-
Seminars in Oncology Jun 2016Plasma cell proliferative disorders cause rare but extremely varied dermatologic manifestations that may occur as an accompaniment to established diagnoses, or may be a... (Review)
Review
Plasma cell proliferative disorders cause rare but extremely varied dermatologic manifestations that may occur as an accompaniment to established diagnoses, or may be a first clue of an underlying neoplasm in the setting of clinical suspicion. In some instances skin lesions result from aggregation of misfolded monoclonal immunoglobulins or their fragments, as in light chain-related systemic amyloidosis. On other occasions the cutaneous lesions result from deposits of malignant plasma cells or monoclonal proteins. In still others, the dermatologic manifestations are related to antibody activity of monoclonal protein, as in many cases of cryoglobulinemia. This report provides insights into the well-recognized cutaneous manifestations associated with plasma cell disorders.
Topics: Amyloidosis; Cryoglobulinemia; Humans; Multiple Myeloma; Myeloproliferative Disorders; POEMS Syndrome; Plasma Cells; Plasmacytoma; Schnitzler Syndrome; Scleromyxedema; Skin Diseases, Infectious; Skin Neoplasms; Xanthomatosis
PubMed: 27178694
DOI: 10.1053/j.seminoncol.2016.02.017 -
Clinical Laboratory Mar 2021Scleromyxedema (SME) is a rare mucinosis associated with monoclonal gammopathy. Several biochemical peculiarities of monoclonal immunoglobulins (Ig) in SME patients were...
BACKGROUND
Scleromyxedema (SME) is a rare mucinosis associated with monoclonal gammopathy. Several biochemical peculiarities of monoclonal immunoglobulins (Ig) in SME patients were reported in case reports or short series, such as IgGλ over-representation, cationic migration, and partial deletion.
METHODS
Monoclonal immunoglobulins (Ig) from the serum of 12 consecutive patients diagnosed with scleromyxedema (SME) were analyzed using electrophoretic and immunoblotting techniques.
RESULTS
All monoclonal Ig from 12 SME were of IgG1 subclass, with an overrepresentation of the lambda-type light chain and a cationic mobility on standard zone electrophoresis, as compared with 21 cases of monoclonal gammopathy of undetermined significance (MGUS) of IgG1 subclass. Reactivity with specific monoclonal antibodies demonstrated no evident deletion of the heavy chain constant domains, which was also confirmed by analysis of Ig heavy chain molecular weight on a purified monoclonal component from one case.
CONCLUSIONS
Significant isotype restriction of both heavy and light chains, and peculiar biochemical properties suggest that monoclonal Ig might be involved in pathophysiological events of SME.
Topics: Antibodies, Monoclonal; Humans; Immunoglobulin G; Immunoglobulin lambda-Chains; Paraproteinemias; Scleromyxedema
PubMed: 33739037
DOI: 10.7754/Clin.Lab.2020.200605 -
Clinical Case Reports Dec 2020Scleromyxedema is a rare and progressive disease that currently has no standard treatment. Triplet therapy with lenalidomide, bortezomib, and dexamethasone can be an...
Scleromyxedema is a rare and progressive disease that currently has no standard treatment. Triplet therapy with lenalidomide, bortezomib, and dexamethasone can be an effective therapy for scleromyxedema, especially in patients with refractory or relapsed disease.
PubMed: 33363876
DOI: 10.1002/ccr3.3302 -
Dermatology Online Journal Oct 2018Cutaneous mucinosis of infancy (CMI) is a rare dermatologic condition, first reported in 1980 and currently classified within the complex group of papular mucinoses. We... (Review)
Review
Cutaneous mucinosis of infancy (CMI) is a rare dermatologic condition, first reported in 1980 and currently classified within the complex group of papular mucinoses. We report a case of CMI and review the prior 13 cases in the literature. The patient was a 5-year-old girl who presented with asymptomatic dermal papules and plaques on her leg and back with no overlying color change. These lesions were first noticed during infancy and had become slightly more evident over time. The patient had a history of birthmarks and eczema. Her family history included eczema, allergies, photosensitivity, and Graves disease. Pre-biopsy clinical differential diagnosis included connective tissue nevus, granuloma annulare, myofibroma, lipofibroma, and lymphangioma. Biopsies revealed significant increase in interstitial mucin within the reticular and mid dermis, without significant sclerosis or fibroblastic proliferation. The relatively quiescent pattern of interstitial mucinosis with slight fibrocyte hyperplasia presenting as dermal papules-plaques on the trunk and extremities was most consistent with a diagnosis of CMI. We report another case of CMI in an otherwise healthy patient. Our patient is unique as she is the first CMI patient with a family history of Graves disease, although our patient appeared euthyroid. We will also review the literature on this rare entity.
