595 results match your criteria Lichen Myxedematosus

Versican deposits in the sclerotic skin of a patient with localized lichen myxedematosus accompanied by joint involvement.

J Dermatol 2021 Jun 2. Epub 2021 Jun 2.

Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.

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Coexistence of scleromyxedema and Sneddon syndrome.

JAAD Case Rep 2021 May 20;11:47-50. Epub 2021 Mar 20.

Section of Dermatology and Venereology, Department of Medicine, University of Verona, Verona, Italy.

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Lichen Myxedematosus Presenting as a Solitary Nodule.

Ann Dermatol 2020 Apr 11;32(2):168-169. Epub 2020 Mar 11.

Department of Dermatology, Chungbuk National University College of Medicine, Cheongju, Korea.

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IgG1 Subclass Restriction and Biochemical Peculiarities of Monoclonal Immunoglobulins in Scleromyxedema.

Clin Lab 2021 Mar;67(3)

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. Read More

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Acral lichen myxedematosus showing acro-osteolysis in a patient with Sjögren syndrome.

J Dermatol 2021 Apr 9;48(4):e173-e174. Epub 2021 Feb 9.

Department of Dermatology, Fukushima Medical University School of Medicine, Fukushima, Japan.

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Successful treatment of dermato-neuro syndrome with plasmapheresis.

BMJ Case Rep 2020 Dec 29;13(12). Epub 2020 Dec 29.

Hematology and Oncology, Providence Park Hospital, Novi, Michigan, USA.

Altered mental status can have many causes ranging from emergent intracranial pathologies to more insidious, systemic toxic aetiologies. We report a rare case of dermato-neuro syndrome in a 71-year-old man with a known history of scleromyxoedema. The patient initially presented with encephalopathy which quickly progressed to generalised tonic-clonic seizures and coma. Read More

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December 2020


J Dtsch Dermatol Ges 2020 Dec;18(12):1449-1467

Department of Dermatology, University of Heidelberg, Germany.

Scleromyxedema is a rare, cutaneous deposition disorder from the group of mucinoses, which can affect multiple organs and is virtually always associated with a monoclonal gammopathy. Cutaneous manifestations are usually generalized, 2 to 3 mm sized, dome-shaped or flat-topped, waxy, slightly red to skin-colored papules and sclerodermoid indurations. Neurological, rheumatological, cardiovascular, gastrointestinal, respiratory tract, renal and ophthalmologic manifestations can occur, with decreasing frequency. Read More

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December 2020

Treatment of scleromyxedema with lenalidomide, bortezomib and dexamethasone: A case report and review of the literature.

Clin Case Rep 2020 Dec 17;8(12):3043-3049. Epub 2020 Sep 17.

Department of Hematology and Oncology University of Arizona Tucson Arizona.

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. Read More

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December 2020

Scleromyxedema in a 21 year old female patient with acute lymphoblastic leukemia: a case report.

BMC Dermatol 2020 12 4;20(1):18. Epub 2020 Dec 4.

Department of Critical Care , An-Najah National University Hospital , Nablus, Palestine.

Background: Scleromyxedema is a rare, para-neoplastic, chronic, progressive condition of the Lichen myxedematosus (LM) family. The clinical picture consists of generalized confluent papular eruptions with possible systemic manifestations, which may be fatal as it still constitutes a therapeutic dilemma. Histologically, it is characterized by dermal mucin deposition, fibroblast proliferation with fibrosis, with monoclonal gammopathy in the absence of thyroid disease. Read More

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December 2020

Mycophenolate mofetil treatment of an H syndrome patient with a SLC29A3 mutation.

Dermatol Ther 2020 11 26;33(6):e14375. Epub 2020 Oct 26.

Department of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.

H syndrome is a complex multi-organ disorder with autosomal recessive inheritance. The skin manifestations include early onset hyperpigmentation and hypertrichosis, followed by skin induration often diagnosed as scleromyxedema and morphea. There is no effective treatment. Read More

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November 2020

A Rare Case of Tumoral Scleromyxedema.

Indian J Dermatol 2020 Jul-Aug;65(4):310-312

Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.

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. Read More

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Long-Term Efficacy of Treatment with Intravenous Immunoglobulin in Scleromyxedema.

Acta Dermatovenerol Croat 2020 Jul;28(1):24-28

Katarina Trčko, MD, PhD, Department of Dermatology and Venereal Diseases, University Medical Centre Maribor, Ljubljanska ulica 5, 2000 Maribo,r Slovenia;

Scleromyxedema or generalized lichen myxedematosus is a rare depositional disorder. Diagnostic criteria encompass a generalized papular and sclerodermoid eruption, monoclonal gammopathy (paraproteinemia), most often with G-lambda type immunoglobulin, a characteristic microscopic triad (mucin deposition, fibroblast proliferation, fibrosis), and absence of thyroid disease. Many internal manifestations of scleromyxedema have been described to date, leading to high mortality and morbidity. Read More

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Characterization of circulating myeloma tumor cells by next generation flowcytometry in scleromyxedema patient: a case report.

