#TheLikenessofLupus: Lupus and Skin - an intro to Calciphylaxis

Lupus is an overachiever. 

It isn't enough that this lifelong disorder causes so many internal problems...
It can also do a number, externally, on the skin. This is called Cutaneous Lupus.

Mayo Foundation for Medical Education and Research, All Rights Reserved.

There are 3 forms of Cutaneous Lupus (Lupus skin disease: disk-shaped, red, scaly, thick, round lesions generally found on scalp and face, but can occur anywhere on the body. Sometimes referred to the 'malar rash' or 'butterfly rash'):

1. Chronic Cutaneous (Discoid) Lupus 
2. Subacute Cutaneous Lupus (meaning between chronic and acute)
3. Acute Cutaneous Lupus
   

The lesions formed are photosensitive (sensitive to sunlight) and can cause permanent scarring, discoloration, hyperpigmentation, and hair loss depending on the location on the body. There are also other skin conditions that can occur while living with lupus. These include:

• Cutaneous Vasculitis Lesions (typically small, red-purple spots and bumps found on arms and legs - some people mistake it for razor burn sometimes. In some cases, it can result in significant damage to skin tissue.)
  
• Hair Loss (Alopecia)(Can occur for reasons outside of lesions and scarring such as medication use and simply having a lupus flare/inflammation. Hair becomes fragile and breaks easily.)
  
• Raynaud's Phenomenon (a condition where the blood vessels in the hands/feet go into spasm, causing restricted blood flow - your hands and feet turn white. Worsened in cold weather.)
  
• Livedo Reticularis and Palmar Erythema (a condition caused by abnormal rates of blood flow through the capillaries and small arteries. A bluish, lace-like splotchiness that will appear beneath the skin, especially on the legs, giving a "fishnet" appearance. Like Raynaud’s phenomenon. Worsened in cold weather.)
  
• Mucosal Ulcerations (sores in the mouth or nose or, less often, in lining of vaginal tissue - not fun. Painful and uncomfortable.)
  
• Petechiae (tiny red spots on the skin, especially on the lower legs, that result from low numbers of platelet in the blood, a condition called thrombocytopenia.)
  
• Calcinosis Cutis (CC)(caused by a buildup of calcium deposits under the skin. These deposits can be painful - excruciating, really. Can occur as a result of a negative reaction from steroid injections or as a result of kidney failure. There are 5 types: calciphylaxis, dystrophic, metastatic, idiopathic, and iatrogenic CC.)

Wounds UK | Volume 14 | No. 3 | 2018

I have personally experienced most of these skin conditions, but the one that has changed my life in ways I could never imagine was developing one of the most painful, life threatening types of CC called Calciphylaxis. (See Calciphylaxis Resource Page for detailed information.)

Calciphylaxis was first described in 1898 and still remains a poorly understood condition (Breakey et al, 2014). It it normally as a result of an abnormal parathyroid and poor renal function. It has been reported in individuals with normal parathyroid and renal function, but it is often a complication of renal

transplantation and end-stage renal disease (Latus et al, 2011; Breakey et al, 2014; Park et al, 2016). About 1-5% of dialysis patients will develop some form of calciphylaxis or CC (Sulková and Válek, 2010). The mortality rate of Calciphylaxis is high, with more than 50 percent of patients dying within one year of diagnosis.

WARNING: Sensitive Content. NSFW. This is where it gets graphic. Not for the faint of heart or those with weak stomachs. You've been warned. 

So, as you know by now, I have lupus (duh) and my kidneys have been affected (double duh). In another post, I will detail how the kidneys can become involved with lupus (he's from the wrong side of town, after all- I'm kidding....ignore me. I'm being silly personifying lupus like a bad boyfriend) and what my particular case/situation looks like. I mention the kidneys because my particular case of Calciphylaxis was a result of my chronic kidney disease and poor clearance during dialysis treatment (I was doing peritoneal dialysis at the time...I'll explain the world of dialysis in a separate post). 

