579 results match your criteria Lichen Myxedematosus


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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http://dx.doi.org/10.1097/MD.0000000000020726DOI Listing

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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http://dx.doi.org/10.36849/JDD.2020.4864DOI Listing

Acute Encephalitic Syndrome Induced by Scleromyxedema.

Am J Med Sci 2020 May 20. 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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http://dx.doi.org/10.1016/j.amjms.2020.05.018DOI Listing

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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http://dx.doi.org/10.1016/j.clindermatol.2019.10.010DOI Listing
October 2019

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

Rheumatol Int 2020 Apr 22. 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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http://dx.doi.org/10.1007/s00296-020-04578-5DOI Listing
April 2020
1.627 Impact Factor

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

Neuropathology 2020 May 19. 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-containing vacuolated fibers. Read More

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http://dx.doi.org/10.1111/neup.12659DOI Listing

Scleromyxedema treatment: a systematic review and update.

Int J Dermatol 2020 May 2. 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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http://dx.doi.org/10.1111/ijd.14888DOI Listing

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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http://dx.doi.org/10.7759/cureus.7185DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7124883PMC

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

Dermatol Ther 2020 May 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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http://dx.doi.org/10.1111/dth.13378DOI Listing

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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http://dx.doi.org/10.1002/ccr3.2639DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7044381PMC
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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http://dx.doi.org/10.1155/2020/7480607DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7037480PMC
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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http://dx.doi.org/10.1111/cup.13665DOI Listing

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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http://dx.doi.org/10.1097/RHU.0000000000001290DOI Listing
February 2020

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

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

Sorbonne Université, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Saint Antoine, Service de Médecine Interne, Paris, 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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http://dx.doi.org/10.1182/blood.2019002300DOI Listing

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
0.594 Impact Factor

Severe but reversible pulmonary hypertension in scleromyxedema and multiple myeloma: a case report.

BMC Pulm Med 2020 Jan 9;20(1). Epub 2020 Jan 9.

Department of Pathology, Marshfield Clinic, Marshfield, WI, USA.

Background: Scleromyxedema is a progressive, systemic connective tissue disorder characterized by fibro-mucous skin lesions and increased serum monoclonal immunoglobulin levels. Pulmonary involvement occurs in a subset of patients, though the overall prevalence of pulmonary lesions in scleromyxedema is unknown. Since pulmonary hypertension presumably occurs in these patients due to disease progression and development of additional conditions, treatment of the underlying plasma cell dyscrasia and connective tissue disorder may improve pulmonary hypertension symptoms. Read More

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http://dx.doi.org/10.1186/s12890-019-1020-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6953266PMC
January 2020

Increased frequency of interleukin-4 and reduced frequency of interferon-γ and IL-17-producing CD4+ and CD8+ cells in scleromyxedema.

J Eur Acad Dermatol Venereol 2020 May 7;34(5):1092-1097. Epub 2020 Jan 7.

Section of Pathology, Department of Surgical and Morphological Sciences, University of Genoa, Genoa, Italy.

Background: Little is known about the pathogenesis of scleromyxedema, a life-threatening fibromucinosis disease with immunological dysregulation.

Objectives: To investigate on T-cell phenotype, function and cytokine biology in search of new insights supporting the immunopathogenesis of the disease.

Methods: We analysed the frequency of circulating lymphocyte subsets, the T-cell maturation stage, the generation of antigen-specific T-cell lines and T-cell cytokine secretion. Read More

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http://dx.doi.org/10.1111/jdv.16136DOI Listing

Lichen myxedematosus: a rare group of cutaneous mucinosis.

An Bras Dermatol 2019 17;94(4):462-469. Epub 2019 Oct 17.

Dermatology Service, Hospital Universitario, Facultad de Medicina, Universidad Autónoma de Nuevo León, Monterrey, Nuevo León, México.

