Case report: Scleromyxedema associated with a monoclonal gammapathy: Successful treatment with intravenous immunoglobulins

Scleromyxedema is a rare idiopathic fibromucinous disorder characterized by a generalized papular and sclerodermoid cutaneous eruption. Patients often have praraproteinemia and extracutaneous, even lethal, manifestations. Yet the prognostic and therapeutic features of scleromyxedema are poorly docum...

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Published inFrontiers in immunology Vol. 13; p. 1099918
Main Authors Wang, Shang-Shang, Chen, Qin-Yi, Xiang, Lei-Hong
Format Journal Article
LanguageEnglish
Published Switzerland Frontiers Media S.A 13.01.2023
Subjects
Exanthema
Female
Humans
Immunoglobulins, Intravenous - therapeutic use
Immunology
intravenous immunoglobulin therapy
Middle Aged
monoclonal gammapathy
Paraproteinemias - complications
Paraproteinemias - diagnosis
Paraproteinemias - drug therapy
Rare Diseases
sclerodermoid disorders
scleromyxedema
Scleromyxedema - diagnosis
Scleromyxedema - drug therapy
Thalidomide - therapeutic use
treatment
treatment
intravenous immunoglobulin therapy
scleromyxedema
sclerodermoid disorders
monoclonal gammapathy
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Summary:Scleromyxedema is a rare idiopathic fibromucinous disorder characterized by a generalized papular and sclerodermoid cutaneous eruption. Patients often have praraproteinemia and extracutaneous, even lethal, manifestations. Yet the prognostic and therapeutic features of scleromyxedema are poorly documented. High-dose intravenous immunoglobulin (IVIG), used either alone or in conjunction with systemic steroids and/or thalidomide, has been suggested as a first-line treatment. We report the case of a 45-year-old woman diagnosed with scleromyxedema with paraproteinemia that initially did not respond to systemic steroids, retinoids, and thalidomide but greatly improvement in terms of systemic and cutaneous symptoms after treatment with IVIG.
Bibliography:Reviewed by: Alakendu Ghosh, Government of West Bengal, India; Nahide Onsun, Bezmiâlem Vakıf Üniversitesi, Turkey
This article was submitted to Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders, a section of the journal Frontiers in Immunology
Edited by: Magdalena Gabig-Cimińska, Institute of Biochemistry and Biophysics (PAN), Poland
These authors have contributed equally to this work
ISSN:1664-3224
1664-3224
DOI:10.3389/fimmu.2022.1099918