Introdução
O que você precisa saber de cara
Líquen mixedematoso é um grupo de distúrbios cutâneos considerados mucinoses.
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Entender a doença
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 18 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 41 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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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
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Líquen mixedematoso
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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
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
Discrete papular lichen myxedematosus successfully treated with isotretinoin.
Acral Persistent Papular Mucinosis in the United States: Case Series and Literature Review.
Acral persistent papular mucinosis (APPM) is a localized variant of lichen myxedematosus (LM) characterized by asymptomatic, flesh-colored papules primarily distributed on the hands and forearms. This chronic dermatosis, distinct from generalized mucinosis due to its lack of systemic involvement, remains underreported in medical literature. In this study, we present two cases of APPM to the limited pool of documented cases in the United States, highlighting its emerging recognition. This is a case series of two patients presenting with asymptomatic papular eruptions on the hands and wrists, consistent with the typical presentation of APPM. Diagnostic confirmation via biopsy revealed focal cutaneous mucinosis. Comprehensive laboratory evaluations, including serum and urine protein electrophoresis, showed no evidence of underlying gammopathy in either patient. Treatment modalities for APPM are limited and often ineffective. Unlike other forms of LM, APPM features are confined to skin lesions, posing primarily as a cosmetic concern with a favorable prognosis. Accurate diagnosis of this localized LM is crucial to differentiate it from the more severe, generalized form, scleromyxedema, which can have organ involvement and may become fatal. Notably, while spontaneous resolution is reported in LM, including discrete papular mucinosis, APPM typically persists without resolution even after extended follow-up. These cases underscore the importance of recognizing APPM and advocating for broader awareness and exploration of its clinical variability, etiology, and management strategies. With increasing recognition, the understanding of APPM can be enhanced, paving the way for optimized management and improved outcomes for affected individuals.
Papular lesions on the forearms in a 56-year-old woman.
Acral persistent papular mucinosis: a rare child case report and literature review.
Acral persistent papular mucinosis (APPM) is a rare idiopathic subtype of localized lichen myxedematosus. To date, there have been less than 41 APPM cases reported worldwide, however, almost all patients were older than 18 years of age. A 7-year-old child was first reported in this paper. A 7-year-old boy was admitted to our hospital with a solitary skin-colored papule on the radial side of the middle segment of his right index finger. The patient wanted to know the exact diagnosis and remove it because the flexion movement of the middle segment had been affected. Thus, a surgery was performed. Histopathological examination of a biopsy specimen obtained from the papule on the radial side of the middle segment of his right index finger showed a focal and well-circumscribed deposit of mucin in the papillary and middermis. The deposit never extended deeply into the reticular dermis. Mucin spared a subepidermal area in the papillary dermis. Alcian blue stains can highlight the mucin. The papule was histologically diagnosed as an APPM and excised surgically. The wound gradually healed after the operation, and no obvious recurrence, scar or other discomfort was observed during follow-up so far. To the best of our knowledge, this is the rare case of a child APPM presenting as a solitary papule affecting the flexion movement of the middle segment. Since it is a rare disease, we report this case to contribute to future research on the diagnosis and pathogenesis of APPM.
Plaque-like cutaneous mucinosis associated with pain: A case report.
Plaque-like cutaneous mucinosis (PCM) is a rare atypical subtype of Lichen myxedematosus, characterized by hyperpigmented plaques typically found on the midline trunk. We report the case of a 26-year-old woman with a 1-year history of painful, pruritic, hyperpigmented plaques over her spine. Physical examination revealed soft plaques in the midline of the back, and histopathology showed dermal mucin deposition, consistent with PCM. The presented case is notable as the first reported instance of PCM presenting with lesional pain. We propose that mechanical stress, likely due to repeated friction at the site of spinous processes, contributed to the development of pain. The fact that pain lessened after the use of a lumbar support pillow and gaining weight supports our hypothesis. Our case demonstrates the importance of considering PCM in the differential diagnosis of hyperpigmented plaques and highlights the need for further research to elucidate its underlying pathogenesis and optimize treatment strategies.
Publicações recentes
Discrete papular lichen myxedematosus successfully treated with isotretinoin.