Cutaneous mucinoses are a heterogeneous group of dermatoses in which excess deposition of mucin in the dermis gives the skin a waxy appearance, with papules and plaques that can vary from self-healing mucinosis to even disrupting the normal shape of a patient's face, conferring a leonine facies, or be part of life threatening diseases like scleromyxedema. This review will describe the most recent classification on lichen myxedematosus in the generalized (scleromyxedema) and the localized forms, as well as the different organ systems involved in scleromyxedema, diagnostic workup, current management, and prognosis. Read More

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http://dx.doi.org/10.1590/abd1806-4841.20198478DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7007042PMC
October 2019

Scleromyxedema and lichen myxedematosus: Is it associated with viral hepatitis?

J Dermatol 2019 Oct 28;46(10):879-885. Epub 2019 Aug 28.

Department of Dermatology, Chang Gung Memorial Hospital, Taipei, Taiwan.

Scleromyxedema (SM) was previously known to be associated with monoclonal gammopathy. The association of SM and its counterpart lichen myxedematosus (LM) with chronic hepatitis has rarely been reported. We retrospectively reviewed medical records and histopathological reports of consecutive patients who presented at our department with the diagnosis of SM or LM from January 2001 to September 2017. Read More

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http://dx.doi.org/10.1111/1346-8138.15039DOI Listing
October 2019
1 Read
2.354 Impact Factor

An unusual presentation of scleromyxedema as inflammatory myopathy.

Acta Myol 2019 03 1;38(1):13-16. Epub 2019 Mar 1.

Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India.

Scleromyxedema is a rare cutaneous mucinosis with frequent extracutaneous manifestations. Myopathy in scleromyxedema is a poorly recognized syndrome among neurologists and can mimic idiopathic and connective tissue disease-associated inflammatory myopathy. Diagnosis is suspected by the characterization of the skin lesions and clinched by skin and muscle biopsies. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6598408PMC
March 2019
4 Reads

[Sclerosing skin diseases].

Internist (Berl) 2019 Aug;60(8):783-791

Hautklinik, Universitätsklinikum Erlangen, Ulmenweg 18, 91054, Erlangen, Deutschland.

Sclerosing skin manifestations are more a symptom than a diagnosis and must therefore be meticulously clarified. Systemic scleroderma as a multiorgan disease must be distinguished from localized scleroderma or morphea because in addition to a different clinical picture they have a different prognosis and necessitate other therapeutic procedures. Rare sclerosing skin diseases with implications for internal medicine are eosinophilic fasciitis, Buschke's scleredema adultorum, scleromyxedema and nephrogenic systemic fibrosis. Read More

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http://dx.doi.org/10.1007/s00108-019-0643-2DOI Listing
August 2019
16 Reads

[Paraneoplastic skin diseases].

Internist (Berl) 2019 Aug;60(8):775-782

Hautklinik und Hauttumorzentrum Rheinpfalz, Klinikum der Stadt Ludwigshafen, Bremserstraße 79, 67063, Ludwigshafen am Rhein, Deutschland.

Various specific skin alterations can occur in patients with malignant diseases. If these skin diseases occur as associated symptoms of a malignant process, they are called paraneoplastic. In this overview, obligate and frequent facultative paraneoplastic skin diseases are assigned according to the triggering type of malignancy. Read More

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http://dx.doi.org/10.1007/s00108-019-0636-1DOI Listing
August 2019
9 Reads

High-Dose Intravenous Immunoglobulin in Skin Autoimmune Disease.

Front Immunol 2019 11;10:1090. Epub 2019 Jun 11.

Department of Dermatology, University of Heidelberg, Heidelberg, Germany.

