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Journal of Cutaneous Pathology May 2024Lichen myxedematosus (LM) is a chronic cutaneous mucinosis that can present as a localized skin lesion or as a generalized systemic disease termed scleromyxedema. The...
Lichen myxedematosus (LM) is a chronic cutaneous mucinosis that can present as a localized skin lesion or as a generalized systemic disease termed scleromyxedema. The differential diagnosis is determined by a combination of clinical presentation, serological studies, and histopathological examination. Currently, well-established and accepted histopathological features to distinguish localized LM from scleromyxedema have not been elucidated. Our recent publication, together with a retrospective literature review, suggests that the presence of groups of light chain-restricted plasma cells represents a distinct histopathological clue for the diagnosis of localized LM. In this report, we provide two additional cases of localized LM with lambda light chain-restricted plasma cells, together with clinical and histopathological findings that are similar to our previous publication. These cases support our theory that the light chain-restricted plasmacytic microenvironment is primarily attributed to the pathogenesis of localized LM. Therefore, we consider these cases to constitute a clinically and pathologically new variant of localized LM and name it primary localized cutaneous LM with light chain-restricted plasma cells.
PubMed: 38699948
DOI: 10.1111/cup.14624 -
Leukemia & Lymphoma Apr 2024Monoclonal Gammopathy of Undetermined Significance (MGUS) is a clonal plasma cell disorder that is considered preneoplastic, asymptomatic, and only requiring...
Monoclonal Gammopathy of Undetermined Significance (MGUS) is a clonal plasma cell disorder that is considered preneoplastic, asymptomatic, and only requiring observation. However, MGUS may result in cutaneous complications, which are poorly understood, causing treatment delays and patient suffering. We present 30 patients with cutaneous findings associated with MGUS, characterizing clinical presentations, isoforms, treatments, and outcomes. These included: MGUS-associated 'rashes' (pruritic eczematous rashes), reactive and mucin-depositional conditions (pyoderma gangrenosum, scleromyxedema), M-protein-related deposition disorders (POEMS syndrome, Waldenstrom macroglobulinemia), and cutaneous lymphomas. Twelve of 30 (40%) patients received multiple myeloma drugs (MMDs). Eleven (92%) patients improved, and those not receiving MMDs rarely improved, suggesting that MMDs have efficacy for cutaneous manifestations of MGUS. Therefore, trialing MMDs may be warranted for patients with MGUS not responding to other therapies. Moreover, evaluation for monoclonal gammopathy in elderly patients with intractable pruritus or other chronic skin conditions that are non-responsive to skin-directed therapies should be considered.
PubMed: 38581379
DOI: 10.1080/10428194.2024.2336046 -
The Australasian Journal of Dermatology May 2024
Topics: Humans; Scleromyxedema
PubMed: 38528703
DOI: 10.1111/ajd.14259 -
British Journal of Haematology Mar 2024
PubMed: 38471663
DOI: 10.1111/bjh.19366 -
Journal of the European Academy of... Jul 2024The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest... (Review)
Review
The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present consensus provides an update to the 2017 European Dermatology Forum Guidelines, focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, updated strategies for the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 2 of this consensus provides clinicians with an overview of the diagnosis and treatment of scleromyxoedema and scleroedema (of Buschke).
Topics: Humans; Scleromyxedema; Consensus; Diagnosis, Differential
PubMed: 38456518
DOI: 10.1111/jdv.19937 -
Dermatologie (Heidelberg, Germany) Mar 2024Scleromyxedema or generalized diffuse lichen myxoedematosus is a rare mucinosis that is associated with monoclonal gammopathy and which frequently affects multiple... (Review)
Review
Scleromyxedema or generalized diffuse lichen myxoedematosus is a rare mucinosis that is associated with monoclonal gammopathy and which frequently affects multiple extracutaneous organ systems. The pathogenesis of scleromyxedema has not been fully elucidated, but includes stimulation of glycosaminoglycan synthesis. The clinical course of scleromyxedema is chronic and often progressive, leading to severe morbidity and even death. The characteristic skin findings encompass multiple waxy papules often on indurated plaques, while thickening of skin leads to conspicuous folds on glabella and dorsal aspects of finger joints. Microscopical manifestations are dermal deposits of glycosaminoglycans between collagen bundles in reticular dermis, increased numbers of fibroblasts and fibrosis as well as loss of elastic fibers. Progressive skin involvement results in decreased mobility of the mouth and joints and even contractures. Extracutaneous manifestations occur in the musculoskeletal or cardiovascular system, in the gastrointestinal or respiratory tract, in the kidneys or in the central and peripheral nervous system. There are no in-label or evidence-based treatments available for scleromyxedema, but by expert consensus high-dose immunoglobulins are considered as treatment of choice, followed in case of insufficient efficacy by systemic glucocorticosteroids and then lenalidomide or thalidomide. In severe and refractory cases, autologous hematopoietic stem cell transplantation has been performed. Long-term maintenance treatment is usually required to prevent recurrences. Close interdisciplinary follow-up is recommended.
Topics: Humans; Scleromyxedema; Skin; Lenalidomide; Thalidomide; Dermis
PubMed: 38363313
DOI: 10.1007/s00105-024-05303-0 -
The Australasian Journal of Dermatology Jun 2024
Topics: Humans; Multiple Myeloma; Lenalidomide; Dexamethasone; Scleromyxedema; Antibodies, Monoclonal; Male; Female; Thalidomide; Aged; Antineoplastic Combined Chemotherapy Protocols; Middle Aged
PubMed: 38326991
DOI: 10.1111/ajd.14218 -
Mayo Clinic Proceedings Feb 2024
Topics: Humans; Multiple Myeloma; Scleromyxedema; Paraproteinemias; Skin
PubMed: 38309938
DOI: 10.1016/j.mayocp.2023.08.022 -
Italian Journal of Dermatology and... Feb 2024
Topics: Humans; Scleromyxedema; Immunoglobulins, Intravenous; Granuloma Annulare; Skin
PubMed: 38226941
DOI: 10.23736/S2784-8671.23.07816-7 -
Journal Der Deutschen Dermatologischen... Mar 2024
Unusual variant of scleromyxedema presenting with severe systemic involvement and atypical adnexal proliferations - A histological pitfall with the risk of unnecessary surgeries.
Topics: Humans; Scleromyxedema; Granuloma Annulare; Diagnosis, Differential
PubMed: 38200613
DOI: 10.1111/ddg.15306