Topics: Biopsy; Child, Preschool; Diagnosis, Differential; Female; Granuloma Annulare; Graves Disease; Humans; Medical History Taking; Mucinoses; Myofibroma; Nevus; Scleromyxedema; Skin Neoplasms
PubMed: 30677812
DOI: No ID Found -
Annales de Pathologie Oct 2018
Review
Topics: Adult; Biopsy; Diagnosis, Differential; Female; Granuloma Annulare; Humans; Lyme Disease; Mycosis Fungoides; Scleroderma, Localized; Scleromyxedema; Syphilis
PubMed: 30236823
DOI: 10.1016/j.annpat.2018.09.001 -
Acta Dermatovenerologica Croatica : ADC Apr 2021Scleromyxedema is a generalized cutaneous mucinosis that may cause internal damage. This condition is frequently associated with monoclonal gammopathy. However, its...
Scleromyxedema is a generalized cutaneous mucinosis that may cause internal damage. This condition is frequently associated with monoclonal gammopathy. However, its physiopathological implications remains uncertain. The natural development of scleromyxedema is unpredictable and may lead to potentially fatal complications. Although there is no standardized treatment, intravenous immunoglobulins are considered the best method for treating scleromyxedema. The effects of this method of treatment on this condition are not well known, and it could be argued that intravenous immunoglobulins interact with the monoclonal gammopathy. This paper describes a case of scleromyxedema without associated monoclonal gammopathy that was treated effectively using monthly courses of treatment with intravenous immunoglobulins.
Topics: Humans; Immunoglobulins, Intravenous; Paraproteinemias; Scleromyxedema; Skin
PubMed: 34477065
DOI: No ID Found -
Rheumatology International Nov 2020Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare childhood disease with characteristic cutaneous and rheumatic manifestations. Cutaneous manifestations... (Review)
Review
Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare childhood disease with characteristic cutaneous and rheumatic manifestations. Cutaneous manifestations include a combination of nodules affecting peri-articular (especially interphalangeal joints) and head and neck areas; and linearly arranged ivory white papules over an erythematous indurated skin. Despite a benign course, an abrupt onset of symptoms with extensive cutaneous involvement often leads to parental anxiety, overenthusiastic evaluation and sometimes aggressive treatment. A peculiar cutaneous distribution in SHJCM including nodular lesions and periorbital edema, arthritis and arthralgia in a few cases, may simulate juvenile dermatomyositis. It is, therefore, important for dermatologists and pediatricians to be aware of this entity. In this report, we describe two cases of SHJCM and briefly review similarly reported cases in children.
Topics: Child, Preschool; Dermatomyositis; Diagnosis, Differential; Female; Humans; Male; Mucinoses; Skin
PubMed: 32462254
DOI: 10.1007/s00296-020-04578-5 -
The Australasian Journal of Dermatology May 2024
Topics: Humans; Scleromyxedema
PubMed: 38528703
DOI: 10.1111/ajd.14259 -
Indian Dermatology Online Journal 2019Scleromyxoedema is a rare generalized cutaneous mucinosis, which in absence of thyroid disease, occurs almost invariably in patients with monoclonal gammopathies. A...
Scleromyxoedema is a rare generalized cutaneous mucinosis, which in absence of thyroid disease, occurs almost invariably in patients with monoclonal gammopathies. A 54-year-old female patient presented with complaint of tightening of skin on the extremities, abdomen, forehead, gradually progressive since 1 year, episodes of generalized tonic-clonic convulsions, and acute psychosis since 5 days. Cutaneous examination revealed nonpitting edema over the face and sclerodermoid changes over extremities. Laboratory investigations showed presence of M-band on serum-protein electrophoresis and monoclonal spike of IgG lambda component on immunofixation. Magnetic resonance imaging of the brain showed periventricular subcortical lacunar infarcts. Skin biopsy with mucin staining was suggestive of scleromyxoedema. All other investigations were normal. Bone marrow biopsy showed a mild focal increase in plasma cells. The cutaneous, serological, and electrophoretic findings as well as the clinical profile of the patient were consistent with the diagnosis of monoclonal gammopathy of undetermined significance associated with scleromyxoedema. This case is presented because of its rare occurrence.
PubMed: 30775300
DOI: 10.4103/idoj.IDOJ_138_18 -
Dermatology Reports Jun 2024The generalized and sclerodermic form of lichen myxedematosus, known as scleromyxedema (SMX), is a chronic mucinosis that manifests cutaneously and has multiple systemic...
The generalized and sclerodermic form of lichen myxedematosus, known as scleromyxedema (SMX), is a chronic mucinosis that manifests cutaneously and has multiple systemic comorbidities. There are few available treatment options and no established therapeutic guidelines. We describe a 48-year-old man who had intravenous immunoglobulins (IVIg), oral corticosteroids, and methotrexate (MTX) for the treatment of SMX, monoclonal gammopathy, and arthritis. Because of its effectiveness and high level of tolerance, IVIg is the most often used first-line therapy for SMX and has been used for an increasing range of skin conditions. In our instance, better control of skin disease and extracutaneous manifestations was made possible by combining IVIg with oral prednisone and MTX. To the best of our knowledge, this is the first instance of SMX treatment that has combined therapeutic approaches with a favorable safety profile.
PubMed: 38957635
DOI: 10.4081/dr.2023.9803