The immunomodulatory potential and low incidence of severe side effects of high-dose intravenous immunoglobulin (IVIg) treatment led to its successful application in a variety of dermatological autoimmune diseases over the last two decades. IVIg is usually administered at a dose of 2 g per kg body weight distributed over 2-5 days every 4 weeks. They are most commonly used as a second- or third-line treatment in dermatological autoimmune disease (pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, dermatomyositis, systemic vasculitis, and systemic lupus erythematosus). Read More

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http://dx.doi.org/10.3389/fimmu.2019.01090DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6579842PMC
June 2019
10 Reads

Dermoscopy and reflectance confocal microscopy for the diagnosis of scleromyxedema.

JAAD Case Rep 2019 May 8;5(5):451-453. Epub 2019 May 8.

Department of Dermatology, Hospital de Santa Maria, Lisboa, Portugal.

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http://dx.doi.org/10.1016/j.jdcr.2019.03.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6510934PMC
May 2019
4 Reads

Myopathy and scleromyxedema.

J Neurol 2019 Aug 21;266(8):2051-2059. Epub 2019 May 21.

Department of Neurology, Nerve-Muscle Unit, CHU Bordeaux (Groupe Hospitalier Pellegrin), University of Bordeaux, Place Amélie Raba-Léon, 33000, Bordeaux, France.

Scleromyxedema is a chronic, idiopathic disorder associated with monoclonal gammopathy, and characterized by dermal mucin deposition. However, systemic manifestations are frequent, including neuromuscular symptoms. We herein present a 71-year-old man who developed a vacuolar myopathy in a context of a known scleromyxedema, and we compare our observation with the nineteen other cases found in the medical literature. Read More

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http://dx.doi.org/10.1007/s00415-019-09379-wDOI Listing
August 2019
2 Reads

sQUIZ your knowledge: Widespread millimetric papules in a patient with monoclonal gammopathy.

Eur J Dermatol 2019 Apr;29(2):244-245

Dermatology Department, Complejo Hospitalario Universitario de Pontevedra, EOXI Pontevedra-Salnés, Spain.

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http://dx.doi.org/10.1684/ejd.2019.3566DOI Listing
April 2019
3 Reads

Reticular Erythematous Mucinosis: A Rare Cutaneous.

Acta Dermatovenerol Croat 2019 Mar;27(1):16-21

Amer Ali Almohssen, MD , Dermatopathology Fellow, State University of New York, Downstate Medical School & The Ackerman Academy of Dermatopathology, New York City , New York, USA;

Reticular erythematous mucinosis (REM) is a rare form of primary cutaneous mucinosis, most often involving the midline of the upper chest or back in middle-aged women. REM bears clinical and histopathologic resemblance to lupus erythematosus tumidus (LET), dermatomyositis, scleredema, and lichen myxedematosus. Early recognition and diagnosis of REM is particularly relevant to exclude the abovementioned diseases, as REM is more benign and has fewer systemic consequences. Read More

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March 2019
18 Reads

Discrete Papular Lichen Myxedematosus and Scleromyxedema with Hypothyroidism: A Report of Two Cases.

Case Rep Dermatol 2019 Jan-Apr;11(1):64-70. Epub 2019 Mar 19.

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

Scleromyxedema and lichen myxedematosus (LM) are rare disorders that fall along the spectrum of primary cutaneous mucinoses. Scleromyxedema is a systemic form that classically presents with generalized waxy papules, sclerodermoid eruption, and monoclonal gammopathy; LM is a localized form limited to the skin that classically presents with white, firm, waxy papules and lacks monoclonal gammopathy. According to diagnostic criteria established in 2001, the diagnosis of both conditions requires absence of thyroid disease. Read More

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http://dx.doi.org/10.1159/000498881DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6465707PMC
March 2019
7 Reads

Clinical and Molecular Phenotyping in Scleromyxedema Pretreatment and Posttreatment With Intravenous Immunoglobulin.

Arthritis Care Res (Hoboken) 2020 Jun 14;72(6):761-767. Epub 2020 May 14.

Johns Hopkins University, Baltimore, Maryland.

