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Escleromixedema
ORPHA:167635CID-10 · L98.5CID-11 · EB90.11DOENÇA RARA

O escleromixedema é uma doença cutânea rara e grave. Os sinais e sintomas incluem acúmulo anormal de mucina na pele (mucinose), causando inchaços papulares e esclerodermóides; aumento da produção de fibroblastos (células do tecido conjuntivo) na ausência de distúrbio da tireoide; e gamopatia monoclonal (proteínas anormais no sangue). Freqüentemente envolve órgãos internos e pode afetar vários sistemas do corpo. A causa do escleromixedema não é conhecida. Não existe um tratamento padrão. O manejo pode envolver o uso de imunoglobulina intravenosa (IVIG), plasmaférese, talidomida e corticóides, ou intervenções mais agressivas, como transplante autólogo de medula óssea.

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Introdução

O que você precisa saber de cara

📋

O escleromixedema é uma doença cutânea rara e grave. Os sinais e sintomas incluem acúmulo anormal de mucina na pele (mucinose), causando inchaços papulares e esclerodermóides; aumento da produção de fibroblastos (células do tecido conjuntivo) na ausência de distúrbio da tireoide; e gamopatia monoclonal (proteínas anormais no sangue). Freqüentemente envolve órgãos internos e pode afetar vários sistemas do corpo. A causa do escleromixedema não é conhecida. Não existe um tratamento padrão. O manejo pode envolver o uso de imunoglobulina intravenosa (IVIG), plasmaférese, talidomida e corticóides, ou intervenções mais agressivas, como transplante autólogo de medula óssea.

Publicações científicas
478 artigos
Último publicado: 2026 Apr 14

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Casos conhecidos
250
pacientes catalogados
Início
Adult
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: L98.5
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

💪
Músculos
5 sintomas
🧬
Pele e cabelo
4 sintomas
🫃
Digestivo
3 sintomas
🧠
Neurológico
2 sintomas
🫁
Pulmão
2 sintomas
👂
Ouvidos
2 sintomas

+ 18 sintomas em outras categorias

Características mais comuns

90%prev.
Anormalidade generalizada da pele
Muito frequente (99-80%)
90%prev.
Paraproteinemia
Muito frequente (99-80%)
90%prev.
Pápula
Muito frequente (99-80%)
55%prev.
Pele rígida
Frequente (79-30%)
55%prev.
Pele espessada
Frequente (79-30%)
55%prev.
Anormalidade do antebraço
Frequente (79-30%)
41sintomas
Muito frequente (3)
Frequente (14)
Ocasional (17)
Muito raro (7)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 41 características clínicas mais associadas, ordenadas por frequência.

Anormalidade generalizada da peleGeneralized abnormality of skin
Muito frequente (99-80%)90%
Paraproteinemia
Muito frequente (99-80%)90%
PápulaPapule
Muito frequente (99-80%)90%
Pele rígidaStiff skin
Frequente (79-30%)55%
Pele espessadaThickened skin
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2025
Total histórico478PubMed
Últimos 10 anos200publicações
Pico202027 papers
Linha do tempo
2025Hoje · 2026🧪 2020Primeiro ensaio clínico📈 2020Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
1Fase 11
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 1 ensaio
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Escleromixedema

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

1 ensaios clínicos encontrados.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
168 papers (10 anos)
#1

Scleromyxedema Managed With High-Dose Intravenous Immunoglobulin and Bortezomib-Dexamethasone: A Case Report.

Case reports in oncological medicine2026

Scleromyxedema is a rare, chronic cutaneous mucinosis marked by widespread waxy papules and potential extracutaneous involvement. This case report discusses the management of a 48-year-old female diagnosed with scleromyxedema, who initially partially responded to high-dose intravenous immunoglobulin (HDIVIG) therapy. After partial relapse, she was induced with bortezomib, a proteasome inhibitor, and dexamethasone, achieving significant clinical improvement. Long-term maintenance with IVIG was utilized to prevent recurrence of symptoms. This case highlights the effectiveness of both HDIVIG and bortezomib-dexamethasone dual therapy as viable treatment options for scleromyxedema, emphasizing the importance of maintenance therapy to prevent relapse.

