Sunday, 16 April 2017

Skin Involvement in Scleroderma Raynaud's Rare Disease - Nicola Whitehill

The skin is the largest organ in the body and is affected in most scleroderma patients. The exception to this rule is the limited subset version of scleroderma patients.

See Day 1 of my Scleroderma Awareness Month 2016 campaign

At the time of my diffuse scleroderma (dcSSc) diagnosis in 1997, I presented with extremely tight skin across my chest, arms, hands, face, legs … all over my body. My hands and fingers were puffy with tight, itchy, shiny skin and painful joints. I was unable to make a fist and could not remove bottle tops, the lids of jam jars, or the like, and had other symptoms (joint pain, gastro-intestinal, pain, lethargy, fatigue, and so on).

I discussed this on Day 4

After years of chemotherapy and immunsuppressants — I am now 12 years cytotoxic drug free — my skin has reversed to 97% normal healthy skin, according to my last medical assessment in May 2016, with the dedicated scleroderma world expert Professor Chris Denton.

The Modified Rodnan Skin Score (mRSS) is the standard measurement assessment test used to identify scleroderma skin activity.
A skin score is important because:
  • Skin score often peaks within one year of dcSSc onset
  • There is an association between severe skin involvement and burden of organ-based complications
  • Death or major organ-based morbidity occurs in 50% of dcSSc cases within 3 years of disease onset
  • Stabalization or improvement in skin sclerosis within 2 years of diagnosis may confound studies designed to prevent skin score worsening
  • Latent Trajectory Modelling identifies distinct subgroups defined by skin score

I have a daily symptom management routine that includes bathing and moisturising my skin. For the bath, I use liquid petroleum (Oilatum Plus) and then, after towel drying, I use Hydromol emollient cream all over my body.

I use sensitive skin skincare laundry products and only wear clothing that is soft against my skin. Sadly, my Armani jeans have remained at the bottom of my wardrobe since diagnosis, as denim is too heavy against my skin, causing my inner Bond girl to go AWOL.

As with most things scleroderma-related, nothing is that simple, and although I have regained the elasticity and texture of healthy skin, I am challenged with skin-related symptoms caused by the scleroderma, such as calcinosis, telangiectasia, Bowen’s disease, and a possible basal cell carcinoma on my forehead.

I discussed these skin challenges on days 10, 18, 19, 27 and 28 of Scleroderma Awareness Month, all worth reviewing:
The mystery of the calcinosis/basal cell carcinoma (BCC) will be investigated in two weeks time, which I am trying to remain calm about, having had a very impromptu, if brief, meltdown in a phone call with the local dermatologist’s secretary to book the appointment last week. I had spoken with this secretary a few years ago, when this calcinosis/BCC mystery commenced its journey, and she remembered me, so luckily she understood that I was having an uncharacteristic, albeit embarrassing, reaction, and she was most kind and supportive. I have already purchased the chocolates to take to her on the appointment day!

I know that most of my fellow patients will wholly understand my little blip when booking another medical appointment due to another complication of scleroderma. I also know that I am very blessed to have gotten to year 19 without a skin biopsy, and I am in awe of my fellow (much braver than I) patients, who have been subjected to skin biopsies when their skin is tight and inflamed.

The small skin patch of Bowen’s on my left knee is thought to have been caused by sun damage. I cannot stress enough how important it is to wear sun protection, especially if you are taking — or have taken — any of the chemotherapy and immunotherapy drugs widely used for scleroderma symptom suppression. I went into more detail about this in The Magic of Summer. Read about it here

I discussed my annual monitoring tests here 

As with the management of most of my Raynaud’s and scleroderma symptoms – prevention is better than cure, since there is no cure.

So, I now avoid being in any direct sunlight in the hope of preventing further unwanted scleroderma skin complications, as, I want to feel good ~ living the dream, scleroderma style.

This article was written and published in August 2016 for my Column with Scleroderma News

To read my articles:

  • the importance of an early diagnosis click here 

  • the role of medical research click here

  • the importance of expert specialist centres click here

  • diet and nutrition click here

  • taking part in research trials click here

  • the UK guidelines for management and treatment of Scleroderma click here

  •  my skin is cured from scleroderma click here

Please DONATE to help fund medical research at the Scleroderma Unit at The Royal Free hospital where 100% of your donation will be used to fund vital medical research. Thank You.

Living the dream, scleroderma style, hoping for a cure

#SclerodermaFreeWorld #RaynaudsFreeWorld
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April 2017.

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