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Rare Cutaneous Manifestations of Erdheim Chester Disease: A Case Report and Literature Review.
Vasandani, Nikhil Low, Jing Er Liau, Yun Hui Ergun, Alexander Balakrishnan, Theogren
Vasandani, Nikhil
Low, Jing Er
Liau, Yun Hui
Ergun, Alexander
Balakrishnan, Theogren
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Date
2023-06-20
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Keywords
braf mutation
cd68+
diagnosis of rare cases
erdheim chester disease
non-langerhans cell histiocytosis
pilomatrixoma
rare skin disease
skin nodule
cd68+
diagnosis of rare cases
erdheim chester disease
non-langerhans cell histiocytosis
pilomatrixoma
rare skin disease
skin nodule
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Abstract
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.
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en
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ISSN
2168-8184
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DOI
10.7759/cureus.40712
PMID
37485173