#2

Dupilumab improves itch, but not fibrosis, in a patient with scleromyxedema: A case report.

SAGE open medical case reports2026

Scleromyxedema is a rare, chronic mucinosis characterized by widespread skin fibrosis and an associated monoclonal gammopathy. Therapeutic options remain limited. We present the case of a male in his 50s with biopsy-confirmed scleromyxedema and IgG λ monoclonal gammopathy who initially responded to intravenous immunoglobulin but later progressed despite full-dose maintenance. Due to severe pruritus and recent evidence implicating Type 2 cytokines in scleromyxedema pathogenesis, dupilumab was added to ongoing intravenous immunoglobulin. Pruritus improved within 4 months, but fibrosis remained unchanged and M-protein levels continued to rise, reaching 35.3 g/L by month 12. Dupilumab was discontinued due to lack of disease modification. Bone marrow biopsy confirmed smoldering multiple myeloma. The patient subsequently responded well to daratumumab, cyclophosphamide, bortezomib, and dexamethasone chemotherapy and autologous stem cell transplant. This case illustrates the potential for IL-4Rα blockade to control symptoms such as pruritus in scleromyxedema, but underscores the need for plasma cell-directed therapy to address the underlying disease process.

#3

Monoclonal gammopathies of cutaneous significance: A nomenclature and pathophysiology-based classification.

Journal of the American Academy of Dermatology2026 Feb 23
#4

Diffuse waxy papules and dermal mucin in skin of color: A case of scleromyxedema.

JAAD case reports2026 Mar
#5

Discrete papular lichen myxedematosus successfully treated with isotretinoin.

JAAD case reports2026 Jan

Publicações recentes

Ver todas no PubMed

📚 EuropePMC342 artigos no totalmostrando 191

2026

Scleromyxedema Managed With High-Dose Intravenous Immunoglobulin and Bortezomib-Dexamethasone: A Case Report.

Case reports in oncological medicine
2026

Monoclonal gammopathies of cutaneous significance: A nomenclature and pathophysiology-based classification.

Journal of the American Academy of Dermatology
2026

Diffuse waxy papules and dermal mucin in skin of color: A case of scleromyxedema.

JAAD case reports
2026

Dupilumab improves itch, but not fibrosis, in a patient with scleromyxedema: A case report.

SAGE open medical case reports
2026

Discrete papular lichen myxedematosus successfully treated with isotretinoin.

JAAD case reports
2025

Unusual presentation of primary cutaneous mucinosis with Blaschkoid Hemibody distribution: case report.

Oxford medical case reports
2025

Hyalase in Dermatology: Applications Beyond Filler Management.

Journal of cosmetic dermatology
2025

Sclerosing diseases of the skin.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2025

Acral Persistent Papular Mucinosis in the United States: Case Series and Literature Review.

JMIR dermatology
2025

Clinicopathologic Features, Pathogenesis, and Treatment of Monoclonal Gammopathy-Associated Myopathies.

Neurology
2025

Skin-Colored Papules on the Face and Chest of a Female Patient.

Clinical case reports
2025

Successful treatment of scleromyxedema with dupilumab: A case report.

JAAD case reports
2025

Monoclonal Gammopathies of Clinical Significance-Scleromyxedema: A Case Report and Literature Review.

Clinical case reports
2025

IVIG Treatment in Scleromyxedema: Do Histologic Variants Influence Outcomes?

The Israel Medical Association journal : IMAJ
2025

Intravenous immunoglobulin in the treatment of scleromyxedema associated with monoclonal gammopathy.

Anais brasileiros de dermatologia
2025

Monoclonal gammopathy of cutaneous significance.

Journal of the American Academy of Dermatology
2025

[Target organ connective tissue: the variability of systemic fibrosis].

Innere Medizin (Heidelberg, Germany)
2025

Unusual Cutaneous Manifestations in a Patient with a History of Hepatitis B: A Case of Scleromyxedema and Literature Review.