Papular lesions on the forearms in a 56-year-old woman.
Acral Persistent Papular Mucinosis in the United States: Case Series and Literature Review.
Plaque-like cutaneous mucinosis associated with pain: A case report.
Lenalidomide and Dexamethasone in Scleromyxedema: A Case Report of Long-Term Efficacy and Challenges in a 54-Year-Old Patient.
📚 EuropePMC100 artigos no totalmostrando 61
Discrete papular lichen myxedematosus successfully treated with isotretinoin.
JAAD case reportsPapular lesions on the forearms in a 56-year-old woman.
JAAD case reportsAcral Persistent Papular Mucinosis in the United States: Case Series and Literature Review.
JMIR dermatologyPlaque-like cutaneous mucinosis associated with pain: A case report.
SAGE open medical case reportsLenalidomide and Dexamethasone in Scleromyxedema: A Case Report of Long-Term Efficacy and Challenges in a 54-Year-Old Patient.
CureusAcral persistent papular mucinosis: a rare child case report and literature review.
Translational pediatricsCombination of intravenous immunoglobulin, oral prednisone, and methotrexate for managing scleromyxedema: case report and literature discussion.
Dermatology reportsPrimary localized cutaneous lichen myxedematosus with light chain-restricted plasma cells: A distinct variant of the localized form of lichen myxedematosus.
Journal of cutaneous pathologyA case of nodular lichen myxedematosus.
The Australasian journal of dermatologyGranulomatous variant of scleromyxedema successfully treated with topical ruxolitinib, dapsone and intravenous immunoglobulin.
JAAD case reportsNodular-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 dermatopathologyDermato-neuro syndrome after COVID-19 infection in a patient with scleromyxoedema: Previously successful treatment with intravenous immunoglobulins.
The Journal of dermatology[Dermato-neuro syndrome-an acute and life-threatening complication of scleromyxedema Arndt-Gottron].
Dermatologie (Heidelberg, Germany)Solitary acral persistent papular mucinosis nodule: A case report and summary of eight Korean cases.
World journal of clinical casesLichen myxedematosus associated with monoclonal gammopathy of undetermined significance: A case report and literature review.
Frontiers in medicineCase for diagnosis. Multiple infiltrated plaques in a patient with human immunodeficiency virus and hepatitis C co-infection: lichen myxedematosus.
Anais brasileiros de dermatologiaIdentifying 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 : JEADVCombination of Medical and Surgical Treatment in Scleromyxedema.
CureusPediatric Lichen Myxedematosus: A Diagnostic and Management Challenge.
Children (Basel, Switzerland)New and emerging conditions of acquired cutaneous mucinoses in adults.
Journal of the European Academy of Dermatology and Venereology : JEADVLichen myxedematosus associated with pulmonary mucosa-associated lymphoid tissue lymphoma.
The Journal of dermatologyAcral persistent papular mucinosis.
Clinics in dermatologyAcral persistent papular mucinosis: A case report and summary of 24 Japanese cases.
The Journal of dermatologyVersican deposits in the sclerotic skin of a patient with localized lichen myxedematosus accompanied by joint involvement.
The Journal of dermatologyLichen Myxedematosus Presenting as a Solitary Nodule.
Annals of dermatologyAcral lichen myxedematosus showing acro-osteolysis in a patient with Sjögren syndrome.
The Journal of dermatologyScleromyxedema in a 21 year old female patient with acute lymphoblastic leukemia: a case report.
BMC dermatologyLong-Term Efficacy of Treatment with Intravenous Immunoglobulin in Scleromyxedema.
Acta dermatovenerologica Croatica : ADCLichen Myxedematosus: Case Report and Review of Literature.
Journal of drugs in dermatology : JDDScleromyxedema in a patient with thyroid disease: an atypical case or a case for revised criteria?
CutisLichen myxedematosus: a rare group of cutaneous mucinosis.
Anais brasileiros de dermatologiaScleromyxedema and lichen myxedematosus: Is it associated with viral hepatitis?
The Journal of dermatologyReticular Erythematous Mucinosis: A Rare Cutaneous.