Medicine (Baltimore) 2020 Jul;99(27):e20726

Hamad Medical Corporation, Hematology and Medical Oncology Department.

Introduction: Scleromyxedema (rare cutaneous mucinosis), is characterized by the formation of lichenoid papules and presence of Serum monoclonal IgG in most cases, or all; after repeated testing.

Patient Concerns: The patient is a 51-year-old male presented with thick, disfiguring elephant-like erythematous skin folds over the forehead, papular shiny eruptions over ears and trunk and waxy erythematous papules over arms and hands without dysphagia or respiratory or neurologic symptoms DIAGNOSIS: : Skin biopsy from right arm was consistent with scleromyxedema. Serum cryoglobulin was reported negative. Read More

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Lichen Myxedematosus: Case Report and Review of Literature.

J Drugs Dermatol 2020 03;19(3):320-322

Lichen myxedematosus (LM) is an idiopathic cutaneous mucinosis, commonly described as localized scleromyxedema. In contrast to scleromyxedema, there is typically no systemic involvement. Treatment options are limited and spontaneous resolution has been reported. Read More

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Acute Encephalitic Syndrome Induced by Scleromyxedema.

Am J Med Sci 2020 08 20;360(2):192-195. Epub 2020 May 20.

Dermatology Department, General Hospital of Athens, "Evangelismos", Athens, Greece.

Dermato-neuro syndrome is a potentially fatal neurological complication of scleromyxedema consisting of fever, seizures, and coma. This is an overlooked scleromyxedema case of a 62-year-old female patient from 2-years ago. She was admitted to our ICU because of high fever, colloid speech, muscle ache, and nausea. Read More

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Sclerodermalike syndromes: Great imitators.

Clin Dermatol 2020 Mar - Apr;38(2):235-249. Epub 2019 Oct 24.

Department of Rheumatology and Immunology, University of Pécs Clinical Center, Pecs, Hungary. Electronic address:

Sclerodermalike syndromes (SLSs) comprise diseases with mucin deposition (eg, scleromyxedema, scleredema), with eosinophilia (eg, eosinophilic fasciitis), metabolic or biochemical abnormalities (eg, nephrogenic systemic fibrosis), or endocrine disorders (eg, POEMS syndrome, or polyneuropathy, organomegaly, endocrinopathy, monoclonal lymphoproliferative disorder, and hypothyroidism). Chronic graft-versus-host disease may also show sclerodermalike skin changes. Inherited progeria syndromes with early aging (eg, Werner syndrome) and a heterogeneous group of hereditary disorders with either skin thickening (eg, stiff skin syndrome) or atrophy and tightening (eg, acrogeria) can also imitate classic systemic sclerosis (SSc). Read More

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September 2020

Self-healing juvenile cutaneous mucinosis, a sclerodermoid disorder simulating juvenile dermatomyositis: a case-based review.

Rheumatol Int 2020 Nov 22;40(11):1911-1920. Epub 2020 Apr 22.

Department of Pediatric Clinical Immunology and Rheumatology, Allergy Immunology Unit, Postgraduate Institute of Medical Education and Research, Advanced Pediatric Center, Chandigarh, 160012, India.

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. Read More

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November 2020

Immunotherapy-refractory vacuolar myopathy with mucin deposition in scleromyxedema: A possible role of fibroblast growth factor 2.

Neuropathology 2020 Oct 19;40(5):492-495. Epub 2020 May 19.

Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

Scleromyxedema (SME) is characterized by widespread waxy papules on the skin, with mucin deposits in the upper dermis. Twenty-one SME cases of myopathy have been reported; of the cases, six showed vacuolar formation, and two showed mucin deposition. We report the first case of SME with mucin-associated vacuolated fibers. Read More

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October 2020

Scleromyxedema treatment: a systematic review and update.

Int J Dermatol 2020 Oct 2;59(10):1191-1201. Epub 2020 May 2.

Department of Gastroenterology, Hotel Dieu de France University Hospital, Beirut, Lebanon - Faculty of Medicine, Saint Joseph University, Beirut, Lebanon.

Importance: Scleromyxedema is a chronic disease with high morbidity and mortality and no definitive therapeutic guidelines.

Objective: To review all available data on the efficacy and the safety of the available treatments of scleromyxedema and suggest a possible therapeutic approach.