Clinical, cosmetic and investigational dermatology
2025

Successful Treatment of a Case of Scleromyxedema With Intravenous Immunoglobulin and Thalidomide and a Two-and-a-Half-Year Follow-Up.

The Australasian journal of dermatology
2025

Unveiling the enigma: a case-based review of scleromyxedema.

Rheumatology international
2024

Scleromyxedema and Breast-Implant Associated Lymphoma: Investigating an Unusual Clinical Association.

Cureus
2024

Therapeutic Plasma Exchange for the Symptomatic Treatment of Scleromyxedema: A Case Report and Literature Review.

Journal of clinical apheresis
2024

Treatment of a Complex Case of Scleromyxedema Using a Novel Intravenous Immunoglobulin Preparation.

Case reports in dermatology
2024

Localized eruption of glabella and eyebrows.

JAAD case reports
2024

[Endogenous skin overloads].

Annales de pathologie
2025

The role of intravenous immunoglobulin in autoimmune diseases with dermatological implications.

Italian journal of dermatology and venereology
2024

Clinical and pathological characteristics of a dog with scleromyxoedema.

The Journal of small animal practice
2024

A novel case report of isolated cardiac myxedematosus.

European heart journal. Case reports
2024

Efficacy of intravenous immunoglobulins in severe scleromyxedema dysphagia assessed by oesophageal scintigraphy.

Clinical and experimental rheumatology
2024

Discrete Acral Papular Mucinosis.

The American Journal of dermatopathology
2025

Plasma cell-directed therapies induce profound clinical and durable responses in patients with severe or relapsed/refractory scleromyxedema.

Journal of the European Academy of Dermatology and Venereology : JEADV
2024

Lenalidomide and Dexamethasone in Scleromyxedema: A Case Report of Long-Term Efficacy and Challenges in a 54-Year-Old Patient.

Cureus
2024

Doughnut Sign: A Tell-Tale Sign of Scleromyxedema.

Indian dermatology online journal
2024

Localized Scleromyxedema Masquerading as Angioedema.

Indian dermatology online journal
2024

Combination of intravenous immunoglobulin, oral prednisone, and methotrexate for managing scleromyxedema: case report and literature discussion.

Dermatology reports
2024

Primary localized cutaneous lichen myxedematosus with light chain-restricted plasma cells: A distinct variant of the localized form of lichen myxedematosus.

Journal of cutaneous pathology
2024

Gammopathic dermopathy: characterization of cutaneous MGUS.

Leukemia &amp; lymphoma
2024

A case of nodular lichen myxedematosus.

The Australasian journal of dermatology
2024

Successful treatment of scleromyxoedema with daratumumab.

British journal of haematology
2024

Consensus statement on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxoedema and scleroedema.

Journal of the European Academy of Dermatology and Venereology : JEADV
2024

Successful treatment of scleromyxedema with daratumumab, lenalidomide and dexamethasone in a patient with multiple myeloma.

The Australasian journal of dermatology
2024

Scleromyxedema and Underlying Multiple Myeloma.

Mayo Clinic proceedings
2024

Rare scleromyxedema granuloma-annulare-like pattern successfully treated with intravenous immunoglobulins.

Italian journal of dermatology and venereology
2024

Unusual variant of scleromyxedema presenting with severe systemic involvement and atypical adnexal proliferations - A histological pitfall with the risk of unnecessary surgeries.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2023

Granulomatous variant of scleromyxedema successfully treated with topical ruxolitinib, dapsone and intravenous immunoglobulin.

JAAD case reports
2023

The realistic positioning of UVA1 phototherapy after 25 years of clinical experience and the availability of new biologics and small molecules: a retrospective clinical study.

Frontiers in medicine
2023

Case of new-onset scleromyxoedema-scleroedema spectrum disorder in a patient with CLL.

RMD open
2024

Nodular-Type Lichen Myxedematosus: A Rare Variant With Exclusive Involvement of the Hands-Report of Two Cases With a Comprehensive Review of the Literature.

