Annular elastolytic giant cell granuloma in a patient with Ehlers–Danlos syndrome
| Dublin Core | PKP Metadata Items | Metadata for this Document | |
| 1. | Title | Title of document | Annular elastolytic giant cell granuloma in a patient with Ehlers–Danlos syndrome |
| 2. | Creator | Author's name, affiliation, country | Tatiana A. Gaydina; Pirogov Russian Scientific Research Medical University; Federal Center of Brain Research and Neurotechnologies of FMBA of Russia; Россия |
| 2. | Creator | Author's name, affiliation, country | Olga I. Patsap; Federal Center of Brain Research and Neurotechnologies of FMBA of Russia; Россия |
| 2. | Creator | Author's name, affiliation, country | Raisa T. Tairova; Pirogov Russian Scientific Research Medical University; Federal Center of Brain Research and Neurotechnologies of FMBA of Russia; Россия |
| 3. | Subject | Discipline(s) | |
| 3. | Subject | Keyword(s) | annular granuloma; annular elastolytic giant cell granuloma; Ehlers–Danlos syndrome; dermatoscopy; microangiopathy; COL3A1 gene defect; hereditary connective tissue diseases; clinical case |
| 4. | Description | Abstract | The article presents a clinical case of annular elastolytic giant cell granuloma (AEGCG) in a young patient with a vascular type of Ehlers–Danlos syndrome. The first clinical manifestations of AEGCG appeared on the skin in the right subclavian area about two years ago. Subsequently, new rashes appeared on the skin of the upper and lower extremities up to four new foci per year. The patient underwent ambulatory therapy as a solution of calcium gluconate 10% 5.0 ml No 10 i/v in every other day; a solution of chloropyramine hydrochloride 1.0 ml No 10 i/m every other day; betamethasone + salicylic acid ointment applied to the affected areas of the skin 2 times a day for 2 weeks. The treatment was ineffective, the rashes did not regress. External therapy with tacrolimus was carried out next, 0.1% ointment 2 times a day in the form of applications for 24 weeks but also without effect. The patient by herself started to take a dietary supplement containing 400 mg of collagen in 1 tablet; 3 types of amino acids 20 mg; vitamins B2 1.1 mg; B6 1.5 mg; calcium pantothenate 5 mg 2 tablets a day during meals. A month after the start of the application, she noticed a slight paling of the rashes. At the moment, the patient is under follow up. |
| 5. | Publisher | Organizing agency, location | Rossijskoe Obschestvo Dermatovenerologov i Kosmetologov |
| 6. | Contributor | Sponsor(s) | |
| 7. | Date | (DD-MM-YYYY) | 19.09.2022 |
| 8. | Type | Status & genre | Peer-reviewed Article |
| 8. | Type | Type | Research Article |
| 9. | Format | File format | PDF (Rus), |
| 10. | Identifier | Uniform Resource Identifier | https://vestnikdv.ru/jour/article/view/1338 |
| 10. | Identifier | Digital Object Identifier (DOI) | 10.25208/vdv1338 |
| 11. | Source | Title; vol., no. (year) | Vestnik dermatologii i venerologii; Vol 98, No 4 (2022) |
| 12. | Language | English=en | ru |
| 13. | Relation | Supp. Files |
Fig. 1. Thin skin with translucent blood network, acrogeria. The patient’s hands pressed the biopsy sites with gauze swabs (716KB) Fig. 2. The photo was taken on 04.09.2021. There are ring-shaped rashes on the skin in right area under the buttocks, formed by small, contiguous, dense, hemispherical, slightly flattened shiny red dermal papules (41KB) Fig. 3. A — an annular focus on the right shin measuring 3 cm in diameter, arrow 1 indicates the location of the biopsy. B — dermatoscopic picture of rashes on the border with healthy skin (1001KB) Fig. 4. A fragment of the dermis with the presence of a scattered lymphocytic infiltrate, a giant multinucleated cell (circled with a black line). There is deposition of hemosiderin granules, fullness of capillary vessels, acantholysis sites, degradated elastic fibers. Staining with hematoxylin and eosin, ×400 (915KB) Fig. 5. Dystrophic and reactive changes of the epidermis, pronounced mitotic activity. There is no mucin deposition in the dermis. PAS stain, ×400 (1MB) Fig. 6. A — four ring-shaped foci on the left shin, the maximum focus is 2.5 cm in diameter, arrow 1 indicates the location of the biopsy. B — dermatoscopic picture of rashes on the border with healthy skin (1023KB) Fig. 7. In the dermis, fragments of collagen and elastic fibers with deformation and fragmentation, lymphocytic infiltrate (region 1), giant multinucleated cell (region 2). Staining with hematoxylin and eosin, ×400 (807KB) Fig. 8. Elastophagocytosis. A giant multinucleated cell with intracytoplasmic inclusion of an elastic fiber fragment was found subepidermally. Mallory stain, ×400 (854KB) Fig. 9. Elastolysis. Moderate hypertrophy and deformation of collagen fibers, degenerative changes and disorganization of elastic fibers in the dermis. Mallory stain, ×400 (2MB) |
| 14. | Coverage | Geo-spatial location, chronological period, research sample (gender, age, etc.) | |
| 15. | Rights | Copyright and permissions |
Copyright (c) 2022 Gaydina T.A., Patsap O.I., Tairova R.T. This work is licensed under a Creative Commons Attribution 4.0 International License. |