Objective: Scleromyxedema (SMX) is a rare systemic sclerosis mimic that often responds to intravenous immunoglobulin (IVIG) therapy, yet the resulting clinical and biochemical changes have not been well characterized. To better understand the pathogenesis of the disease and the efficacy of IVIG, we sought to explore whether IVIG would introduce a measurable biologic effect corresponding with clinical improvement.

Methods: Fifteen patients with SMX were recruited for the study. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1002/acr.23908
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http://dx.doi.org/10.1002/acr.23908DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6810715PMC
June 2020
5 Reads

Abrupt Onset of Dysphagia, Arthritis, Joint Contractures, and Sheets of Monomorphous Waxy Papules.

JAMA Dermatol 2019 Jun;155(6):737-738

Department of Dermatology, Oregon Health and Science University, Portland.

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http://dx.doi.org/10.1001/jamadermatol.2018.5627DOI Listing
June 2019
2 Reads

Scleromyxedema (Arndt - Gottron Syndrome) Developing Under Tenofovir Treatment for Hepatitis B: Unique Presentation in a Bulgarian Patient!

Open Access Maced J Med Sci 2019 Mar 28;7(5):782-785. Epub 2019 Feb 28.

Medical Institute of Ministry of Interior (MVR), Department of Dermatology, Venereology and Dermatologic Surgery, General Skobelev 79, 1606 Sofia, Bulgaria.

Background: Scleromyxedema, also referred to as the Arndt-Gottron (S-AG) syndrome or the systemic form of Lichen myxedematosus (LM), is a cutaneous mucinosis with a chronic course and high lethality from systemic involvement of other organs and systems. Interesting in several aspects is the association between scleromyxedema and viral hepatitis about: 1) hepatitis virus infection as a possible etiological factor for the development of scleromyxedema, 2) antiretroviral therapy for the treatment of hepatitis as a method of reversing scleromyxedema and 3) antiviral drugs as inducers of scleromyxedema.

Case Report: We present a 53-year old patient who for nine months had been on tenofovir disoproxil 245 mg (0-0-1) therapy for chronic hepatitis B. Read More

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https://www.id-press.eu/mjms/article/view/oamjms.2019.181
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http://dx.doi.org/10.3889/oamjms.2019.181DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6447340PMC
March 2019
24 Reads

Plasma Cell Myeloma Masquerading as Scleromyxedema.

Indian Dermatol Online J 2019 Jan-Feb;10(1):50-53

Department of General Medicine, St. John's Medical College, Bengaluru, Karnataka, India.

Scleromyxedema is a rare progressive cutaneous mucinosis of unknown etiology with equal prevalence in both men and women. It is usually associated with monoclonal gammopathy in most of the cases. Various treatment modalities have been tried for scleromyxedema including steroids, intravenous immunoglobulin (IVIg), autologous hematopoietic stem cell transplantation, and melphalan, but none has proved to be fully effective. Read More

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http://dx.doi.org/10.4103/idoj.IDOJ_135_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6362738PMC
February 2019
7 Reads

Unwelcomed face of scleromyxedema.

Int J Dermatol 2019 May 11;58(5):e100-e102. Epub 2019 Feb 11.

Mansoura Dermatology, Venerology and Leprology Hospital, Mansoura, Egypt.

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http://dx.doi.org/10.1111/ijd.14400DOI Listing
May 2019
10 Reads

Cutaneous paraneoplastic syndromes.

Semin Diagn Pathol 2019 Jul 31;36(4):211-228. Epub 2019 Jan 31.

PRW Laboratories, Charlottesville, VA, United State.

A variety of cutaneous abnormalities can be seen in patients with malignant diseases, some of which are infectious, with others representing direct involvement of the skin by the underlying disorder. Yet another group of lesions can be regarded as associated markers of the malignant process, and, as such, are termed "paraneoplastic." This review considers the latter collection of conditions, grouping them by the generic type of malignancy that is usually linked to the paraneoplasia. Read More

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http://dx.doi.org/10.1053/j.semdp.2019.01.001DOI Listing
July 2019
44 Reads

Cutaneous mucinosis of infancy: report of a rare case and review of the literature.

