Please keep in mind in reading this the fact that I have been told by multiple physicians the severity of my case is unlike any PLEVA case they have ever seen.
I had my first breakout at the age of 19.
It happened right after my vacation to northern Wisconsin for a fishing trip. On the morning of August 1st, 2009 when I got out of the shower I noticed I had 2 or 3 small red spots on my waistline. I thought they were small pimples and I wasn’t washing good enough. I mean, it happens. So I blew it off and went about my day. The next morning; same scenario. This time, I noticed about 15-20 of these same spots between my belly button and just down my right thigh. I decided to ask my mom if she changed laundry detergents, but of course it turns out she didn’t. This time I told myself it was probably a small rash of some sort. No big deal right? So I blew it off and went about my day.
Somewhere within the next few nights, I woke up and I was breaking out in these little red spots. Only this time it was between my knees and shoulders, front side and back. I did notice they had a small itch to them, so I thought to myself, ...chicken pox? I did have a pretty bad case when I was 7 years old, but heard it was possible to get them again. Just to be sure my mom and I went to the immediate care center for a professional’s opinion.
[DOCTOR VISIT #1]
To make a short visit even shorter, I had two physicians tell me I had an “Atypical presentation” of chicken pox. They were convinced because of the different stages of the lesions and its symmetrical presentation. So, they sent me on my way. When I got home I started the typical remedies for chicken pox, such as applying calamine lotion and taking oatmeal baths. I also found it very odd that I never had any flu-like symptoms.
2 weeks later, I am still breaking out in these spots. At this point I was able to identify many different looks to the lesions. The spots were any combination of the following:
-Small red spots. -Large red spots. -Raised red bumps. -Raised pimple like appearance filled with a dark gray liquid. -Raised donut-shape [with a sunken center] -Raised/Red with surrounding purple skin. -A brown/yellow scab. -Black depressed scabs up to 15mm.
I knew I didn't have chickenpox. It was time for a second opinion. Keep in mind at this point I am not on any medication that is suspected to ease pain for this disease.
[DOCTOR VISIT #2]
On August 25th I visited an infectious disease doctor. He had asked me the routine doctor office questions, and seemed to be on to something in suggesting I may have had vasculitis. I was prescribed a Medrol dose pack, sent on my way and scheduled a follow up. Another 2 weeks went by; another 2 weeks of developing new lesions.
[DOCTOR VISIT #3]
On september 8th, I had my first visit with a dermatologist during any of this due to the difficult task of getting appointments quickly with them. Here are a couple quotes from my report of the visit...
“He has a total body rash consisting of violaceous papules. Some of which have an eschar and some of which have pustules including one on the left calf and left hand. No papules on the hands, groin, or face. He has no papules on the palms and a few on the dorsum of his hands.”
“Purpuric pustular dermatitis of unclear etiology. Discussed possibility of PLEVA [pytriasis lichenoides et varioliformis acuta], LyP [lymphomatoid papulosis], pustular drug eruption, psoriasis, Sweet’s disease, vasculitis, etc. Could also be a very atypical form of pytriasis rosea. Awaiting biopsy results which may or may not help with diagnosis. Because vasculitis in adolescents is most often related to infection, ordered tests for hepatitis and ASO. Ordered ANCA looking for more unusual forms of vasculitis like cutaneous Wegener’s. Sweet’s is a neutrophilic dermatosis and can be associated with hepatitis and blood dyscrasia. Rheumatoid arthritis also can be seen in association with a neutrophilic dermatosis. Ordered CBC, CMP, sed rate, ANCA, Hep B, Hep C antibodies, rheumatoid factor. Samples given of Clobex foam and spray given. if culture negative and medrol helping pt may benefit from Prednisone with higher dose and longer course.
Also I should note that I did not develop any lesions above my jaw bone. I had about 20 on the whole circumference of my neck, but none on my face. I did not develop any on the palms of my hands or the bottoms of my feet.