Acta dermatovenerologica Croatica : ADCDiscrete Papular Lichen Myxedematosus and Scleromyxedema with Hypothyroidism: A Report of Two Cases.
Case reports in dermatologyScleromyxedema (Arndt - Gottron Syndrome) Developing Under Tenofovir Treatment for Hepatitis B: Unique Presentation in a Bulgarian Patient!
Open access Macedonian journal of medical sciencesLocalized Lichen Myxedematosus With Plasma Cell Light Chain Restriction: Is It the Exception or the Rule?
The American Journal of dermatopathologyResolution of Atypical Lichen Myxedematosus Following Successful Treatment of Chronic Hepatitis C Virus Infection With Sofosbuvir-Velpatasvir Combination Therapy.
JAMA dermatologyLichen myxedematosus associated with monoclonal gammopathy of undetermined significance: A case report and a review of Japanese patients.
The Journal of dermatologyBreast cancer cutaneous metastases mimicking Papilloma Cutis Lymphostatica. Biopsy to avoid pitfalls.
International journal of surgery case reports[Is scleromyxedema a skin problem or systemic pathological process?].
Arkhiv patologiiPapular mucinosis, or localized lichen myxedematosis (LM) (discrete papular type).
Dermatology online journalReview of Primary Cutaneous Mucinoses in Nonlupus Connective Tissue Diseases.
Journal of cutaneous medicine and surgeryAtypical Lichen Myxedematosus: A Case with Remarkable Response to Low Dose Melphalan.
Indian dermatology online journalLichen myxedematosus: how to reach the accurate diagnosis.
International journal of dermatologyCutaneous mucinosis in a patient taking ustekinumab for palmoplantar psoriasis.
Dermatology online journalAcral papular mucinosis: a new case of this rare entity.
Anais brasileiros de dermatologiaLichen myxedematosus: eine Fallserie mit Schwerpunkt auf klinischen Merkmalen, Krankheitsassoziationen und Behandlung.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGLichen myxedematosus: toward established classification and diagnostic criteria: Response to the letter 'Lichen myxedematosus: suggestions on the newly proposed diagnostic criteria'.
International journal of dermatologyLichen myxedematosus: suggestions on the newly proposed diagnostic criteria.
International journal of dermatologyCase for diagnosis. Lichen myxedematosus.
Anais brasileiros de dermatologiaAcral persistent papular mucinosis (APPM): Dermoscopy of an uncommon disease.
Journal of the American Academy of DermatologyLichen myxedematosus: a case series focusing on clinical features, disease associations, and management.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGLichen myxedematosus: diagnostic criteria, classification, and severity grading.
International journal of dermatologyLocalized lichen myxedematosus in childhood: what is the accurate diagnosis?
International journal of dermatologyMucin in the dermis: a case of tender tumors.
Dermatology online journalAtypical 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 : JEADVMidfacial involvement in lichen myxedematosus: a clinical pearl in diagnosis.
Journal of the European Academy of Dermatology and Venereology : JEADVLichen Myxedematosus: Strict Classification and Diagnostic Criteria are Still Lacking.
Indian journal of dermatologyHistopathologic characteristics of scleromyxedema: A study of a series of 34 cases.
Journal of the American Academy of DermatologyFrequency of cutis laxa-like clinical features and elastolysis in scleromyxedema: a retrospective clinicopathologic study of 19 patients with scleromyxedema.
Journal of cutaneous pathologyDiscrete ('acral nonpapular') localised lichen myxedematosus.
BMJ case reportsAssociações
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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.
- Discrete papular lichen myxedematosus successfully treated with isotretinoin.
- Acral Persistent Papular Mucinosis in the United States: Case Series and Literature Review.
- Papular lesions on the forearms in a 56-year-old woman.
- Acral persistent papular mucinosis: a rare child case report and literature review.
- Plaque-like cutaneous mucinosis associated with pain: A case report.
- Lenalidomide and Dexamethasone in Scleromyxedema: A Case Report of Long-Term Efficacy and Challenges in a 54-Year-Old Patient.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:402007(Orphanet)
- MONDO:0018432(MONDO)
- GARD:21709(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q6543199(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