The American Journal of dermatopathology
2025

SCLEROMYXEDEMA ASSOCIATED WITH MACULAR EDEMA AND RETINAL HEMORRHAGE.

Retinal cases &amp; brief reports
2024

Dermato-neuro syndrome after COVID-19 infection in a patient with scleromyxoedema: Previously successful treatment with intravenous immunoglobulins.

The Journal of dermatology
2023

Immunologic and nonimmunologic sclerodermal skin conditions - review.

Frontiers in immunology
2023

Dermoscopic and Cytological Findings in Scleromyxedema.

Dermatology practical &amp; conceptual
2023

Scleromyxedema: A rare case report with dermoscopic findings.

Indian journal of dermatology, venereology and leprology
2023

Scleromyxedema in an adult following Sinopharm BBIBP-CorV vaccination: An extremely rare phenomenon.

Journal of scleroderma and related disorders
2023

[Dermato-neuro syndrome-an acute and life-threatening complication of scleromyxedema Arndt-Gottron].

Dermatologie (Heidelberg, Germany)
2023

Checkpoint Inhibitor-Associated Scleroderma and Scleroderma Mimics.

Pharmaceuticals (Basel, Switzerland)
2023

Generalized Muscular Uptake of 99m Tc-MDP on Bone Scan in a Patient With Scleromyxedema-Associated Myopathy.

Clinical nuclear medicine
2023

Isotretinoin for treatment of a challenging case of scleromyxoedema. Comment on 'Localized papulonodular facial eruption in HIV'.

Clinical and experimental dermatology
2023

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

Frontiers in medicine
2023

Case for diagnosis. Multiple infiltrated plaques in a patient with human immunodeficiency virus and hepatitis C co-infection: lichen myxedematosus.

Anais brasileiros de dermatologia
2022

Small lymphocytic lymphoma presenting as chronic diffuse lip swelling.

Dermatology online journal
2023

Identifying the potential origin of mucin in primary cutaneous mucinoses-A retrospective study and analysis using histopathology and multiplex fluorescence staining.

Journal of the European Academy of Dermatology and Venereology : JEADV
2022

Case report: Scleromyxedema associated with a monoclonal gammapathy: Successful treatment with intravenous immunoglobulins.

Frontiers in immunology
2022

Combination of Medical and Surgical Treatment in Scleromyxedema.

Cureus
2023

Adverse reactions to the injection of face and neck aesthetic filling materials: a systematic review.

Medicina oral, patologia oral y cirugia bucal
2022

Dermato-neuro Syndrome after COVID-19 Vaccination in a Patient with Scleromyxoedema Previously Controlled with Bortezomib and Intravenous Immunoglobulins.

Acta dermato-venereologica
2022

Renal injury in scleromyxoedema due to monoclonal gammopathy associated C3 glomerulonephritis.

BMJ case reports
2022

Autologous stem cell transplantation in a patient with scleromyxedema.

EJHaem
2022

Clinical Characteristics and Treatment Outcomes of Scleromyxedema: A 10-Year Retrospective Survey.

Case reports in dermatology
2022

Early onset of scleromyxedema Arndt-Gottron associated with a monoclonal gammapathy: Successful treatment with intravenous immunoglobulins.

Clinical case reports
2022

An unusual case of granulomatous scleromyxedema.

JAAD case reports
2022

Monoclonal gammopathy of clinical significance: what the rheumatologist needs to know.

The Lancet. Rheumatology
2022

Clinical and histopathological improvement of scleromyxedema-induced microstomia after hyaluronidase injection.

Journal of cosmetic dermatology
2022

Cutaneous manifestations of monoclonal gammopathy.

Blood cancer journal
2021

Unusual type of scleromyxedema with multiple subcutaneous nodules, IgM-λ paraproteinemia, and scleroderma-like microangiopathy.

Journal of scleroderma and related disorders
2022

A Case of Scleromyxedema Development Following Intravesical Bacillus Calmette-Guérin Administration.

The Journal of rheumatology
2022

New and emerging conditions of acquired cutaneous mucinoses in adults.