My experience with Calciphylaxis was a blur. The amount of pain I was in had me so sedated and in a dissociative fugue state. A lot of my memories are really my partner's and parent's recollections. Especially in the beginning stages. Once I was finally in the burn unit at Santa Clara Medical Center in San Jose, CA (the hospital that saved me) and the focus was wound/ulcer/lesion care, I was more "with it" (but, like, barely...because pain and drugs).

Calciphylaxis started out like what felt like a bump on my booty near my tailbone. I honestly thought it was a pimple. C'mon. Admit it. We all get butt pimples. But when I would sit down, it felt like my tailbone was protruding and sharp. Abnormal. Like whatever cushion existed in that space was all of a sudden gone. This was weird to me because a) I was always a cutie with a booty and 2) I always sat on the floor on pillows or whatever. The pain I felt made it almost impossible to do that anymore. and it was spreading.

I go to several hospitals in the beginning and they were unsure what it was, initially. On top of dealing with this development, I was also struggling with other internal issues (at one point I ended up intubated because my lungs had a hole and was filling up with blood. I couldn't breathe on my own...I was just spitting up blood. And between hospitals - like literally driving from one to another - my husband, mother, and I get into a car accident that totals our car. I remember distinctly having to be alone in this new ER because we all got split up and taken to different hospitals after the accident. So my family, medical teams, and myself were a bit overwhelmed, to say the least). 

I eventually get to University of California San Francisco Medical Center and after a lot of tests and confusion and pain and time, they diagnose and treat the Calciphylaxis. Yay, right? I'm cured! .........ha! If only it were that easy.

That was only the beginning...

It spread to my lower extremeties and what needed to be treated were these persistent, deep ulcers. If I didn't act quick, I could lose my legs or, worse, my life. The doctors at UCSF told me that they were able to treat the condition, but the physical signs and symptoms (necrosis and ulcers/wounds) would get so much worse before it got better. I was discharged with a LARGE (I'm talking very, very big) bottle of liquid Dilaudid (basically liquid heroin), a dermatology consult in 2-3 weeks, a trained husband in wound care, and a prayer. It left me at the beginning stages of what I imagine it must feel like to be filleted alive without having to move at all. 

It quickly became clear to us that this was not manageable at home. 

The amount of pain I was in was out of this world that this is where things get blurry. I was on enough narcotics to kill an army of elephants. 

In order to reduce the risk of infection and the spread of necrosis, it became apparent that these deep ulcers needed debriding (to remove damaged tissue) and skin grafts. This would occur over 11 surgeries, little by little. This is eventually what it looked like (brace yourself):

After debriding.

After a few skin graft surgeries.

My burn unit surgical team literally had to scoop out the dead/dying tissue, then borrow skin from my calves to go on my thighs and butt. I was only the 2nd or 3rd case they had ever seen since most people don't survive. I had an incredible team and feel incredibly lucky. My legs are still healing 2+ years later. 

I lost my ability to sit up, stand, walk, or, really, do much of anything for myself. Between surgeries, I also did physical therapy to regain my mobility. I can walk again, but I cannot run (in a zombie apocalypse, just leave me behind) and still struggle with balance. Let me tell you, when your body and your brain aren't communicating, it's a hard thing to shake and it does a bang-up job on a person's mental health...but that's a whole other conversation.

66% of people with lupus will develop some form of skin disease (lupus.org). In fact, skin conditions comprise 4 of the 11 criteria used by the American College of Rheumatology for classifying and diagnosing lupus (hopkinslupus.org).  These signs and symptoms vary from patient to patient. There have been cases where a patient shows no signs of skin-related lupus symptoms. Then they have cases like mine. It can go either way. 

Listen to you body when it is trying to tell you something. Care for it like you would an infant - delicately. Be patient with yourself. and kind.

Til next time...

Solange the Little Lupie 

xx