Definition and research guide
This page combines a MeSH definition, publication statistics and selected research abstracts. It is a starting point for reading the literature, not a medical textbook or a systematic evidence review.
Content available: 4 complete abstracts (454 words), 10 references. Full article text is not stored or reproduced here; follow the PMID, DOI or PMC links to read the original sources and their access terms.
Definition
A connective tissue disorder characterized by widespread thickening of SKIN with a cobblestone-like appearance. It is caused by proliferation of fibroblasts and deposition of mucin in the dermis in the absence of thyroid disease. Most scleromyxedema cases are associated with a monoclonal gammopathy, immunoglobulin IgG-lambda.
Definition: NLM Medical Subject Headings (MeSH) scope note (Public domain (U.S. National Library of Medicine); attribution requested). View the MeSH record.
Also known as: Lichen Myxedematosus; Myxedematosus, Lichen; Papular Mucinosis; Mucinosis, Papular.
Where it fits in the MeSH classification
- Broader topics
- Mucinoses
- See also
- Myxedema
Related concepts
Research landscape
PubMed indexes 325 records tagged with the MeSH heading “Scleromyxedema” (counts retrieved 2026-11-04). 71 of them (21.8%) were published from 2020 onward, and the busiest period so far is 2010-2019 with 164 records.
Reviews account for 46 records (14.2%) and meta-analyses for 0. Randomized controlled trials number 0 (0.0%) as a share of all records on the topic. Randomized trials make up only a small share of this literature, so much of what is known comes from laboratory, observational or descriptive work.
Show the data
| Period | Records |
|---|---|
| through 1999 | 36 |
| 2000-2009 | 54 |
| 2010-2019 | 164 |
| 2020 onward | 71 |
Publication-type chart data
| Type | Records | Share |
|---|---|---|
| Reviews | 46 | 14.2% |
| Meta-analyses | 0 | 0.0% |
| Randomized controlled trials | 0 | 0.0% |
Method: PubMed query "Scleromyxedema"[MeSH Terms] with publication-type and date filters, retrieved 2026-11-04.
Reading the evidence in bones, joints, muscles and skin
Joint, bone, muscle and skin conditions are often judged by pain scores, function questionnaires, imaging or photographs, and symptoms can fluctuate, so placebo response and natural improvement matter. Imaging changes are common in people without symptoms. Nothing on this page is advice about treatment. Check how outcomes were measured, whether the comparison group received a placebo or usual care, and how long the benefit lasted.
Evidence from open-access studies
These open-access studies come from PubMed’s best-match results for this heading (papers whose main topic it is), ranked here by citation count, with one recent addition. Each abstract is reproduced in full under its recorded open licence so that you can read the authors’ own words. They illustrate the literature; they are not a systematic summary of it. Citation counts measure attention, not reliability or clinical benefit.
| Study / authors | Year | Source label / design | Europe PMC citations | Licence / source |
|---|---|---|---|---|
| Scleromyxedema: clinical diagnosis and autopsy findings | 2016 | Research article Design not recorded in structured metadata | 16 | cc by PMID 28300892 · DOI · PMC5324991 |
| Papulonodular mucinosis, Guillain-Barré syndrome and nephrotic syndrome in a patient with systemic lupus erythematosus: a case report | 2017 | Research article Design not recorded in structured metadata | 8 | cc by PMID 28143420 · DOI · PMC5286654 |
| Acral papular mucinosis: a new case of this rare entity | 2016 | Research article Design not recorded in structured metadata | 7 | cc by PMID 28300913 · DOI · PMC5325012 |
| Sclerosing diseases of the skin | 2025 | Review article Design not recorded in structured metadata | 1 | cc by PMID 41030177 · DOI · PMC12548314 |
Scleromyxedema: clinical diagnosis and autopsy findings [1]
Abstract reading summary — source excerpts
- Opening excerpt
- Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology.
- Closing excerpt
- We describe a rare case of a patient with scleromyxedema without paraproteinemia with systemic involvement that evolved to death despite treatment with cyclophosphamide.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (95 words)
Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been reported with partial or inconsistent responses. Despite showing unpredictable evolution, systemic consequences of scleromyxedema and treatment side effects may result in death. We describe a rare case of a patient with scleromyxedema without paraproteinemia with systemic involvement that evolved to death despite treatment with cyclophosphamide.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 28300892 · DOI · PMC5324991
Papulonodular mucinosis, Guillain-Barré syndrome and nephrotic syndrome in a patient with systemic lupus erythematosus: a case report [2]
Abstract reading summary — source excerpts
- Opening excerpt
- BackgroundAwareness of the spectrum of clinical manifestations of systemic lupus erythematosus (SLE), especially uncommon changes, is essential for diagnosis and effective management of patients.
- Closing excerpt
- ConclusionWe reported a rare case of male patient with SLE with manifestation of class V lupus nephritis, Guillain-Barré syndrome and papulonodular mucinosis.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (142 words)
BackgroundAwareness of the spectrum of clinical manifestations of systemic lupus erythematosus (SLE), especially uncommon changes, is essential for diagnosis and effective management of patients.Case presentationA 26-year-old Chinese man with SLE initially manifested cutaneous papulonodular mucinosis and developed acute Guillain-Barré syndrome and class V lupus nephritis 2 years later. His cutaneous nodules had not been idententified for 2 years and were resected by surgical procedures twice until SLE was diagnosed. The kidney biopsy revealed class V lupus nephritis. The patient responded well to a short course of intravenous immunoglobulins and his muscle strength almost completely recovered. So far, he has undergone five cycles of cyclophosphamide combined with hydroxychloroquine and tapering prednisone, resulting in partial remission of lupus nephritis and disappearance of hypocomplementemia.ConclusionWe reported a rare case of male patient with SLE with manifestation of class V lupus nephritis, Guillain-Barré syndrome and papulonodular mucinosis.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 28143420 · DOI · PMC5286654
Acral papular mucinosis: a new case of this rare entity [3]
Abstract reading summary — source excerpts
- Opening excerpt
- Acral persistent papular mucinosis (APPM) is a rare subtype of localized lichen myxedematosus.
