A Cautionary Tale

The blasting of the innocuous-looking spot.

It's thirteen years since I was diagnosed with probable CLIPPERS and like everyone else, I'm ageing, slowly and hopefully gracefully. The skin on my face is increasingly dry and irritated and as someone who is very fair-skinned I know I need to be careful with sun-exposure. Recently I noticed a small but persistent spot on my cheek and eventually decided to get it checked out. The doctor said that she could see I was taking Azathioprine and that my skin wasn't great, but she couldn't see a record of skin-reviews. I said that I was aware I had to be careful with my skin but I had never been invited for a skin-review. So that's how I found myself referred to the dermatology clinic in the local hospital under the NHS rapid referral scheme which is usually used when there is a cancer concern. The doctor said she was almost certain there was nothing serious present, but with my history I should be looked at quickly.

    The dermatologist diagnosed various keratoses, told me there was nothing to be concerned about, and then produced a liquid nitrogen gun and froze the original innocuous spot. You can see the evolution of it from almost invisible spot to a blister and then slowly healing skin in the picture. Now there is no sign of it.

    I'm taking a few seconds of your time in the hope it will remind anyone on Azathioprine or drugs with similar warnings about sunlight exposure and the need for skin reviews to take them seriously and hopefully head off any unnecessary unpleasantness.  Although I slipped through the original monitoring net, at least someone alert picked up on it when I subsequently did have a concern.

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Living With CLIPPERS by Bill Crum is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.
A Book on CLIPPERS is now available.

A Book on CLIPPERS promotion

Just a quick update that the ebook edition of "A Book on CLPPERS" will be free worldwide on Amazon from tomorrow (Thursday 5th October 2023) for 5 days.

Read other articles in this series at Living With CLIPPERS.


Living With CLIPPERS by Bill Crum is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.
A Book on CLIPPERS is now available.

Updates

On Cheddar Gorge in 2022

Time for a few brief updates. Thanks to those who have read the CLIPPERS book and those who left a rating on Amazon; I hope you enjoyed it or found it useful, or both.  You may have read that Amazon are raising their global printing costs in June 2023, but the CLIPPERS book will remain at it's original pricing for now.

I had a telephone review with my consultant recently and, following a brain-scan last year which reported essentially normal (or at least no change), had nothing much to update him with. It's nice to have retained the same consultant through my CLIPPERS journey, which makes catching up easier (for both of us!). We had our now traditional conversation about future treatment strategy. I've been lucky to remain stable on Azathioprine since being weaned off steroids about 9 months after diagnosis. Taking Azathioprine comes with a small hypothetical risk but trying to compare that against the risk of not taking it for CLIPPERS is very hard. My view has always been that CLIPPERS can cause serious and potentially long-lasting problems, which in my case meant a month in hospital and easily six months in recovery; but at least I did recover. The risk of relapse is real but unfortunately unpredictable. Judging by the number of CLIPPERS case reports still appearing, relapse is still common so as before,  I said I thought that relapse was a bigger gamble and my consultant was happy to support that decision.

The fact that I still can't gauge a risk of relapse made me think. I don't have an inside track to the latest knowledge about CLIPPERS, but reading some of the recent papers  makes me feel that in some ways things haven't changed very much. Here's an example from 2023:

"The diagnosis of CLIPPERS is difficult and requires extensive differential diagnosis. A specific biomarker in serum or cerebrospinal fluid (CSF) for this disorder is currently unknown. The pathogenesis of CLIPPERS remains poorly understood and its nosological* position has not yet been established. Whether CLIPPERS represents an independent, genuine new disorder or a syndrome in the course of diseases with heterogeneous aetiology and/or their precursor stages remains debatable and incompletely clarified."

(*nosological = disease classification including an understanding of mechanism)

I accept everything said in this extract, but it could have been written for virtually any CLIPPERS paper over the last ten years. I hope in another ten years a similar extract will read differently.

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Living With CLIPPERS by Bill Crum is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

Systematic review of CLIPPERS studies

Wind-battered trees on Brean Down

One of the difficulties in studying conditions like CLIPPERS is their rarity meaning that there are comparatively few cases to draw from. CLIPPERS, with it's variability of symptoms and outcomes and difficulties in concrete diagnosis has additional challenges. However, now that there have been over ten years of scientific publications on CLIPPERS there are opportunities to get a better overview by combining together earlier work. Dr Al-Chalabi and colleagues from the University of Toeldo published a paper in 2022  titled "Clinical characteristics, management, and outcomes of CLIPPERS: A comprehensive systematic review of 140 patients from 100 studies" to try and achieve this. A systematic review is where a collection of previous works are combined following a protocol which strives to ensure quality and minimize bias to hopefully come to stronger conclusions than might be possible looking at the individual studies. In the case of this review, the "clinical characteristics, treatment strategies and outcomes" of CLIPPERS were assessed.

My reading of the review is that the authors drew together some common themes from the individual studies but that the amount of variability and uncertainty still hindered their ability to draw firm conclusions, even in over 100 nominal CLIPPERS patients. They found 60% of their cohort were male and the mean age of onset was 46 years (which is very close to the age I first got symptoms). The most common (but not the only) symptoms, in order, were ataxia , diplopia, and dysarthria; my own experience was diplopia first, then quite quickly followed by ataxia, and latterly by some dysarthria just as I began treatment. They also found that around 15% of the patients studies had some form of malignancy which presumably means CLIPPERS wasn't their ultimate diagnosis (but I am not a doctor). In terms of long-term treatment, Azathioprine and Methotrexate, were the most common, but not the only, drugs. 

The authors also report that a shorter time on steroids was associated with an increased risk of CLIPPERS relapse which, to me, is the most interesting of their conclusions, possibly, and very unscientifically and with no evidence, because I have long had a gut feeling that longer steroid tapers might be better. The authors suggest that, going forward, steroid tapers should be very slow, although they don't state what "slow" means - presumably months?

These studies are important and as well as making the most of previous studies can hopefully direct future research.

Read other articles in this series at Living With CLIPPERS.


Living With CLIPPERS by Bill Crum is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.