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New or Unusual Skin Manifestations in Monoclonal Gammopathies

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New and Emerging Entities in Dermatology and Dermatopathology

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Abstract

Skin manifestations associated with monoclonal gammopathy (MG) are common and can present with various clinical and pathological aspects. They can be the first clinical manifestation leading to the diagnosis of MG. Patients may have an underlying hemopathy (multiple myeloma and Waldenström’s disease in particular) or an MGUS, and the “monoclonal gammopathy of cutaneous significance” (MGCS) concept has been introduced in the literature. They can represent specific lesions, including lymphoplasmacytic or pure plasma cell neoplastic infiltrates and monoclonal immunoglobulin deposits, either in the vessels (type I cryoglobulinemia) or in extravascular sites (AL amyloidosis, macroglobulinosis, and follicular spicules). Many other skin manifestations are associated with MG, but can also develop in patients without an underlying MG, including neutrophilic infiltrates (Schnitzler’s syndrome), mucinoses (scleromyxedema and scleredema), or xanthomatoses. A better understanding of the pathophysiology of these diseases (biological activity of the monoclonal Ig in xanthomas and acquired angioneurotic edema, IL1 in Schnitzler’s syndrome, VEGF in POEMS) allowed recent improvement in the treatment of the patients.

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Author information

Authors and Affiliations

  1. Department of Pathology, Hopital Henri Mondor, Assistance-Publique – Hopitaux de Paris, Creteil, France

    Nicolas Ortonne

  2. Department of Dermatology, Hopital Henri Mondor, Assistance-Publique – Hopitaux de Paris, Creteil, France

    Saskia Ingen-Housz-Oro

Authors
  1. Nicolas Ortonne
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  2. Saskia Ingen-Housz-Oro
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Correspondence to Nicolas Ortonne .

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Editors and Affiliations

  1. Unit of Clinical Dermatology, Vita-Salute S.Raffaele University, Milan, Italy

    Franco Rongioletti

  2. Department of Pathology, University of Rochester, Rochester, NY, USA

    Bruce R. Smoller

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Ortonne, N., Ingen-Housz-Oro, S. (2021). New or Unusual Skin Manifestations in Monoclonal Gammopathies. In: Rongioletti, F., Smoller, B.R. (eds) New and Emerging Entities in Dermatology and Dermatopathology. Springer, Cham. https://doi.org/10.1007/978-3-030-80027-7_20

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  • DOI: https://doi.org/10.1007/978-3-030-80027-7_20

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  • Online ISBN: 978-3-030-80027-7

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