Rare Cutaneous Manifestations of Erdheim Chester Disease: A Case Report and Literature Review.
| Autor: | Vasandani N; Department of Plastic and Reconstructive Surgery, University Hospital Galway, Galway, IRL., Low JE; Department of Plastic and Reconstructive Surgery, University Hospital Galway, Galway, IRL., Liau YH; Department of General Surgery, University Hospital Galway, Galway, IRL., Ergun A; Department of Plastic and Reconstructive Surgery, University Hospital Galway, Galway, IRL., Balakrishnan T; Department of Plastic and Reconstructive Surgery, University Hospital Galway, Galway, IRL. |
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| Jazyk: | angličtina |
| Zdroj: | Cureus [Cureus] 2023 Jun 20; Vol. 15 (6), pp. e40712. Date of Electronic Publication: 2023 Jun 20 (Print Publication: 2023). |
| DOI: | 10.7759/cureus.40712 |
| Abstrakt: | Erdheim Chester disease (ECD) is a rare and complex non-Langerhans histiocytic systemic disease that affects multiple organ systems, including the bones, heart, lungs, and central nervous system. Fewer than 1,000 cases have been reported in the medical literature and dermatological manifestations of the disease are rare but can provide valuable diagnostic clues for this challenging disease. The cutaneous manifestations of ECD can take many forms, including nodules, plaques, papules, and xanthomas. These lesions can occur on any part of the body and may be solitary or multiple. Cutaneous manifestations of ECD have been reported to occur in up to 20% of cases, but the true prevalence may be higher, as many cases may go undiagnosed. We present the case of a 62-year-old gentleman with a history of ECD currently on vemurafenib who presented with multiple painless subcutaneous nodules on his back after an excision biopsy under local anesthetic revealed histological features of ECD. The objective of this case report is to raise awareness of ECD and its dermatological manifestations. Further research is warranted to better understand the pathogenesis and morphology of cutaneous involvement in ECD. Competing Interests: The authors have declared that no competing interests exist. (Copyright © 2023, Vasandani et al.) |
| Databáze: | MEDLINE |
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