Dermatol Online J 2018 10 15;24(10). Epub 2018 Oct 15.

Geisel School of Medicine at Dartmouth, Hanover, New Hampshire.

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. Read More

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October 2018
6 Reads

Clinical features of scleromyxoedema in an Afro-Caribbean man.

BMJ Case Rep 2019 Jan 20;12(1). Epub 2019 Jan 20.

Medicine, Princess Margaret Hospital, Nassau, Bahamas.

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http://dx.doi.org/10.1136/bcr-2018-227588DOI Listing
January 2019
7 Reads

ACUTE MYOCARDITIS WITH CARDIOGENIC SHOCK AND MULTIPLE ORGAN FAILURE, FOLLOWED BY BILATERAL PANUVEITIS MASQUERADING AS ENDOGENOUS ENDOPHTHALMITIS, DUE TO TOXOPLASMA GONDII IN AN IMMUNOCOMPETENT PATIENT.

Retin Cases Brief Rep 2019 Jan 17. Epub 2019 Jan 17.

Massachusetts Eye Research and Surgery Institution (MERSI), Waltham, Massachusetts.

Purpose: To report a case of systemic and ocular toxoplasmosis in an immunocompetent patient, who developed myocarditis with resulting cardiogenic shock and multiple organ failure, followed by bilateral panuveitis masquerading as endogenous endophthalmitis.

Methods: Single case report with images.

Results: A 59-year-old man with a history of monoclonal gammopathy of undetermined significance and associated scleromyxedema but otherwise immunocompetent was admitted to the intensive care unit for cardiogenic shock and multiple organ failure due to presumed viral myocarditis. Read More

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http://dx.doi.org/10.1097/ICB.0000000000000855DOI Listing
January 2019
10 Reads

Localized Lichen Myxedematosus With Plasma Cell Light Chain Restriction: Is It the Exception or the Rule?

Am J Dermatopathol 2019 Jul;41(7):505-510

Global Pathology Laboratory, Aurora Diagnostics, Miami Lakes, FL.

Lichen myxedematosus is a chronic cutaneous mucinosis that can present on a spectrum from localized cutaneous lesions to systemic disease of scleromyxedema. The clinical presentation of localized cutaneous lichen myxedematosus is waxy lichenoid papules, nodules, and/or plaques that have histopathologic findings of mucin deposition and a variable degree of fibroblast proliferation. There is an absence of serum paraproteins, and there are no other systemic causes of cutaneous mucinosis such as thyroid disease. Read More

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http://dx.doi.org/10.1097/DAD.0000000000001335DOI Listing
July 2019
17 Reads

Scleromyxoedema: the importance of physical examination.

BMJ Case Rep 2018 Oct 12;2018. Epub 2018 Oct 12.

Unit of Clinical Immunology, Centro Hospitalar Porto, Porto, Portugal.

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22714
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http://dx.doi.org/10.1136/bcr-2018-227144DOI Listing
October 2018
38 Reads

Acitretin: a promising therapy for localized childhood lichen myxoedematosus.

Clin Exp Dermatol 2019 Jul 7;44(5):574-576. Epub 2018 Oct 7.

Department of Histopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.

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http://doi.wiley.com/10.1111/ced.13799
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http://dx.doi.org/10.1111/ced.13799DOI Listing
July 2019
1 Read

[Mimetics of systemic sclerosis].

Z Rheumatol 2019 Feb;78(1):14-23

Klinik für Rheumatologie und klinische Immunologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland.

Background: Systemic sclerosis (SSc) is characterized by heterogeneous clinical symptoms. Peripheral skin fibrosis can be a common symptom. Nevertheless, a variety of diseases with different etiologies are associated with a thickening of the skin and make the initial diagnosis of systemic sclerosis more difficult. Read More

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http://dx.doi.org/10.1007/s00393-018-0538-yDOI Listing
February 2019
22 Reads

[Dermatopathology histoseminar. Interstitial granuloma annulare, case 8].

