Your Questions Answered: Conference 2019

Dr. Pradeep Chopra and Dr. Andrew Maxwell

1. What should be considered if a patient has MCAS and a variable blood pressure going from low normal to very high?

MCAS can trigger POTS. If that is the case, then treating MCAS first would make sense. 

This is always tough to manage since it’s difficult to give medications that enhance BP if sometimes it changes to very high. It makes me want to look for an auto-immune reason in particular antibodies that act on the cardiovascular receptors and then consider management with IVIG.

2. When exercising and/or during exertion, head pressure/ache and heart beat pulse in ear increases…is this common in idiopathic intracranial hypertension?

Yes, this is common in intracranial hypertension. 

Can also be common in POTS situations where diastolic pressures are low with wide pulse pressures. For those patients, correcting the volume and volume distribution problem can correct this problem.

3. What would cause numbness in the arms while sleeping on your back?

This could be a feature of thoracic outlet syndrome. If not, check neck instability.

It could be a potential Chiari issue.

Dr. Scott Walsh

1. How useful is a skin biopsy in helping determine what type of EDS a patient has if they show skin signs of a couple different types?

It is not very helpful in differentiating hypermobile and classic type as they both show similar features, but if there is concern about rare types like dermatosparaxis, it can be very helpful.

2. Is small blood vessel fragility (petichiae that is recurrent/spontaneous, bruising) combined with thin / translucent skin a common finding in the genetically determinable types of EDS, and would skin biopsy be useful?

It is a common feature in vascular EDS and some of the very rare types that are due to proteoglycan abnormalities.  A skin biopsy will typically show variation in collagen size and less collagen than normal and would help support a diagnosis of EDS, but wound not be a defining criterion.

3.  What other skin disorders are comorbid with EDS?

There are many skin findings that can occur that have to do with herniations (different layers of skin breaking through each other) such as ganglions, piezogenic pedal papules, etc; easy bruising from blood vessel fragility.

4.  What do you recommend to replace Zantac?

  • famotidine or cimetidine in women
  • famotidine only in men