What are the treatment options for itching in a patient with Postural Orthostatic Tachycardia Syndrome (POTS) and Ehlers-Danlos Syndrome (EDS)?

Treatment Options for Itching in Patients with POTS and EDS

For patients with Postural Orthostatic Tachycardia Syndrome (POTS) and Ehlers-Danlos Syndrome (EDS) experiencing itching, treatment should focus on addressing potential mast cell activation, which is commonly associated with these conditions, using histamine receptor antagonists and mast cell stabilizers as first-line therapy. 1, 2

Understanding the Connection

• Patients with EDS, particularly hypermobile EDS (hEDS), frequently experience concurrent POTS and mast cell activation syndrome (MCAS), with research showing a marked percentage (31%) of MCAS among patients diagnosed with both POTS and EDS 3
• The relationship between these three conditions creates a complex clinical picture where symptoms like itching may be attributed to mast cell activation 3, 4

Diagnostic Approach for Itching

• When MCAS is suspected as the cause of itching in POTS/EDS patients, diagnostic testing with serum tryptase levels should be collected at baseline and 1-4 hours following symptom flares; increases of 20% above baseline plus 2 ng/mL indicate mast cell activation 1
• Patients with suspected MCAS should be referred to an allergy specialist or mast cell disease research center for additional testing (e.g., urinary N-methylhistamine, leukotriene E4, 11β-prostaglandin F2) 1

Treatment Algorithm for Itching in POTS/EDS

First-Line Treatments:

1. Histamine Receptor Antagonists

   • H1 blockers (e.g., cetirizine, loratadine) and H2 blockers (e.g., famotidine) should be used in combination 2
   • These medications help control histamine-mediated itching that commonly occurs in patients with MCAS 1, 2

2. Mast Cell Stabilizers

   • Medications that prevent mast cell degranulation (e.g., cromolyn sodium, ketotifen) 2
   • These help reduce the release of histamine and other inflammatory mediators that cause itching 1

3. Trigger Avoidance

   • Identify and avoid known triggers such as certain foods, alcohol, strong smells, temperature changes, mechanical stimuli, emotional distress, or specific medications (e.g., opioids, NSAIDs) 1, 2

Second-Line Approaches:

1. Dietary Modifications

   • Low-histamine diet may significantly improve itching symptoms 1, 4
   • Other elimination diets (e.g., gluten-free, dairy-free) should be implemented with proper nutritional counseling to avoid restrictive eating pitfalls 1

2. Topical Treatments

   • Capsaicin cream may be applied to affected areas 3-4 times daily (for adults and children 18 years and older) 5
   • Wash hands thoroughly with soap and water immediately after application unless treating hands 5

3. POTS Management (which may indirectly improve itching by reducing overall symptom burden)

   • Increase fluid and salt intake 1
   • Implement exercise training programs 1, 6
   • Use compression garments to reduce venous pooling 1, 6
   • Consider pharmacological treatments for volume expansion, heart rate control, and vasoconstriction in consultation with cardiology or neurology if conservative measures fail 1, 7

Special Considerations and Pitfalls

• Avoid opioids for pain management in these patients as they can trigger mast cell activation and worsen itching 1
• Beware of polypharmacy as patients with EDS/POTS often take multiple medications that may interact or have cumulative side effects 7
• Consider occupational therapy to help manage symptoms and improve quality of life through environmental modifications, adaptive equipment, and energy conservation techniques 6
• Monitor for comorbid conditions such as gastrointestinal manifestations that may exacerbate symptoms or require additional treatment 1

Multidisciplinary Approach

• Integrated care involving gastroenterology, cardiology, neurology, and allergy specialists is essential for optimal management 1, 7
• Regular follow-up to assess treatment efficacy and adjust therapies as needed 1
• Patient education about the relationship between EDS, POTS, and mast cell activation is crucial for treatment adherence and symptom management 6, 7