Unusual and painful skin lesions, doctors do not know the cause. Can anyone help?

[Pentar]

I know that this is a very long post, but I am hoping to add enough information about what is going on for those that may reply. Please, if anyone has seen anything like this or has any information that may help give me some direction in figuring out the cause of these lesions, I will be forever grateful.

I have been developing unusual and very painful skin lesions for about 4 1/2 - 5 years now. I will include other symptoms, test results, as well as what doctors have considered and ruled out. I have included links to photos of the lesions; they resemble "sunken holes" or "craters" on the scalp. The process as the lesions form as as follows:

1. Normal scalp appearance.
2. The area starts to feel hot to the touch.
3. Pain starts (sharp tearing, ripping mostly although had one that felt like the nerve was on fire).
4. The area make become slightly red.
5. The area becomes squishy or spongy underneath the skin.
6. The lesion starts to sink, looking like a crater (still remains remains soft).
7. The time for a lesion to form can take anywhere from 6 hours to 4 days.
8. The size of the lesions varies greatly (from 1 - 6 cm in diameter and .5 - 1 cm deep.
9. NEVER do the lesions open, rupture, or have any fluid.

The lesions constantly ache with occasional sharp pain. As a new lesion forms it feels as if the skin is tearing and ripping, is hot to the touch, and the pain is almost unbearable. The last one to form the pain was different, feeling as the nerve was on fire (or like someone was poking a hot poker in it) and also felt as a piece of glass was moving through it. The following links as pictures of the lesions:

http://s822.beta.photobucket.com/user/Pentar/media/Hole1.jpg.html?sort=3&o=8
http://s822.beta.photobucket.com/user/Pentar/media/100_0071.jpg.html?sort=3&o=3
http://s822.beta.photobucket.com/user/Pentar/media/100_0052.jpg.html?sort=3&o=6

Besides the skin lesions on my scalp, I have had small blister like lesions (about 4-5 mm in size) on my legs and lower abdomen twice (once about 6 months ago, then again about 5 months ago). These small blisters itched badly and a few were painful (sharp stinging pain when slightly touched). The blisters had a clear fluid inside is ruptured. These did clear up after about 2 weeks leaving behind brown spots.

Other symptoms include persistent nausea, frequent diarrhea, feeling "cold" very often, and insomnia. Not certain if relevant, I did have a stroke about 14 years ago (not certain of the type). The stroke was in the left frontal lobe just in front of the left ventricle. A small porencephalic cyst (1.6 cm) is now in that location. I now have encephalomalacia on the left side as well.

They have ran a lot of tests trying to find the cause. Below is a list (and I may have forgot some tests):

1. Upper and lower GI were normal.
2. Abdominal ultrasound was normal, although I was told that my liver was "fuzzy".
3. Two sets of skull x-rays were normal.
4. CT scan and MRI were abnormal (showing the cyst and encephalomalacia).
5. Full body bone scan normal.
6. Two separate ANA tests were both negative.
7. SPEP and UPEP were both negative.
8. MS battery normal.
9. Blood tests for vasculitis and related disorders were negative.
10. Hormone tests were normal, although a glucagon test has not been done.
11. Zinc levels were normal.
12. Platelet count on multiple tests were in the low range of normal (161-170).
13. MCH high in the normal range - 33.1 (27-34) and 32.6 (27-33).
14. Last urinalysis did show trace amounts of blood, otherwise normal.
15. Two skin biopsies were both abnormal (See below).

I have had two skin biopsies done on to different lesions. The first was a punch biopsy that showed necrolytic dermatitis, and the second was an incisional biopsy that showed the fat tissue was degenerating. The first biopsy slide was sent to the National Cancer Institute (NCI). They said that biopsy had histological features that are suggestive of necrolytic migratory erythema.

I have seen multiple specialists and thus far no one knows what is going on. One doctor ruled it as lupus (that diagnosis has since been removed). The NCI suggested 3 possibilities - glucagonoma, acrodermatitis enteropathica, or zinc deficiency. Zince levels were normal, and I have yet to have a glucagon hormone test and / or abdominal CT scan to rule out glucagonoma. I have recently been to UCLA Medical Center and they have suggested panniculitis or (because of the stroke) Degos Disease, although the skin lesions do not resemble either. They are reviewing the two biopsy slides together (and the biopsy is the test for Degos Disease).

The symptoms are becoming increasing worse. If anyone has seen this before, or has some possible ideas of the cause, please help solve this medical mystery. The pain has worsened and being undiagnosed for so long is very hard. I thank everyone in advance for their replies.

Additional Details
I am a 44 year old male. I live in the United States and have not traveled to any tropical regions or even out of the country. I was born in England and the only notable medical history from there was that I was given the live TB vaccine (as other born there during that time). I did have an unknown illness at the age of 6 weeks old. The illness was SIMILAR to cerebral meningitis. I was told recently that there was something wrong with my red and white blood cells (parents and grandparents do not remember much about it), and I was given immunostimulants (some were experimental) - although no one knows what I was given.

One little bit on information that I forgot which may or may not bear any meaning is my fingernails. They curl down along my fingertips.

[Pentar]

Thank you for your comment. I essentially dismissed the idea of panniculitis after looking at photos of the disease. You're comment has made me question my decision. Also, the staff at the UCLA Medical Center did mention panniculitis, and that is something they are currently trying to determine, along with the suggestion of Degos Disease.

The idea of panniculitis really baffled me initially, as the lesions looked completely different. I know that the doctors are considering extremely rare causes, and you are the first to mention Atypical Mycobacteria. You have given me insight as to why the doctors mentioned panniculitis, for that I thank you.

I am hoping to hear back from the UCLA Medical Center within 2 weeks. Although I can not be certain of the cause until the doctors do the tests, the possibility of Degos Disease was unsettling to say the least. You have given me some piece of mind during that time, and as I mentioned, you have informed me of the reason the doctors are considering panniculitis. Again, I thank you so very much.

[Pentar]

Thank you for your comment. I have only briefly researched Osteogenesis; the defining symptoms of it do not appear similar. My bones appear very strong, and the calcium uptake from the bone scan was also normal. Moreover, something I did not mention prior, is that although I have has some broken bones, they have healed perfect, without any calcium scarring.

I am currently awaiting a response from the UCLA Medical Center (within 2 weeks), but I do expect their review of the biopsies to show that I do not have Degos Disease. Therefore I am hoping to find a direction that the doctors may continue that will lead to a diagnosis and a course of treatment.

[Life360_Dave]

Hi and sorry your going through this. Have you researched Osteogenesis? The babys head has this which allows the skull to grow but this disease can take place which is a bone disorder and some feel it is genetic.