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Cutaneous metaplastic synovial cyst in Ehlers-Danlos syndrome.
J Cutan Pathol. 2020 Mar 14 [Online ahead of print]JC

Abstract

Classic Ehlers-Danlos syndrome (EDS) is a connective tissue disorder characterized by laxity. The skin, as one of the organs involved, shows hyperextensibility, which makes it prone to trauma. In this context, it would seem logical for cutaneous synovial metaplasia, which is considered a form of repair, to be commonly found in cases of EDS. However, there are only two previously published cases of synovial metaplasia in EDS. We present a third case in a 56-year-old woman with painful redundant skin in both elbows and knees for whom a skin fold of the left elbow was removed to relieve her symptoms. The biopsy showed preservation of the elastic and collagen fibers. The main alteration was the evidence of dermal cystic spaces lined by fibrinoid rests with focal pseudopapillary projections. However, in some zones the cellular lining was preserved, and it was composed of vimentin-positive, fibroblast-like flat, elongated cells, as well as CD68-positive macrophages. No birefringent particles were found in an examination under polarized light.

Authors+Show Affiliations

Department of Cellular Pathology, Hospital Universitario El Bierzo, Ponferrada, Spain. Department of Cellular Pathology, Hospital de la Reina, Ponferrada, Spain. Research Department, Institute for Biomedical Research of A Coruña (INIBIC), University of A Coruña (UDC), A Coruña, Spain.Department of Dermatology, Hospital Universitario El Bierzo, Ponferrada, Spain.

Pub Type(s)

Case Reports

Language

eng

PubMed ID

32170975

Citation

Fernandez-Flores, Angel, and Juan M. Barja-Lopez. "Cutaneous Metaplastic Synovial Cyst in Ehlers-Danlos Syndrome." Journal of Cutaneous Pathology, 2020.
Fernandez-Flores A, Barja-Lopez JM. Cutaneous metaplastic synovial cyst in Ehlers-Danlos syndrome. J Cutan Pathol. 2020.
Fernandez-Flores, A., & Barja-Lopez, J. M. (2020). Cutaneous metaplastic synovial cyst in Ehlers-Danlos syndrome. Journal of Cutaneous Pathology. https://doi.org/10.1111/cup.13684
Fernandez-Flores A, Barja-Lopez JM. Cutaneous Metaplastic Synovial Cyst in Ehlers-Danlos Syndrome. J Cutan Pathol. 2020 Mar 14; PubMed PMID: 32170975.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cutaneous metaplastic synovial cyst in Ehlers-Danlos syndrome. AU - Fernandez-Flores,Angel, AU - Barja-Lopez,Juan M, Y1 - 2020/03/14/ PY - 2020/01/13/received PY - 2020/02/23/revised PY - 2020/03/10/accepted PY - 2020/3/15/pubmed PY - 2020/3/15/medline PY - 2020/3/15/entrez KW - Ehlers-Danlos KW - cutaneous synovial metaplasia KW - synovial cyst JF - Journal of cutaneous pathology JO - J. Cutan. Pathol. N2 - Classic Ehlers-Danlos syndrome (EDS) is a connective tissue disorder characterized by laxity. The skin, as one of the organs involved, shows hyperextensibility, which makes it prone to trauma. In this context, it would seem logical for cutaneous synovial metaplasia, which is considered a form of repair, to be commonly found in cases of EDS. However, there are only two previously published cases of synovial metaplasia in EDS. We present a third case in a 56-year-old woman with painful redundant skin in both elbows and knees for whom a skin fold of the left elbow was removed to relieve her symptoms. The biopsy showed preservation of the elastic and collagen fibers. The main alteration was the evidence of dermal cystic spaces lined by fibrinoid rests with focal pseudopapillary projections. However, in some zones the cellular lining was preserved, and it was composed of vimentin-positive, fibroblast-like flat, elongated cells, as well as CD68-positive macrophages. No birefringent particles were found in an examination under polarized light. SN - 1600-0560 UR - https://www.unboundmedicine.com/medline/citation/32170975/Cutaneous_metaplastic_synovial_cyst_in_Ehlers-Danlos_syndrome L2 - https://doi.org/10.1111/cup.13684 DB - PRIME DP - Unbound Medicine ER -
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