On this September 8th visit, we took a biopsy of one of the lesions on my left forearm midway between my wrist and elbow. We also took 3 cultures of the pustules on the back side of my left leg, and plenty of blood for testing.
All of my blood tests; normal. The cultures; no bacteria. The biopsy; inconclusive. At this point I was ready to kill someone.
I went on taking all of the suggested medicines, and missing class because I couldn’t walk. I had to call off work a bunch of times because I was in so much pain. PLEVA burns, itches, and bleeds. I remember one night getting out of the shower specifically. Once the water stopped hitting the lesions, they flared up... really bad. It burned ssoooo bad. My brain brought my hand to my right hip and just started clawing at my skin. I ripped a couple open, making them bleed. I couldn’t control it. It was so instinctive, needing to remove the sensation from my body.
Just as any human being would do when desperately seeking answers, I turned to the internet. At first, I thought I had self diagnosed myself with Celiac’s Disease. The pictures I saw online of it’s dermatitis looked similar to my presentation. Also, the night they flared up really bad, I had a Subway footlong on wheat, and wheat spaghetti for dinner. But, being 19 years old, I didn't think I would have just developed a wheat allergy over night. I stopped researching online because everything I read scared me. It wasn't worth the worrying for not even knowing what I had.
[DOCTOR VISIT #4]
I ended up seeking out the help of another dermatologist. Upon initial inspection, she was not too certain on a diagnosis and recommended multiple biopsies. We had brought all of the paperwork from visits prior, and PLEVA was definitely being considered. Next thing I knew, I was in a hospital gown, with five of the clinic’s staff members around me going “Oh my God you poor thing.” One of the PAs had a camera and was snapping pictures of me. I’m not going to lie, I started crying a little bit. I had always been a healthy kid, and here I am... under the lights, and desperate for answers. We took 2 punch biopsies from just above my left knee of relatively fresh lesions.
Here is the important information from my biopsy report...
September 9th, 2009
DIAGNOSIS: Pityriasis Lichenoides Et Varioliformis Acuta
NOTE: The lesion is crusted. Immunofluorescence studies performed separately were negative.
GROSS DESCRIPTION: 5.0 x 5.0 x 7.0 mm
MICROSCOPIC DESCRIPTION: Spongiosis, focal necrosis, a scale crust, wedge-shaped perivascular inflammation, purpura and scattered neutrophils are present in a pattern most suggestive of pytriasis lichenoides et varioliformis acuta [Mucha Haberman]
After this, I started on Erythromycin, topical Clobetasol Propionate steroid cream, and experimenting with Domeboro bath soaks and Aveeno oatmeal baths. It only took about 3 days for the Erythromycin to hurt my stomach. It wasn't so much stomach pain or cramps, but more of an aching diaphragm. I did not notice any benefit to using the two different bath remedies. I would advise to not take baths altogether. We also decided to try Ultraviolet Phototherapy.
[DOCTOR VISIT #5]
The main purpose of visiting this doctor was because my dermatologist’s office did not have phototherapy booths. I went for 8 or 9 phototherapy sessions and didn't notice any change. The sessions were never longer than a minute and 10 seconds. They start with small amounts of time and increase it as you go to see how your body reacts. I did not continue light treatments due to my school schedule and the inconvenience of travel time. Under this doctor, I was kept on Minocycline 100mg, and given Biafine cream for open sores. At this point, I started losing scabs and some wounds were left exposed.
I think I started to feel comfortable [physically] around mid November. I felt like going out with my friends and exercising. The picture shown below is how recovery looks when the pain starts to become minimal. There is really nothing to be alarmed with at this point. It's just a waiting game.
From the beginning of my story to HERE was a course of 3 months.
Between mid November 09' and January 10' there was no activity. I was only taking the Minocycline, and went back to living a normal life for the most part. In this time I did not have any new lesions. I decided to start posting updates which you may find useful.