Ann Pathol 2018 Oct 17;38(5):305-313. Epub 2018 Sep 17.

Cabinet de dermatopathologie Mathurin-Moreau, 35, avenue Mathurin-Moreau, 75019 Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.annpat.2018.09.001DOI Listing
October 2018
6 Reads

[Scleromyxedema].

Hautarzt 2018 Nov;69(11):916-921

Universitätsklinik und Poliklinik für Dermatologie und Venerologie, Universitätsklinikum Halle (Saale), Martin-Luther-Universität Halle-Wittenberg, Halle, Deutschland.

Scleromyxedema is a rare disorder that frequently affects multiple extracutaneous organ systems and is usually associated with monoclonal gammopathy. The pathogenesis of scleromyxedema is unknown. The clinical course is chronic and progressive and can lead to marked morbidity or death. Read More

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http://dx.doi.org/10.1007/s00105-018-4257-8DOI Listing
November 2018
9 Reads

Lichen myxedematosus associated with monoclonal gammopathy of undetermined significance: A case report and a review of Japanese patients.

J Dermatol 2019 Jan 2;46(1):e32-e33. Epub 2018 Jul 2.

Department of Dermatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.

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http://dx.doi.org/10.1111/1346-8138.14511DOI Listing
January 2019
10 Reads

[Scleromyxedema as a systemic disease of glycosaminoglycan accumulation].

Arkh Patol 2018;80(3):53-58

V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.

Scleromyxedema is a rare mucinosis with a primary skin lesion due to diffuse mucin deposition, sclerosis, and lichenoid eruptions in the absence of hypothyroidism. The paper describes scleromyxedema cases and gives recommendations for the histological diagnosis of the disease by histochemical reactions to detect acid and neutral glycosaminoglycans. Read More

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http://dx.doi.org/10.17116/patol201880353-58DOI Listing
April 2019
2 Reads

Monoclonal Gammopathy-Associated Scleromyxedema Presenting as Leonine Facies.

Arthritis Rheumatol 2018 10;70(10):1694

University of Michigan, Ann Arbor, MI.

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http://dx.doi.org/10.1002/art.40530DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6160324PMC
October 2018
10 Reads

Breast cancer cutaneous metastases mimicking Papilloma Cutis Lymphostatica. Biopsy to avoid pitfalls.

Int J Surg Case Rep 2018 10;46:31-33. Epub 2018 Apr 10.

Department of Plastic and Reconstructive Surgery, University of Bari, Italy.

Introduction: Secondary lymphedema is the most frequent long-term complication of axillary lymphadenectomy. It can result in complication as erysipelas, warts, Papilloma Cutis Lymphostatica (PCL), or angiosarcomas. Moreover, in women affected by breast cancer an accurate differential diagnosis among these conditions or complication related to radiation dermatitis or cutaneous metastasis is essential. Read More

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http://dx.doi.org/10.1016/j.ijscr.2018.03.025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6000767PMC
April 2018
28 Reads

[Is scleromyxedema a skin problem or systemic pathological process?]

Arkh Patol 2018;80(1):63-66

N.I. Pirogov Russian National Research Medical University, Moscow, Russia.

Scleromyxedema is regarded as a rare cutaneous mucinosis from a group of lichen myxedematosus characterized by diffuse mucin deposition, sclerosis, and lichenoid eruptions in the absence of thyroid disease. The paper discusses the pathogenesis of the disease and histological changes in tissues. It underlines the need for using histochemical tests to identify acidic and neutral glycosaminoglycans and gives a differential diagnosis of this disease. Read More

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http://dx.doi.org/10.17116/patol201880163-66DOI Listing
April 2019
2 Reads