Cutaneous manifestations of monoclonal gammopathy

Claveau, Jean-Sébastien; Wetter, David A.; Kumar, Shaji

doi:10.1038/s41408-022-00661-1

Cited by 23 publications

(13 citation statements)

References 200 publications

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“…Because of the generalized papules and MGUS in this case, the first clinical consideration was scleromyxedema, a subtype of LM, which often has systemic involvement and is even life-threatening. Scleromyxedema is also considered to be a form of monoclonal gammopathy of cutaneous significance because of its high association (>50%) with monoclonal globulin ( 4 , 5 ). However, the histological findings did not support the diagnosis of scleromyxedema.…”

Section: Discussionmentioning

confidence: 99%

Lichen myxedematosus associated with monoclonal gammopathy of undetermined significance: A case report and literature review

Huang

Qian²

2023

Front. Med.

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Lichen myxedematosus (LM) is an idiopathic cutaneous mucinosis disorder, and monoclonal gammopathy of undetermined significance (MGUS) is a preneoplastic plasma cell disease with a monoclonal increase in globulin. Patients with LM combined with monoclonal gammopathy are normally diagnosed with scleromyxedema. However, we report a case of generalized papules combined with MGUS in a 78-year-old man who was eventually diagnosed with atypical or intermediate forms of LM because it only involved the skin, and the pathological type was not consistent with scleromyxedema. Few cases of atypical or intermediate forms of LM have been reported, so the course of atypical or intermediate forms of LM is unpredictable. We report the diagnosis and treatment of a case of atypical forms of LM to discuss the current understanding of the disease, hoping to provide a reference for clinical research on this disease.

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Section: Discussionmentioning

confidence: 99%

Lichen myxedematosus associated with monoclonal gammopathy of undetermined significance: A case report and literature review

Huang

Qian²

2023

Front. Med.

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“…Cutaneous manifestations of MG are classified into several subgroups. Group I conditions are pathologically caused by malignant or clonal plasma cells (for example, polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin disease; POEMS), group II conditions are strongly associated with a MG, group III conditions are anecdotally linked to MG and group IV conditions are related to immunoglobulins or M-proteins that may or may not be clonal 37 .…”

Section: Paraprotein-mediated Tissue Damagementioning

confidence: 99%

Monoclonal gammopathy of increasing significance: time to screen?

et al. 2022

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Monoclonal gammopathy (MG) is a frequently detected clonal B-cell or plasma cell disorder. Importantly, every multiple myeloma (MM) case is preceded by MG. Although clinical algorithms now allow earlier treatment of patients with biomarkers of malignancy before MM-induced tissue damage (CRAB) occurs, most patients are still diagnosed late. It is important to revisit how MG should be managed in clinical practice and whether screening is required. As the prevalence of MG and other medical co-morbidities both rise with increasing age, the degree of contribution of MG to disease states other than malignant progression is often unclear. This can lead to monitoring lapses and under recognition of the organ dysfunction that can occur with monoclonal gammopathy of clinical significance (MGCS). Therefore, models of progression to MM and/or MGCS require further refinement. Whilst currently MG is detected incidentally, a case for screening has been made with ongoing studies in this area. Screening has the potential benefit of earlier detection and prevention of both MGCS and delayed MM presentations, but important drawbacks include the psychosocial impact on individuals and resource burden on healthcare services. MG terminology should transition alongside our increasing understanding of the condition and genomic characterisation that have already begun to revise the MG nomenclature. The biology of MG has been poorly understood and is often inferred from the biology of MM, which is unhelpful. We review the literature and case for MG screening in this paper. In particular, we highlight areas that require focus to establish screening for MG.

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“…The direct toxicity of M-protein, host immune abnormalities, specific cytokines, and PC infiltration can, among other mechanisms, produce severe skin manifestations. Thus, MGSS has been classically divided into four different groups according to the type of clone and the type of association between the cutaneous disorder and the underlying MG [ 69 , 70 ] ( Table 5 ):…”

Section: Monoclonal Gammopathies Of Skin Significancementioning

confidence: 99%

“…Every patient with MG and a new skin lesion of unknown origin should be investigated. In these cases, a skin biopsy and bone marrow examination, in addition to the standard laboratory evaluation of PC dyscrasia, should be performed [ 70 ]. Only two of these disorders will be described to emphasize the complex clinical presentation as a syndrome and the need to fulfil the diagnostic criteria.…”

Section: Monoclonal Gammopathies Of Skin Significancementioning

confidence: 99%

Monoclonal Gammopathies of Clinical Significance: A Critical Appraisal

Ríos-Tamayo

Lahuerta

Martínez‐López

et al. 2022

Cancers

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Monoclonal gammopathies of clinical significance (MGCSs) represent a group of diseases featuring the association of a nonmalignant B cells or plasma cells clone, the production of an M-protein, and singularly, the existence of organ damage. They present a current framework that is difficult to approach from a practical clinical perspective. Several points should be addressed in order to move further toward a better understanding. Overall, these entities are only partially included in the international classifications of diseases. Its definition and classification remain ambiguous. Remarkably, its real incidence is unknown, provided that a diagnostic biopsy is mandatory in most cases. In fact, amyloidosis AL is the final diagnosis in a large percentage of patients with renal significance. On the other hand, many of these young entities are syndromes that are based on a dynamic set of diagnostic criteria, challenging a timely diagnosis. Moreover, a specific risk score for progression is lacking. Despite the key role of the clinical laboratory in the diagnosis and prognosis of these patients, information about laboratory biomarkers is limited. Besides, the evidence accumulated for many of these entities is scarce. Hence, national and international registries are stimulated. In particular, IgM MGCS deserves special attention. Until now, therapy is far from being standardized, and it should be planned on a risk and patient-adapted basis. Finally, a comprehensive and coordinated multidisciplinary approach is needed, and specific clinical trials are encouraged.

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Cutaneous manifestations of monoclonal gammopathy

Cited by 23 publications

References 200 publications

Lichen myxedematosus associated with monoclonal gammopathy of undetermined significance: A case report and literature review

Lichen myxedematosus associated with monoclonal gammopathy of undetermined significance: A case report and literature review

Monoclonal gammopathy of increasing significance: time to screen?

Monoclonal Gammopathies of Clinical Significance: A Critical Appraisal

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