Evidence Review: We performed a systematic literature review in Pubmed/Medline, Embase, and Cochrane collaboration databases, searching for all articles since 1990 on the treatments of scleromyxedema, with no limits on participant age, gender, or nationality. Read More

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October 2020

Successful Coronary Artery Bypass Grafting in a Patient With Uncontrolled Scleromyxedema.

Cureus 2020 Mar 5;12(3):e7185. Epub 2020 Mar 5.

Internal Medicine, Wayne State University, Detroit Medical Center, Detroit, USA.

Scleromyxedema is a rare disorder characterized by diffuse cutaneous and systemic mucinosis with paraproteinemia. Affected patients usually develop numerous waxy, firm papules and plaques as a result of subcutaneous mucin deposition and fibrosis. Systemic manifestations may involve the cardiovascular, gastrointestinal, pulmonary, musculoskeletal, renal, or nervous systems and are known to lead to significant morbidity and mortality if left untreated. Read More

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Scleromyxedema with multiple systemic involvement: Successful treatment with intravenous immunoglobulin.

Dermatol Ther 2020 05 15;33(3):e13378. Epub 2020 Apr 15.

Division of Rheumatology, Department of Internal Medicine, Soonchunhyang University Seoul Hospital, Soonchunhyang University College of Medicine, Seoul, South Korea.

Scleromyxedema is a rare connective tissue disorder characterized by a generalized lichenoid eruption and sclerodermoid induration with histologic features of dermal mucin deposition. A 44-year-old man presented with a 3-year history of generalized progressive skin thickening and sclerosis. He had diffuse skin-colored to erythematous firm papules coalescing into indurated plaques over his whole body. Read More

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A rare case of acral persistent papular mucinosis.

Clin Case Rep 2020 Feb 3;8(2):344-346. Epub 2020 Jan 3.

National Skin Center Singapore Singapore.

In patients with asymptomatic papules of hands and feet, a clinical differential of acral persistent papular mucinosis should be thought of. Read More

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February 2020

Generalized Acquired Cutis Laxa Associated with Monoclonal Gammopathy of Dermatological Significance.

Case Rep Dermatol Med 2020 12;2020:7480607. Epub 2020 Feb 12.

Division of Hematology/Oncology and Department of Dermatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.

Background: Cutis laxa is a rare dermatosis that is inherited or acquired and clinically features loose, wrinkled, and redundant skin with decreased elasticity. This heterogeneous connective tissue disorder may be localized or generalized, with or without internal manifestations. Generalized cutis laxa often has a cephalocaudal progression and is attributed to inflammatory cutaneous eruptions, medications, and infections. Read More

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February 2020

Scleromyxedema in a patient with thyroid disease: an atypical case or a case for revised criteria?

Cutis 2020 Jan;105(1):E6-E10

Department of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, USA.

Lichen myxedematosus (LM), commonly referred to as papular mucinosis, is a rare papular eruption defined by mucin deposition in the dermis. Scleromyxedema (SM) is a generalized papular and sclerodermoid form of LM. It is a progressive disease of unknown etiology with systemic manifestations that cause serious morbidity and mortality. Read More

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January 2020

Scleromyxedema histopathologically mimicking hypercellular fibrous papules (angiofibomas): Case report of an unusual histopathological presentation.

J Cutan Pathol 2020 Jul 20;47(7):638-642. Epub 2020 Feb 20.

Department of Dermatology, SUNY Downstate Medical Center, Brooklyn, New York.

Scleromyxedema (SMX) is an inflammatory condition of unknown etiology strongly associated with monoclonal gammopathy. Classical histopathology of SMX is characterized with the triad of diffuse mucin deposits, increased amount of collagen, and presence of stellate fibroblasts. Herein, we report an unusual histopathological variant of SMX in a 41-year-old female with lesions of the nose histopathologically mimicking cellular angiofibromas. Read More

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Scleromyxedema and Hyperphosphatemia: Causal Relationship or Coincidence?

J Clin Rheumatol 2020 Feb 5. Epub 2020 Feb 5.

School of Medicine, Tehran University of Medical Science, Tehran, Iran.

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February 2020

Plasma cell-directed therapies in monoclonal gammopathy-associated scleromyxedema.

Blood 2020 04;135(14):1101-1110

Service de Dermatologie, Poissy-Saint Germain Hospital, Saint-Germain-en-Laye, France.

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. Read More

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Rare case of infiltrative cardiomyopathy secondary to scleromyxoedema.

Intern Med J 2020 01;50(1):127-128

Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.

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January 2020

Widespread skin thickening.

Cutis 2019 Dec;104(6):E15-E17

Icahn School of Medicine at Mount Sinai Hospital, New York, New York, USA.

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December 2019