What Is Perniosis?

Hi everyone!

It’s me again, and I hope you’re doing okay?! As I work my hardest to raise awareness about Ehlers-Danlos Syndrome (EDS) across all of my social media platforms and it’s been a while since I went into detail about my health issues. So, I thought it might be a good idea to talk about my different health issues that are all down to the EDS. If you’re new to my blog and wondering what EDS is then scroll back to a previous post from around two years ago and you’ll see what it is. To put it in simple term, it’s a connective tissue disorder caused by a faulty collagen. 

In this post, I want to talk about Perniosis. I’ll tell you how it affects me and then some information about it. I started getting pain, discomfort and difficulty in putting pressure on my feet. This affected me more during certain weather conditions. At first I thought it was from a twisted ankle injury that I sustained previously so tried to rest it when I could and ignore it other times. I’d seen a GP about it who then referred me to their podiatrist at the local hospital who had special insoles made for me but still after some time, I was still getting the same troubles. So when I come home for good, I went to my own GP who wanted to investigate further so I was then referred to a orthopaedic surgeon at my local hospital who said he didn’t think it was to do with the bones so he sent me to see a vascular surgeon. There with all of my notes, X-rays etc, the surgeon did a few little tests like holding a little pen like device to my skin which was connected to a machine. It didn’t hurt. After a few moments, he sat us down and explained that I have a vascular condition and it’s more than likely to be caused by the EDS. He said ‘it’s like the blood is spasm underneath the surface of the skin and that’s why you’re getting the issues you are.’ It was a relief to finally know what was wrong with my feet especially after being passed around from one specialist to another. I did think to myself ‘oh great, another one to add to the ever extending list of all my conditions! Why does my body have to be so greedy?! Ha!’ Although to be honest, I was used to this by now, as you’ll see from this series of blog posts about all the different conditions I have. Even now after many years of living with Perniosis, it still affects me. The other day when I went into town, I came back home with the bottom of my leg swollen and feeling very tender. There were dark markings on my leg in patches and it felt uncomfortable to touch them. 

According to the rarediseases.org site, it says the following in my own words. Perniosis is an inflammatory disorder that is triggered by exposure to cold and damp weather conditions. (Please note that for me, the hot weather affects it too). It is where the blood vessels are inflamed. It is characterised by painful, itchy, tender skin lesions on the lower legs, toes, hands, feet, ears and face. There are different forms of the disorder. 

Basically, it is an abnormal reaction to the temperature in eg, the cold and hot. A typical feature may be a blue-ish red discolouration of the skin that can cause pain, intense itching, burning and swelling of the skin. Even more so as the body warms up. 

They are unsure of what the cause is. The cold weather causes the small veins or arteries close to the skin to tighten or construct. When the tissues are rewarmed, blood leaks into the tissue and causes it to swell. This means that the swelling irritates the nerves, and pain is the result. Some cases are believed to be genetic factors. Other causes may be of nutrition, local infection, hormonal changes and other systemic diseases - EDS is a multi-systemic connective tissue disorder. 

It is seen in more females than males and usually under the age of 20. 

The related conditions are as follows: 

Raynaud’s Disease. 

Chilblain Lupus Erythromatosus

Vasculitis

Cold Urticaria

It is diagnosed by a physical examination and a close examination of the rewarming process. 

To treat it is to protect the affected area from the cold and warming it carefully and slowly. Try not to scratch or rub the affected area to avoid further damage to the skin. Using Corticosteroid creams may help relieve the intense itching. If you are okay taking medication then they may prescribe Nifedipine which is a calcium blocker that will ease the cold related symptoms of Perniosis. I was on this but decided to not take it anymore as I don’t feel comfortable in taking medication due to personal reasons and the condition doesn’t always affect me. I try to keep my lower legs at room temperature and don’t expose them to the cold. Raising my feet up help eases symptoms as well if they are feeling uncomfortable and hurting from the Perniosis. 

I will be writing about another one of the many conditions that I have in another blog post. I hope that I have been informative and explained it in the simplest way that I can. Hopefully I can help raise just how complex Ehlers-Danlos Syndrome really is and that those who know me will fully understand why I am where I am in my life. Thank you for reading this long post and I will see you in my next one. Take care 😊❤️xxx