Journal of the European Academy of Dermatology and Venereology : JEADV
2021

Solitary Cutaneous Focal Mucinosis.

Cureus
2021

SARS-CoV-2: A potential trigger of dermato-neuro syndrome in a patient with scleromyxedema.

JAAD case reports
2022

Papular mucinosis associated with band acro-osteolysis: a new syndrome associated with connective tissue-disease.

Rheumatology (Oxford, England)
2021

Widespread, waxy, coalescing cutaneous papules.

Clinical and experimental dermatology
2021

Scleromyxedema Without Monoclonal Gammopathy Treated with Intravenous Immunoglobulins.

Acta dermatovenerologica Croatica : ADC
2022

Lichen myxedematosus associated with pulmonary mucosa-associated lymphoid tissue lymphoma.

The Journal of dermatology
2021

Acral persistent papular mucinosis.

Clinics in dermatology
2021

Stem cell therapy in dermatology.

Indian journal of dermatology, venereology and leprology
2021

First report of histopathological and ultrasonographic correlation of localized lichen myxoedematosus.

Clinical and experimental dermatology
2021

Acral persistent papular mucinosis: A case report and summary of 24 Japanese cases.

The Journal of dermatology
2021

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

The Journal of dermatology
2021

Coexistence of scleromyxedema and Sneddon syndrome.

JAAD case reports
2021

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

Clinical laboratory
2021

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

The Journal of dermatology
2020

Successful treatment of dermato-neuro syndrome with plasmapheresis.

BMJ case reports
2020

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

Clinical case reports
2020

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

BMC dermatology
2020

An atypical presentation of discrete papular mucinosis in the genitalia mimicking a molluscum contagiosum infection.

Dermatology online journal
2020

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

Dermatologic therapy
2020

A Rare Case of Tumoral Scleromyxedema.

Indian journal of dermatology
2020

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

Acta dermatovenerologica Croatica : ADC
2020

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

Medicine
2020

Lichen Myxedematosus: Case Report and Review of Literature.

Journal of drugs in dermatology : JDD
2020

Acute Encephalitic Syndrome Induced by Scleromyxedema.

The American journal of the medical sciences
2020

Sclerodermalike syndromes: Great imitators.

Clinics in dermatology
2020

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

Rheumatology international
2020

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

Neuropathology : official journal of the Japanese Society of Neuropathology
2020

Scleromyxedema treatment: a systematic review and update.

International journal of dermatology
2020

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

Cureus
2020

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

Dermatologic therapy
2020

Of lions, shar-pei, and doughnuts: a tale retold.

Blood
2020

A rare case of acral persistent papular mucinosis.

Clinical case reports
2020

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

Case reports in dermatological medicine
2020

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

Cutis
2020

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

Journal of cutaneous pathology
2021

Scleromyxedema and Hyperphosphatemia: Causal Relationship or Coincidence?

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2020

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

Blood
2020

Rare case of infiltrative cardiomyopathy secondary to scleromyxoedema.

Internal medicine journal
2019

Widespread skin thickening.

Cutis
2020

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

BMC pulmonary medicine
2020

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

Journal of the European Academy of Dermatology and Venereology : JEADV
2019

Lichen myxedematosus: a rare group of cutaneous mucinosis.

Anais brasileiros de dermatologia
2019

Multiple skin-colored papules on the face and upper extremities.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2019

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

The Journal of dermatology
2019

An unusual presentation of scleromyxedema as inflammatory myopathy.

Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology
2019

[Sclerosing skin diseases].

Der Internist
2019

[Paraneoplastic skin diseases].

Der Internist
2019

High-Dose Intravenous Immunoglobulin in Skin Autoimmune Disease.

Frontiers in immunology
2019

Dermoscopy and reflectance confocal microscopy for the diagnosis of scleromyxedema.

JAAD case reports
2019

New insights on scleromyxedema.

Journal of scleroderma and related disorders
2019

Myopathy and scleromyxedema.

