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Clinical Case Reports Aug 2022Arndt-Gottron (S-AG) syndrome or scleromyxedema is a scarce disease characterized by a generalized papular and sclerodermoid eruption and systemic manifestations that...
Arndt-Gottron (S-AG) syndrome or scleromyxedema is a scarce disease characterized by a generalized papular and sclerodermoid eruption and systemic manifestations that can lead to significant morbid-mortality. Interesting, S-AG can be associated with a paraprotein. We report an exceptional scleromyxedema case associated with a benign monoclonal gammapathy in an old woman.
PubMed: 35937020
DOI: 10.1002/ccr3.6122 -
BMJ Case Reports Oct 2018
Topics: Administration, Intravenous; Adult; Biopsy; Diagnosis, Differential; Fibroblasts; Humans; Immunoglobulins; Immunologic Factors; Immunosuppressive Agents; Male; Mucins; Polychondritis, Relapsing; Scleromyxedema; Skin; Thalidomide; Treatment Outcome
PubMed: 30317211
DOI: 10.1136/bcr-2018-227144 -
JAAD Case Reports Sep 2017
PubMed: 28831414
DOI: 10.1016/j.jdcr.2017.04.005 -
Journal of the European Academy of... Jul 2023Primary cutaneous mucinoses (PCM) are rare diseases characterized by dermal or follicular mucin deposits.
BACKGROUND
Primary cutaneous mucinoses (PCM) are rare diseases characterized by dermal or follicular mucin deposits.
OBJECTIVES
A retrospective study characterizing PCM to compare dermal with follicular mucin to identify its potential origin on a single-cell level.
MATERIAL AND METHODS
Patients diagnosed with PCM between 2010 and 2020 at our department were included in this study. Biopsy specimens were stained using conventional mucin stains (Alcian blue, PAS) and MUC1 immunohistochemical staining. Multiplex fluorescence staining (MFS) was used to investigate which cells were associated with MUC1 expression in select cases.
RESULTS
Thirty-one patients with PCM were included, 14 with follicular mucinosis (FM), 8 with reticular erythematous mucinosis, 2 with scleredema, 6 with pretibial myxedema and one patient with lichen myxedematosus. In all 31 specimens, mucin stained positive for Alcian blue and negative for PAS. In FM, mucin deposition was exclusively found in hair follicles and sebaceous glands. None of the other entities showed mucin deposits in follicular epithelial structures. Using MFS, all cases showed CD4+ and CD8+ T cells, tissue histiocytes, fibroblasts and pan-cytokeratin+ cells. These cells expressed MUC1 at different intensities. MUC1 expression in tissue histiocytes, fibroblasts, CD4+ and CD8+ T cells, and follicular epithelial cells of FM was significantly higher than the same cell types in the dermal mucinoses (p < 0.001). CD8+ T cells were significantly more involved in expression of MUC1 than all other analysed cell types in FM. This finding was also significant in comparison with dermal mucinoses.
CONCLUSION
Various cell types seem to contribute to mucin production in PCM. Using MFS, we showed that CD8+ T cells seem to be more involved in the production of mucin in FM than in dermal mucinoses, which could indicate that mucin in dermal and follicular epithelial mucinoses have different origins.
Topics: Humans; Mucinoses; Mucins; Retrospective Studies; Alcian Blue; Scleromyxedema; Staining and Labeling
PubMed: 36807595
DOI: 10.1111/jdv.18992 -
BMJ Case Reports Feb 2015
Topics: Biopsy; Diagnosis, Differential; Female; Humans; Scleromyxedema
PubMed: 25721845
DOI: 10.1136/bcr-2015-209462 -
Clinical Case Reports Feb 2020In patients with asymptomatic papules of hands and feet, a clinical differential of acral persistent papular mucinosis should be thought of.
In patients with asymptomatic papules of hands and feet, a clinical differential of acral persistent papular mucinosis should be thought of.
PubMed: 32128185
DOI: 10.1002/ccr3.2639 -
BMJ Case Reports Jan 2019
Topics: Black People; Caribbean Region; Cytoreduction Surgical Procedures; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Male; Middle Aged; Paraproteinemias; Scleromyxedema
PubMed: 30665930
DOI: 10.1136/bcr-2018-227588 -
International Journal of Dermatology Sep 2014
Topics: Humans; Immunosuppressive Agents; Male; Scleromyxedema; Thalidomide
PubMed: 25124465
DOI: 10.1111/ijd.12835 -
JAAD Case Reports Dec 2023
PubMed: 38156097
DOI: 10.1016/j.jdcr.2023.10.010 -
Indian Dermatology Online Journal 2017
PubMed: 28217476
DOI: 10.4103/2229-5178.198779