Hypovolemic shock due to massive subcutaneous hemorrhage in a patient with musculocontractural Ehlers-Danlos syndrome (mcEDS)

Musculocontractural Ehlers-Danlos syndrome (mcEDS) is a rare autosomal recessive connective tissue disorder characterized by fragility of skin, vasculature and musculoskeletal structures. We report a case of a young male with CHST14(Carbohydrate sulfotransferase 14)-related mcEDS who developed a massive subcutaneous hematoma following minor trauma, necessitating surgical evacuation. This case highlights the potential for life-threatening bleeding complications in mcEDS and underscores the importance of early recognition and multidisciplinary management.

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Publication Details

Journal
Wiener klinische Wochenschrift
Published
2025-08-04
DOI
https://doi.org/10.1007/s00508-025-02577-9
Primary Topic
Connective tissue disorders research
Type
article
Field-Weighted Citation Impact
0.00

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article

Hypovolemic shock due to massive subcutaneous hemorrhage in a patient with musculocontractural Ehlers-Danlos syndrome (mcEDS)

Luise Adam, Roberta Laggner, Stephan Payr, D. Baron
Wiener klinische Wochenschrift
Connective tissue disorders research
article

Hypovolemic shock due to massive subcutaneous hemorrhage in a patient with musculocontractural Ehlers-Danlos syndrome (mcEDS)

Luise Adam, Roberta Laggner, Stephan Payr, D. Baron
article en

Abstract

Musculocontractural Ehlers-Danlos syndrome (mcEDS) is a rare autosomal recessive connective tissue disorder characterized by fragility of skin, vasculature and musculoskeletal structures. We report a case of a young male with CHST14(Carbohydrate sulfotransferase 14)-related mcEDS who developed a massive subcutaneous hematoma following minor trauma, necessitating surgical evacuation. This case highlights the potential for life-threatening bleeding complications in mcEDS and underscores the importance of early recognition and multidisciplinary management.

Wiener klinische WochenschriftVol. 138(1-2)
Medical University of Vienna (AT)
Universität Wien, Medizinische Universität Wien
Good health and well-being
Openalex Percentile: Top 78%
Connective tissue disorders research
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