-
Cureus Dec 2022Scleromyxedema is an uncommon and progressive fibromucinous disorder characterized by disseminated papular eruption with histological features of dermal mucin...
Scleromyxedema is an uncommon and progressive fibromucinous disorder characterized by disseminated papular eruption with histological features of dermal mucin deposition. The skin changes associated with this disease are highly visible and they tend to affect the patient's quality of life. We report a case of a 50-year-old male patient that presented a 3-year-old history of disseminated asymptomatic firm papules-associated systemic symptoms. Medical treatment with oral corticosteroid and thalidomide was indicated and surgical treatment on residual facial folds was performed, with an excellent outcome.
PubMed: 36686085
DOI: 10.7759/cureus.32729 -
Annals of the Academy of Medicine,... Aug 2016
Topics: Humans; Leg; Magnetic Resonance Imaging; Male; Middle Aged; Scleromyxedema
PubMed: 27683746
DOI: No ID Found -
Indian Journal of Dermatology 2015Scleromyxedema is characterized by indurated erythematous papules disseminated on the face, chest and limbs. About twenty cases treated with thalidomide, stem cells,...
Scleromyxedema is characterized by indurated erythematous papules disseminated on the face, chest and limbs. About twenty cases treated with thalidomide, stem cells, melphalan and immunoglobulin with varying results have been described. We present the case of a 28-year-old male patient diagnosed with scleromyxedema not associated with monoclonal gammopathy, multi-treated with anti-leprosy drugs, UVA1, and thalidomide for 4 years with no improvement.
PubMed: 25814746
DOI: 10.4103/0019-5154.152600 -
Anais Brasileiros de Dermatologia 2016Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin...
Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been reported with partial or inconsistent responses. Despite showing unpredictable evolution, systemic consequences of scleromyxedema and treatment side effects may result in death. We describe a rare case of a patient with scleromyxedema without paraproteinemia with systemic involvement that evolved to death despite treatment with cyclophosphamide.
Topics: Biopsy; Fatal Outcome; Humans; Male; Middle Aged; Mucins; Scleromyxedema; Skin
PubMed: 28300892
DOI: 10.1590/abd1806-4841.20164527 -
Indian Journal of Dermatology 2020Scleromyxedema is an uncommon disease, affecting the skin mainly and other internal organs sometimes, characterized by fibroblasts proliferation, fibrosis, and mucous...
Scleromyxedema is an uncommon disease, affecting the skin mainly and other internal organs sometimes, characterized by fibroblasts proliferation, fibrosis, and mucous deposition in the absence of thyroid disorder. It is associated with monoclonal gammopathy in most cases. We are reporting a case with a rare presentation of tumoral scleromyxedema in the neck, with a mass mimicking other tumoral lesions, highlighting the importance of diagnosis and histopathologic correlation.
PubMed: 32831375
DOI: 10.4103/ijd.IJD_450_18 -
Indian Journal of Dermatology 2015Scleromyxedema is a rare, chronic and persistent idiopathic disorder characterized by a generalized papular eruption due to dermal mucin deposition with an increase in...
Scleromyxedema is a rare, chronic and persistent idiopathic disorder characterized by a generalized papular eruption due to dermal mucin deposition with an increase in dermal collagen. Patients usually have associated paraproteinemia. We describe the case of a 59-year-old gentleman with features of scleromyxedema, who had severe pruritus, scalp involvement, unrestricted mobility and associated peripheral eosinophilia, but no monoclonal gammopathy.
PubMed: 26120184
DOI: 10.4103/0019-5154.156456 -
JAAD Case Reports Aug 2022
PubMed: 35899146
DOI: 10.1016/j.jdcr.2022.06.022 -
Blood Apr 2020Scleromyxedema is a rare skin and systemic mucinosis that is usually associated with monoclonal gammopathy (MG). In this French multicenter retrospective study of 33...
Scleromyxedema is a rare skin and systemic mucinosis that is usually associated with monoclonal gammopathy (MG). In this French multicenter retrospective study of 33 patients, we investigated the clinical and therapeutic features of MG-associated scleromyxedema. Skin molecular signatures were analyzed using a transcriptomic approach. Skin symptoms included papular eruptions (100%), sclerodermoid features (91%), and leonine facies (39%). MG involved an immunoglobulin G isotype in all patients, with a predominant λ light chain (73%). Associated hematologic malignancies were diagnosed in 4 of 33 patients (12%) (smoldering myeloma, n = 2; chronic lymphoid leukemia, n = 1; and refractory cytopenia with multilineage dysplasia, n = 1). Carpal tunnel syndrome (33%), arthralgia (25%), and dermato-neuro syndrome (DNS) (18%) were the most common systemic complications. One patient with mucinous cardiopathy died of acute heart failure. High-dose IV immunoglobulin (HDIVig), alone or in combination with steroids, appeared to be quite effective in nonsevere cases (clinical complete response achieved in 13/31 patients). Plasma cell-directed therapies using lenalidomide and/or bortezomib with dexamethasone and HDIVig led to a significant improvement in severe cases (HDIVig refractory or cases with central nervous system or cardiac involvement). The emergency treatment of DNS with combined plasmapheresis, HDIVig, and high-dose corticosteroids induced the complete remission of neurological symptoms in 4 of 5 patients. Quantitative reverse-transcriptase polymerase chain reaction analysis of 6 scleromyxedema skin samples showed significantly higher profibrotic pathway levels (transforming growth factor β and collagen-1) than in healthy skin. Prospective studies targeting plasma cell clones and/or fibrotic pathways are warranted for long-term scleromyxedema management.
Topics: Adult; Aged; Antineoplastic Agents; Bortezomib; Dexamethasone; Female; Glucocorticoids; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Lenalidomide; Male; Middle Aged; Paraproteinemias; Plasma Cells; Plasmapheresis; Retrospective Studies; Scleromyxedema; Skin; Transcriptome
PubMed: 32027747
DOI: 10.1182/blood.2019002300 -
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 -
Journal of Scleroderma and Related... Jun 2021Scleromyxedema is a mysterious cutaneous mucinosis of unknown etiology. Various types of scleromyxedema variant have been reported, which often give us a clue to...
Scleromyxedema is a mysterious cutaneous mucinosis of unknown etiology. Various types of scleromyxedema variant have been reported, which often give us a clue to understand the key aspects of this disease. Here, we describe a woman with highly unusual type of scleromyxedema. In addition to the rare manifestations of multiple subcutaneous nodules and IgM-λ paraproteinemia, our patient showed several characteristic symptoms of scleroderma such as shortened nails and fingertips, sclerodactyly, and bone resorption of fingertips and mandibles as a result of peripheral circulatory insufficiency, although this disease is known to be pathophysiologically different from scleroderma. A skin biopsy revealed cutaneous microvascular stenosis and occlusion due to intravascular mucin deposition and fibrotic changes, suggesting that scleromyxedema potentially develops peripheral circulatory disorders and other vascular involvement. The subcutaneous nodules were responsive to high-dose intravenous immunoglobulin. Scleromyxedema can represent a wide variety of systemic involvement, and therefore, we should pay attention to those symptoms as well as skin lesions.
PubMed: 35386736
DOI: 10.1177/2397198320974190