: Scleredema is a rare sclerodermoid skin condition characterized by diffuse symmetrical thickening of the upper part of the body. Its association with monoclonal gammopathy and myeloma was recently described; very few cases have been reported to date.

Case presentation: A 66-year-old Sri Lankan woman who had been followed in a dermatology unit for 34 years with diffuse systemic sclerosis presented with an acute exacerbation of the skin disease. Absence of Raynaud's phenomenon; sclerodactyly; characteristic lung, gastrointestinal, and cardiac involvement of systemic sclerosis; and repeatedly negative antinuclear antibodies test results led to reevaluation for the possibility of scleredema. Skin biopsies from four body sites showed normal epidermis and thickened reticular dermis with swollen collagen bundles separated from one another by clear spaces, resulting in fenestration. The skin appendages were not atrophied or bound down. Alcian blue staining showed interstitial mucin deposition. Serum protein electrophoresis demonstrated an abnormal monoclonal band in the β-region with a paraprotein level of 8.9 g/dl. Immunofixation showed an abnormal band in the γ-region consisting of immunoglobulin A and κ. Bone marrow biopsy revealed abnormal monoclonal plasma cells (15%) with multinuclearity. There was no evidence of end organ damage, and whole-body magnetic resonance imaging did not reveal any evidence of bone involvement. The patient's diagnosis was revised as scleredema type 2 associated with IgA-κ, and she was referred to a hemato-oncologist for chemotherapy, which led to significant improvement in the skin condition.

Conclusions: Scleredema is a rare disorder that has an enigmatic, rare association with monoclonal gammopathy. Dermatologists should be aware of this rare but important association.

Keywords: Monoclonal gammopathy; Scleredema; Smoldering myeloma.

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Ethics approval and consent to participate

Not applicable.

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Competing interests

The authors declare that they have no competing interests.

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Figures

Fig. 1
Fig. 1
Widespread thickening of the skin predominantly involving the trunk and proximal extremities
Fig. 2
Fig. 2
The patient did not have sclerodactyly but had deformities in keeping with osteoarthritis
Fig. 3
Fig. 3
Normal whole-body magnetic resonance imaging
See this image and copyright information in PMC

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