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Indian Journal of Dermatology 2020Scleromyxedema is an uncommon disease, affecting the skin mainly and other internal organs sometimes, characterized by fibroblasts proliferation, fibrosis, and mucous...
Scleromyxedema is an uncommon disease, affecting the skin mainly and other internal organs sometimes, characterized by fibroblasts proliferation, fibrosis, and mucous deposition in the absence of thyroid disorder. It is associated with monoclonal gammopathy in most cases. We are reporting a case with a rare presentation of tumoral scleromyxedema in the neck, with a mass mimicking other tumoral lesions, highlighting the importance of diagnosis and histopathologic correlation.
PubMed: 32831375
DOI: 10.4103/ijd.IJD_450_18 -
Clinical and Experimental Dermatology Dec 2021
Topics: Biopsy; Diagnosis, Differential; Humans; Male; Middle Aged; Scleromyxedema; Skin
PubMed: 34490932
DOI: 10.1111/ced.14659 -
International Journal of Dermatology Mar 2017Lichen myxedematosus (LM) is a rare, chronic idiopathic disorder characterized clinically by waxy, closely set papules and histopathologically by diffuse dermal mucin...
BACKGROUND
Lichen myxedematosus (LM) is a rare, chronic idiopathic disorder characterized clinically by waxy, closely set papules and histopathologically by diffuse dermal mucin deposition and fibroblast proliferation. The most recent classification of LM was proposed in 2001; however, it seems to be complex, confusing, and imprecise. Herein, we present seven cases of LM to evaluate the validity of the current classification, to propose new diagnostic criteria and classification, and to suggest a clinically relevant severity grading system for this rare disorder.
MATERIALS AND METHODS
The study included seven patients with different presentations and severities of LM. All patients were subjected to thorough dermatological and systemic examination, routine laboratory tests, evaluation of thyroid function, protein electrophoresis, and detailed investigations to detect systemic involvement.
RESULTS
The current classification does not meet the requirements of proper diagnosis of different presentations of LM. Subtyping of the studied patients differs greatly according to the old classification and the newly proposed one. New diagnostic criteria, classification, and grading are consequently suggested.
CONCLUSIONS
We propose two sets of diagnostic criteria to define the disease more precisely and to avoid confusion associated with the other classification. The first set comprises constant clinical and histopathological features that are always present in every case, and the second set includes associated features that were variably reported in some patients. LM is then subclassified according to the presence or absence of systemic manifestations into a systemic severe form (scleromyxedema) and a non-disabling, pure cutaneous form.
Topics: Aged; Aged, 80 and over; Female; Humans; Male; Middle Aged; Paraproteinemias; Scleromyxedema; Severity of Illness Index; Thyroid Diseases; Young Adult
PubMed: 27667657
DOI: 10.1111/ijd.13437 -
International Journal of Dermatology Jul 2016
Topics: Paraproteinemias; Scleromyxedema
PubMed: 26971585
DOI: 10.1111/ijd.13245 -
Journal of the American Academy of... Jun 2016Few histologic studies describe the histopathologic aspects of scleromyxedema.
BACKGROUND
Few histologic studies describe the histopathologic aspects of scleromyxedema.
OBJECTIVE
We sought to describe the histopathologic and immunohistochemical features of scleromyxedema in a large series of patients.
METHODS
We studied all the cases with scleromyxedema diagnosed between 2000 and 2014 at participating centers. Sections with hematoxylin-eosin and special stains were examined. Immunohistochemistry for CD3, CD4, CD8, CD20, CD68, and factor XIIIa was performed in 10 cases.
RESULTS
A total of 44 skin biopsy specimens from 34 patients were reviewed. Two different histopathologic patterns were observed: the classic microscopic triad (dermal mucin deposition, fibroblast proliferation, fibrosis) was identified in 34 specimens, whereas an interstitial granuloma annulare-like pattern was found in 10 specimens. A superficial perivascular infiltrate with T lymphocytes was found in all specimens whereas an interstitial proliferation of CD68(+) epithelioid cells was identified in the 10 specimens with an interstitial granuloma annulare-like pattern. Elastic fibers were largely lost, explaining the redundant folds of the disease.
LIMITATIONS
This was a retrospective study.
CONCLUSIONS
Scleromyxedema shows 2 histopathologic patterns, including the classic type with the microscopic triad of mucin, fibroblast proliferation and fibrosis, and an interstitial granuloma annulare-like pattern. Recognition of these histologic presentations expands the spectrum of scleromyxedema and highlights the difficulty in diagnosing this disabling condition in the absence of a clinicopathological correlation.