Journal of neurology
2019

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

European journal of dermatology : EJD
2019

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

Case reports in dermatology
2020

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

Arthritis care &amp; research
2019

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

JAMA dermatology
2019

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

Open access Macedonian journal of medical sciences
2019

Plasma Cell Myeloma Masquerading as Scleromyxedema.

Indian dermatology online journal
2019

Unwelcomed face of scleromyxedema.

International journal of dermatology
2019

Cutaneous paraneoplastic syndromes.

Seminars in diagnostic pathology
2018

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

Dermatology online journal
2019

Clinical features of scleromyxoedema in an Afro-Caribbean man.

BMJ case reports
2021

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.

Retinal cases &amp; brief reports
2019

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

The American Journal of dermatopathology
2018

Scleromyxoedema: the importance of physical examination.

BMJ case reports
2019

Acitretin: a promising therapy for localized childhood lichen myxoedematosus.

Clinical and experimental dermatology
2019

[Mimetics of systemic sclerosis].

Zeitschrift fur Rheumatologie
2018

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

Annales de pathologie
2018

Resolution of Atypical Lichen Myxedematosus Following Successful Treatment of Chronic Hepatitis C Virus Infection With Sofosbuvir-Velpatasvir Combination Therapy.

JAMA dermatology
2019

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

The Journal of dermatology
2018

[Scleromyxedema as a systemic disease of glycosaminoglycan accumulation].

Arkhiv patologii
2018

Monoclonal Gammopathy-Associated Scleromyxedema Presenting as Leonine Facies.

Arthritis &amp; rheumatology (Hoboken, N.J.)
2018

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

Arkhiv patologii
2017

Papular mucinosis, or localized lichen myxedematosis (LM) (discrete papular type).

Dermatology online journal
2018

Paraneoplastic plaque-like cutaneous mucinosis in a patient with lung adenocarcinoma.

Journal of cutaneous pathology
2018

Scleroderma with an update about clinico-pathological correlation.

Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia
2018

Skin-colored papules on the elbows with a granuloma-annulare-like histological pattern.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2018

Letter on 'European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis'.

Journal of the European Academy of Dermatology and Venereology : JEADV
2017

High-dose intravenous immunoglobulins for the treatment of dermatological autoimmune diseases.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2018

Ineffectiveness of infliximab CT-P13 for the treatment of scleromyxedema: A case report.

Dermatologic therapy
2018

Interstitial Granulomatous Variant of Scleromyxedema-A Diagnostic Pitfall.

The American Journal of dermatopathology
2017

Pathophysiological Mechanisms in Sclerosing Skin Diseases.

Frontiers in medicine
2017

Paraneoplastic atypical scleromyxedema with advanced gastric cancer.

JAAD case reports
2017

European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis.

Journal of the European Academy of Dermatology and Venereology : JEADV
2017

Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review.

Clinical reviews in allergy &amp; immunology
2018

Review of Primary Cutaneous Mucinoses in Nonlupus Connective Tissue Diseases.

Journal of cutaneous medicine and surgery
2017

[Granulomatous reaction pattern of the skin : Interstitial granulomatous dermatitis - lymphoma - vasculitis].

Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete
2018

Scleroderma-like Disorders.

Current rheumatology reviews
2017

Lichen myxedematosus: how to reach the accurate diagnosis.

International journal of dermatology
2016

A Case of Autoimmune Polyglandular Syndrome .ype 2 Associated with Atypical Form of Scleromyxedema.

Ethiopian journal of health sciences
2017

Scleromyxoedema in a dog.

Veterinary dermatology
2017

Uncommon Presentation of Scleromyxoedema with a Distribution Pattern Mimicking a Photodermatosis.

Acta dermato-venereologica
2017

Fever, Joint Pain, Seizures, and Rash in a 53-Year-Old Woman.

Arthritis care &amp; research
2017

High-dose intravenous immunoglobulin therapy for scleromyxoedema: a prospective open-label clinical trial using an objective score of clinical evaluation system.

Journal of the European Academy of Dermatology and Venereology : JEADV
2017

Cutaneous mucinosis in a patient taking ustekinumab for palmoplantar psoriasis.

Dermatology online journal
2016

Acral papular mucinosis: a new case of this rare entity.

