Impact of Hypermobile Ehlers-Danlos Syndrome on Adipose Tissue

Connective Tissue Abnormalities in Adipose Tissue

Skin biopsies in hEDS patients show altered collagen fibril structure that triggers fibroblast dysfunction within connective tissue, affecting adhesion and cytoskeletal response 1
Evidence indicates that in hEDS, connective tissue is softer and less stiff than in control subjects, which likely extends to adipose tissue that contains significant amounts of connective tissue 1

Mast cells migrate into connective tissue and mucosa, including adipose tissue, where they can proliferate and potentially cause inflammatory responses 1, 2
In hEDS, there is evidence of early or excessive mast cell degranulation leading to mast cell activation disorders that can affect multiple tissue systems including adipose tissue 1
When Mast Cell Activation Syndrome (MCAS) is suspected in hEDS patients, treatment with histamine receptor antagonists and mast cell stabilizers may benefit adipose tissue function 1
MCAS symptoms can be triggered by mechanical stimuli (e.g., friction), which may explain why adipose tissue in hypermobile areas could experience increased inflammation 2

Dietary interventions may be necessary for hEDS patients with adipose tissue abnormalities, particularly when MCAS is present, including low-histamine diets 1, 2
Mechanical stimuli that trigger mast cell activation should be minimized to reduce inflammation in adipose tissue 2

The Beighton scale is essential for diagnosing hEDS, with age-specific thresholds: ≥6/9 points in children before puberty, ≥5/9 points for adults up to age 50, and ≥4/9 points for adults over 50 3
Up to 60% of hEDS patients experience gastrointestinal symptoms, which may be related to altered connective tissue affecting both the GI tract and surrounding adipose tissue 3

Treatment should focus on the most prominent symptoms, including those that may be related to adipose tissue abnormalities 2
For patients with suspected MCAS affecting adipose tissue, avoiding triggers such as certain foods, alcohol, strong smells, temperature changes, and mechanical stimuli is recommended 1
Special diets including low-histamine approaches may be beneficial but should be delivered with appropriate nutritional counseling to avoid restrictive eating 1
Integrated care involving multiple specialties may be necessary for comprehensive management of hEDS patients with adipose tissue abnormalities 2