- Closing excerpt
- We present a 27-year-old healthy woman with asymptomatic papules on her upper extremities, which adequately meet clinical and pathological criteria of acral papular mucinosis.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (111 words)
Acral persistent papular mucinosis (APPM) is a rare subtype of localized lichen myxedematosus. It consists of small papules localized exclusively on the back of the hands, wrists and extensor aspects of distal forearms with no other clinical or laboratory manifestations. The lesions tend to persist and may increase slowly in number. Histologically, hematoxylin-eosin and Alcian blue staining demonstrate mucin accumulation in the upper reticular dermis with separation of collagen fibers as a result of hyaluronic acid deposition. Treatment is rarely necessary due to the absence of symptoms. We present a 27-year-old healthy woman with asymptomatic papules on her upper extremities, which adequately meet clinical and pathological criteria of acral papular mucinosis.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 28300913 · DOI · PMC5325012
Sclerosing diseases of the skin [4]
Abstract reading summary — source excerpts
- Opening excerpt
- Sclerosing skin diseases comprise a group of distinct dermatological conditions characterized by fibrotic changes that may severely impair patients' quality of life.
- Closing excerpt
- In addition, diagnostic tools and treatment options are addressed in detail.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (106 words)
Sclerosing skin diseases comprise a group of distinct dermatological conditions characterized by fibrotic changes that may severely impair patients' quality of life. These conditions often present with cutaneous manifestations and, in some cases, may extend to extracutaneous tissues, potentially resulting in significant morbidity and mortality. This review explores the current understanding of the four most prevalent sclerosing skin diseases - localized scleroderma (morphea), systemic sclerosis (SSc), scleromyxedema, and scleredema adultorum of Buschke - as well as sclerotic conditions induced by external agents. Here, we discuss the pathophysiology, clinical characteristics, and disease course of these entities. In addition, diagnostic tools and treatment options are addressed in detail.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 41030177 · DOI · PMC12548314
Key reviews and meta-analyses
The most cited review-type papers among PubMed’s best-match results for this heading, listed with bibliographic details only. Follow the links for the full text and access terms.
- Research article Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease [5]
- Research article European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis [6]
- Research article Scleromyxedema [7]
- Research article Scleromyxedema and the dermato-neuro syndrome: case report and review of the literature [8]
- Research article Scleroderma mimics [9]
- Research article Scleromyxedema treatment: a systematic review and update [10]
References
- Sala AC, Cunha PR, Pinto CA, Alves CA, Paiva IB, Araujo AP. Scleromyxedema: clinical diagnosis and autopsy findings. . 2016. PMID 28300892 · DOI · PMC5324991
- Su X, Qiao X, Li J, Gao L, Wang C, Wang L. Papulonodular mucinosis, Guillain-Barré syndrome and nephrotic syndrome in a patient with systemic lupus erythematosus: a case report. . 2017. PMID 28143420 · DOI · PMC5286654
- Gómez Sánchez ME, Manueles Marcos F, Martínez Martínez ML, Vera Berón R, Azaña Défez JM. Acral papular mucinosis: a new case of this rare entity. . 2016. PMID 28300913 · DOI · PMC5325012
- Kalantari Y, Meier K, Ghoreschi K, Kinberger M, Solimani F. Sclerosing diseases of the skin. . 2025. PMID 41030177 · DOI · PMC12548314
- Rongioletti F. Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease. Semin Cutan Med Surg. 2006. PMID 16908401 · DOI
- Knobler R, Moinzadeh P, Hunzelmann N, Kreuter A, Cozzio A, Mouthon L, et al.. European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis. J Eur Acad Dermatol Venereol. 2017. PMID 28786499 · DOI
- Cokonis Georgakis CD, Falasca G, Georgakis A, Heymann WR. Scleromyxedema. Clin Dermatol. 2006. PMID 17113967 · DOI
- Fleming KE, Virmani D, Sutton E, Langley R, Corbin J, Pasternak S, et al.. Scleromyxedema and the dermato-neuro syndrome: case report and review of the literature. J Cutan Pathol. 2012. PMID 22515222 · DOI
- Nashel J, Steen V. Scleroderma mimics. Curr Rheumatol Rep. 2012. PMID 22131103 · DOI
- Haber R, Bachour J, El Gemayel M. Scleromyxedema treatment: a systematic review and update. Int J Dermatol. 2020. PMID 32358980 · DOI
About this article
- Sources
- NLM Medical Subject Headings (definition, synonyms); NCBI PubMed (counts, identifiers); Europe PMC (open-access abstracts and citation counts).
- Verification
- Every reference was resolved against PubMed on 2026-11-04; title, year and DOI matched.
- Review state
- automated source verified; no human expert review has been recorded yet.
- Version
- 1, last updated 2026-11-04.
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Evidence strength
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