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Case Reports
. 2021 Oct;6(3):311-315.
doi: 10.1177/23971983211013977. Epub 2021 May 12.

Scleredema with biopsy-confirmed cardiomyopathy: A case report

Mei Nakatsuji  1 , Naoto Ishimaru  1 , Jun Ohnishi  1 , Shimpei Mizuki  1 , Yohei Kanzawa  1 , Kei Kawano  1 , Takahiro Nakajima  1 , Masaru Kuroda  2 , Nobuya Sano  3 , Saori Kinami  1
Affiliations
Case Reports

Scleredema with biopsy-confirmed cardiomyopathy: A case report

Mei Nakatsuji et al. J Scleroderma Relat Disord. 2021 Oct.

Abstract

Scleredema is a rare cutaneous mucinosis characterized by diffuse swelling and non-pitting induration. A 63-year-old man reported a 5-year history of skin thickening of the trunk and a 3-week history of dyspnea. Echocardiography revealed diffuse hypokinesis. Skin biopsies obtained from the waist showed thickened dermis with mucin. Myocardial biopsies showed alcian blue-stained tissue between the muscle fibers. The patient was referred to a dermatologist for phototherapy. Cardiomyopathy should be considered in patients with scleredema. Scleredema usually has a good prognosis; however, the mortality risk could be high when accompanied by cardiomyopathy.

Keywords: Scleredema; cardiomyopathy; myocardial biopsy; skin thickening.

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Conflict of interest statement

Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
Widespread thickening of the skin predominantly involving the trunk (a). Non-pitting and ill-defined thickening on the lower back (b).
Figure 2.
Figure 2.
Electrocardiogram showing low voltages in the limb leads and flat T-wave in lead I and aVL (a). Echocardiography (long axis view) showing diffuse hypokinesis with left ventricular ejection fraction of 44.4, diastolic diameter 52.5 mm, systolic diameter 44.5 mm, anteroseptal basal wall 8.5 mm, and posterior wall thickness 11.4 mm (b). Diffuse thickening of the skin and subcutaneous tissue in the lower back. T2-weighted magnetic resonance imaging showing hyperintense signals (c).
Figure 3.
Figure 3.
Incisional skin biopsy from the lower back revealing thickened dermis with large collagen bundles separated by blue-stained mucopolysaccharide deposits with alcian blue stain (a, b). Histopathology of the myocardium showing alcian blue-positive depositions between fibers surrounded by a small number of lymphocytes and fibroblasts (c, d).
See this image and copyright information in PMC

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