Topics: Adult; Aged; Aged, 80 and over; Antigens, CD; Antigens, CD20; Antigens, Differentiation, Myelomonocytic; CD3 Complex; CD4 Antigens; CD4-Positive T-Lymphocytes; CD8 Antigens; CD8-Positive T-Lymphocytes; Cytoprotection; Factor XIIIa; Female; Fibroblasts; Fibrosis; Histiocytes; Humans; Immunohistochemistry; Male; Middle Aged; Mucins; Retrospective Studies; Scleromyxedema; Skin
PubMed: 26897387
DOI: 10.1016/j.jaad.2015.12.021 -
Cureus Dec 2022Scleromyxedema is an uncommon and progressive fibromucinous disorder characterized by disseminated papular eruption with histological features of dermal mucin...
Scleromyxedema is an uncommon and progressive fibromucinous disorder characterized by disseminated papular eruption with histological features of dermal mucin deposition. The skin changes associated with this disease are highly visible and they tend to affect the patient's quality of life. We report a case of a 50-year-old male patient that presented a 3-year-old history of disseminated asymptomatic firm papules-associated systemic symptoms. Medical treatment with oral corticosteroid and thalidomide was indicated and surgical treatment on residual facial folds was performed, with an excellent outcome.
PubMed: 36686085
DOI: 10.7759/cureus.32729 -
The Journal of Dermatology Oct 2019Scleromyxedema (SM) was previously known to be associated with monoclonal gammopathy. The association of SM and its counterpart lichen myxedematosus (LM) with chronic...
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. The patients' demographic details, cutaneous presentation, associated underlying diseases and hepatitic profile were studied and compared with previous published cases. In all, 28 patients were enrolled, including one SM, 19 LM and eight atypical LM. Of the patients, 50% (n = 14/28) had hepatitis. Of these, 21.4% (n = 6/28) had hepatitis C, 10.7% (n = 3/28) hepatitis B, 7.1% (n = 2/28) concurrent hepatitis B and C, whereas 10.7% (n = 3/28) had alcoholic liver disease. The prevalence of hepatitis C in our patients was 6.5-times higher than that of the general population (28.6% vs 4.4%) and the prevalence of hepatitis B was similar (17.9% vs 17.3%). Polyclonal gammopathy was found in 28.6% (n = 8/28) of the patients and monoclonal gammopathy was found in 7.1% (n = 2/28). The extent of clonality did not correlate with disease severity. Our study did not notice a significant association with monoclonal gammopathy but the prevalence of hepatitis C was found to increase 6.5-times in these patients compared with the general population. We recommend dermatologists to be aware of hepatitis investigations in such patients and future studies are warranted to understand the mechanism behind such association.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Comorbidity; Female; Hepatitis B; Hepatitis C; Humans; Liver Diseases, Alcoholic; Male; Middle Aged; Paraproteinemias; Prevalence; Retrospective Studies; Scleromyxedema; Severity of Illness Index; Skin; Young Adult
PubMed: 31461182
DOI: 10.1111/1346-8138.15039 -
Anais Brasileiros de Dermatologia 2016Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin...
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.
Topics: Biopsy; Fatal Outcome; Humans; Male; Middle Aged; Mucins; Scleromyxedema; Skin
PubMed: 28300892
DOI: 10.1590/abd1806-4841.20164527 -
JAAD Case Reports Aug 2022
PubMed: 35899146
DOI: 10.1016/j.jdcr.2022.06.022 -
Case Reports in Dermatology 2022Scleromyxedema is a rare but important mucinosis disorder of the skin that is presented with dermatological manifestations such as waxy papules, diffuse induration, and...
Scleromyxedema is a rare but important mucinosis disorder of the skin that is presented with dermatological manifestations such as waxy papules, diffuse induration, and nondermatologic involvements like neurological and renal disorders. We report a case series of the data regarding the characteristics and treatment of 14 patients diagnosed with scleromyxedema and their follow-up. Patients entered the study based on scleromyxedema diagnosis criteria. Comorbidities were also recorded to evaluate their effect on the treatment process. Clinicopathological and laboratory findings and responses to their treatment were evaluated separately. There was a significant improvement after administering intravenous immunoglobulin (IVIG). Despite the lack of a definite treatment for this condition, the present study shows that the application of IVIG can improve both cutaneous and systemic symptoms. Younger patients, in particular, responded significantly to the use of IVIG. More studies are required to investigate the potential efficacy of IVIG in the treatment of scleromyxedema.
PubMed: 35950147
DOI: 10.1159/000525211