Anais brasileiros de dermatologia
2016

Scleromyxedema: clinical diagnosis and autopsy findings.

Anais brasileiros de dermatologia
2017

A case of scleromyxedema responding to lenalidomide and dexamethasone.

Indian dermatology online journal
2017

Papulonodular mucinosis, Guillain-Barré syndrome and nephrotic syndrome in a patient with systemic lupus erythematosus: a case report.

BMC nephrology
2017

Lichen myxedematosus: toward established classification and diagnostic criteria: Response to the letter 'Lichen myxedematosus: suggestions on the newly proposed diagnostic criteria'.

International journal of dermatology
2017

Lichen myxedematosus: suggestions on the newly proposed diagnostic criteria.

International journal of dermatology
2016

Case for diagnosis. Lichen myxedematosus.

Anais brasileiros de dermatologia
2016

Flesh-colored papular eruption.

Cutis
2017

Acral persistent papular mucinosis (APPM): Dermoscopy of an uncommon disease.

Journal of the American Academy of Dermatology
2017

Lichen myxedematosus: a case series focusing on clinical features, disease associations, and management.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2017

[Progressive induration of the skin with rough papules].

Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete
2016

Scleromyxedema without Paraproteinemia: Treatment with Thalidomide and Prednisolone.

Case reports in dermatology
2016

[Therapeutic administration of immunoglobulins].

Zeitschrift fur Rheumatologie
2015

Dermato-neuro syndrome in a case of scleromyxedema.

European journal of rheumatology
2016

Atypical scleromyxedema presenting with cutaneous and cardiovascular manifestations.

International medical case reports journal
2017

Lichen myxedematosus: diagnostic criteria, classification, and severity grading.

International journal of dermatology
2017

Localized lichen myxedematosus in childhood: what is the accurate diagnosis?

International journal of dermatology
2016

Mucin in the dermis: a case of tender tumors.

Dermatology online journal
2017

Monoclonal gammopathy of cutaneous significance: review of a relevant concept.

Journal of the European Academy of Dermatology and Venereology : JEADV
2016

Atypical discrete papular lichen myxedematosus associated with monoclonal gammopathy: report of four cases and review of the literature.

Journal of the European Academy of Dermatology and Venereology : JEADV
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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Scleromyxedema Managed With High-Dose Intravenous Immunoglobulin and Bortezomib-Dexamethasone: A Case Report.
    Case reports in oncological medicine· 2026· PMID 41766765mais citado
  2. Dupilumab improves itch, but not fibrosis, in a patient with scleromyxedema: A case report.
    SAGE open medical case reports· 2026· PMID 41625143mais citado
  3. Monoclonal gammopathies of cutaneous significance: A nomenclature and pathophysiology-based classification.
    Journal of the American Academy of Dermatology· 2026· PMID 41740896mais citado
  4. Diffuse waxy papules and dermal mucin in skin of color: A case of scleromyxedema.
    JAAD case reports· 2026· PMID 41675018mais citado
  5. Discrete papular lichen myxedematosus successfully treated with isotretinoin.
    JAAD case reports· 2026· PMID 41362867mais citado
  6. Treatment of scleromyxedema Arndt-Gottron with a novel intravenous immunoglobulin preparation.
    Dermatol Reports· 2026· PMID 41978975recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:167635(Orphanet)
  2. MONDO:0015665(MONDO)
  3. GARD:7615(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q17144875(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Escleromixedema
Compêndio · Raras BR

Escleromixedema

ORPHA:167635 · MONDO:0015665
Prevalência
<1 / 1 000 000
Casos
250 casos conhecidos
CID-10
L98.5 · Mucinose da pele
CID-11
Início
Adult
Prevalência
0.0 (Worldwide)
MedGen
UMLS
CL480915
Repurposing
19 candidatos
azosemideelectrolyte reabsorption inhibitor
benzthiazidecarbonic anhydrase inhibitor
bumetanidesolute carrier family member inhibitor
+16 outros
EuropePMC
Wikidata
Papers 10a
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