Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2025 Oct;175(13-14):309-310.
doi: 10.1007/s10354-025-01095-7. Epub 2025 Jul 7.

Periocular purpura as a manifestation of amyloid light-chain amyloidosis

Affiliations
Case Reports

Periocular purpura as a manifestation of amyloid light-chain amyloidosis

Daniel Craus et al. Wien Med Wochenschr. 2025 Oct.

Abstract

This report emphasizes the importance of a thorough clinical examination for detecting bilateral periocular purpura, a key clinical hallmark of amyloid light-chain (AL) amyloidosis. Amyloid light-chain amyloidosis, often secondary to plasma cell dyscrasia, results from amyloid light-chain deposition, potentially leading to multiorgan involvement with progressive damage. Diagnosis relies on histopathology with Congo red staining, serum protein studies, and bone marrow analysis. Early recognition and treatment of the underlying hematologic malignancy are crucial for improving patient outcomes.

Keywords: Amyloid light-chain amyloidosis; Congo red staining; Plasma cell dyscrasia; Raccoon sign.

PubMed Disclaimer

Conflict of interest statement

Declarations. Conflict of interest: D. Craus, A. Kreuter, F. Bertling, S. Theodoropoulou, and V.L. Müller declare that they have no competing interests. Ethical standards: For this article no studies with human participants or animals were performed by any of the authors. All studies cited were in accordance with the ethical standards indicated in this case. Additional written informed consent was obtained from the patient for publishing this article, also for publication of the identifying information.

References

    1. Gillmore JD, Wechalekar A, Bird J, et al. Guidelines on the diagnosis and investigations of AL amyloidosis. Br J Haematol. 2015;168(2):207–18. - DOI - PubMed
    1. Real de Asúa D, Costa R, Galván JM, et al. Systemic AA amyloidosis: epidemiology, diagnosis, and management. Clin Epidemiol. 2014;6:369–77. - DOI - PubMed - PMC
    1. Falk RH, Comenzo RL, Skinner M. The systemic amyloidoses. N Engl J Med. 1997;337(13):898–909. - DOI - PubMed
    1. Schönland SO. Progress in the diagnosis and treatment of amyloidosis. Dtsch Ärztebl Int. 2006;103(34):A2237–A44.

Publication types

Substances

LinkOut - more resources