Leukocytoclastic vasculitis

Left leg 2013-03-17, the day before my birthday. I didn't photograph my other leg at the time because I was just trying to show mom the LV itself, not the secondary infection that had set in on the right leg. Also, I was in intense pain, so I wasn't exactly thinking about chronicling.

This article is a stub. I'll get around to lengthening it when I feel like it :)

TL;DR

For the TL;DR crowd: In February I developed a condition called leukocytoclastic vasculitis, which is essentially a disease where the immune system decides your blood vessels are foreign invaders and attempts to destroy them. It appears to be chronic, however, there doesn't appear to be internal organ involvement, and it looks like we're finally getting it under control with oral steroids.

2013-06-27: *Deep breath* Here goes...

When your dermatologist hands you a copy of the page from her reference book and you see the first line describing your condition includes "small-vessel necrotizing vasculitis", the teenage boy in you reading his mom's medical encyclopedia starts shouting "Cool! I have something with necrotizing in the description!", but then the middle-aged man in you kicks in and says "YOU FOOL! DON'T YOU UNDERSTAND THAT MEANS DEATH! YOUR OWN IMMUNE SYSTEM IS OUT TO KILL YOU!" However, the teenager mumbles under his breath as he grabs the keys for the new car and dodges out the door, "I still think it's cool."

Leukocytoclastic vasculitis is the most common form of small-vessel necrotizing vasculitis[...]   The characteristic lesions are referred to as palpable purpura. The lesion begins as a localized area of cutaneous hemorrhage that acquires substance and becomes palpable as blood leaks out of damaged venules. Nodules, bullae, and ulcers may arise from these purpuric areas and indicate more severe vessel imflamation and necrosis[...]   Prodromal symptoms include fever, malaise, myalgia, and joint pain. Small lesions itch and are painful; nodules, ulcers, and bullae may be very painful.   Lesions appear in crops and last 1 to 4 weeks. Recurrences are common and new crops may appear for weeks, months, or years. Clinical Dermatology: A Color Guide to Diagnosis and Therapy, page 376

This is a post I've been promising to write for a long time now, but I've been reluctant to put it out to the public and have only told a handful of close friends. I've procrastinated on it until I knew what it was, then until we saw if it involved the kidneys, then until I got confirmation from the biopsy, then seeing how the treatment was working. However, it's time to stop procrastinating. I have e-mails from friends casually asking how I am that I haven't replied to in months. Our annual family get-together is in a few weeks and my father's birthday is today, so people are going to be wondering why I'm not going. So, here goes...

About a week after my friend Jana came to visit me in February, I started getting small red spots around my knee. They itched and so at first I thought they might be bedbug bites, since I had spent nearly two months in hotels at field tests at SRNL, in CO for the holidays, and after totaling my car in Iowa. A week later, the spots had spread across to the other leg and became more of an itchy rash on my thigh. In the beginning of March, I had spots on my left hand and swelling and joint stiffness in my hands. At this point, the spots on my legs started turning purple and started coalescing. A week later, a secondary infection had set in on my right leg and the spots were turning black. At this point, when I showed our secretary, she told me "Go to an emergency room NOW!" This finally made me take it seriously and I went to see my doctor, who then referred me to a dermatologist. The dermatologist instantly recognized it and told me it was leukocytoclastic vasculitis, giving me the above page from her reference manual. We started a course of oral antibiotics to fight the secondary infection and started topical steroids and antibiotics to try to bring it under control.

At this point, I took two weeks of sick leave then switched over to half-time sick leave for the next month. (Yay for benefits!) Due to the swelling in the legs, I was on bedrest much of this time, which led to the spread of the rash form up onto my back, arms, and lower abdomen.

Expressions I've experienced since, aside from the rash and lesions, include leaking blisters (bullae), blisters that dry up and give you fun times playing with the dry hockey pucks floating under your skin, and ulcerations. (The ulcerations take the longest to heal.)

• symptoms
• stages (rash/itch, purpura/sensitivity, RLS from hell (acid needle), oozing/ice pick hypersensitivity
• hypersensitivity, screaming
• malaise, insomnia
• Scare with bloody underwear
• blood in all pieces of clothing.... *sigh*
• bloody socks are a regular thing.
• causes
• depression
• tracking progress with band-aids and bandages

Biopsy results

On 2013-05-10 I went in to a surgeon to get a two-punch biopsy on a fresh lesion. The biopsy results indicated the following:

COMMENT: THE HISTOLOGIC CHANGES SUGGEST A MIXED VASCULITIC PROCESS OF MEDIUM AND SMALL-SIZED BLOOD VESSELS WITH SOME AREAS THAT ARE LEUKOCYTOCLASTIC IN OTHERS WHICH ARE LYMPHOID. THE SECOND TISSUE SAMPLE FRAGMENT HAS BEEN SUBMITTED FOR IMMUNOFLUORESCENCE STUDIES WHICH WILL BE CORRELATED IN AN AMMENDMENT TO THIS REPORT Surgical Pathology Report

The findings are non-specific and do not follow a recognizable pattern of immunopathological disease. Dermatopathology Report *Amended*

Translation: "Yeah, it's leukocytoclastic vasculitis, but we don't have a clue what's causing it." The rheumatologist gave the analogy of being set down from a balloon in the middle of a cornfield. You know you are in a cornfield, but you don't have a clue what country you are in.

What does this mean? Well, it means we have a definite diagnosis on LV, but we do not know the initial trigger. In approximately 50% of the cases, they never find out a trigger, so I don't have my hopes up. Through blood and urine tests, we have largely ruled out systemic (internal organ) involvement, so that is a Good Thing^TM.

The vasculitides are conditions of unknown aetiology. Until recently, relatively little was known about their incidence and prevalence. What is known about the epidemiology of the vasculitides?, by Watts, et al

Epidemiology

The occurrence of leukocytoclastic vasculitis in adults is rare. Studies in the UK, Spain, etc. have shown rates ranging from 10-40 adults per million per year. Anecdotally my dermatologist sees about ten cases per year (including children) and my rheumatologist has seen about 10 cases in his entire career. While a the surface these seem contradictory, it's not. The dermatologist's numbers include children where it is known as Henoch-Schönlein purpura. Dermatologists are usually the first line of defense and the condition is often only referred to a rheumatologist when it becomes apparent that it is likely chronic. My understanding is that, in children, the condition usually goes away quickly and is rarely chronic.

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• Leukocytoclastic Vasculitis Images
• Images of my progress with leukocytoclastic vasculitis
• 

  2013-03-17
  Left leg the day before my birthday. I didn't photograph my other leg at the time because I was just trying to show mom the LV itself, not the secondary infection that had set in on the right leg. Also, I was in intense pain, so I wasn't exactly thinking about chronicling.

• 

  2013-04-19
  Both legs a month later. A second outbreak had occurred on the backs of my legs, but by this time was finally clearing up and we were hoping things were over. A new outbreak on my feet and ankles would start 2 days later. This round also started showing ulcerations on the thigh and ankle.

• 

  2013-04-22
  The beginning of the third round of LV outbreaks. This was the one that prompted my dermatologist to turn me over to a rheumatologist, since it was starting to look like a chronic condition.

• 

  2013-05-24
  The beginning of the fourth round of LV outbreaks. You can see how large the third round ankle spot got.

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  2013-06-20
  Fourth round of LV outbreaks is finally healing after treatment with oral steroids. You can see the swelling has gone down considerably between the previous picture and this one.