Linear Behcet's Disease skin ulcerations

[behcets]

I've had Behcet's Disease for many years, seemingly with the arthritis-acne-skin lesion cluster along with the major/minor diagnostic criteria.  HLA-B51 and pathergy tests are negative, but that's not uncommon for Americans of Northern European heritage.  While I get many individual or scattered skin ulcerations, sometimes they appear in linear or near-linear configurations. Over the past couple years, I've been photographing my skin ulcerations, which have responded to prednisone, pentoxifylline, Enbrel, nicotine patches, methotrexate, and dapsone.  Some doctors, including dermatologists, have questioned whether Behcet's can cause linear skin lesions and are accusing me of causing these by scratching, which is definitely NOT the case.  My skin will get a prodromal burning and/or itching sensation, swell a bit, redden, and -- without me touching the area-- break out in small ulcers which heal in 5-10 days depending on size and whether some fuse together.  Some rheumatologists have suggested the ulcers might be following blood vessels beneath the skin, but there have been no reports of linear skin ulcerations in Behcet's.  No biopsies have been taken, probably because they suspect neurotic excoriations.  The linear skin ulcers are identical to the scattered or singular ones, so why are some of them in this pattern?  The linear ulcers are commonly found on my extremities, but occasionally on my trunk.  I've also had erythema nodosum and painful skin pustules, plus ulcerations on my scalp.   I cannot find any photos online of similar skin ulcerations.

[maatson]

Good to hear that there is a consensus here.Speedy recovery and my best wishes.

[behcets]

After more research and consultation, my dermatologists have now agreed that my linear skin ulcers AND my acne are both caused by my Behcet's Disease, not anything I'm doing to myself.  They apologized for their earlier doubts and want to continue with me as their patient.  I appreciate other dermatologists who gave me their opinions and advice.  

[maatson]

Very interesting.I do believe that the process is the same on the linear lesions as elsewhere.
Only a biopsy would help in establishing a certain diagnosis.I do understand the difficulty, since the vesicles are very evanescent.
Best wishes.

[behcets]

I just can't find any description or photos online that resemble my linear lesions, which heal and leave hypopigmented scars.  They never blister, they just open up as shallow ulcers (difficult to photograph) and then begin to heal with a thin border scab (easier to photograph).  The majority of my skin ulcers appear singularly or in scattered crops; only a few show a linear pattern.

Everything I've read about the Koebner phenomenon and IgA linear bullous dermatosis, and online photos of such, does not seem to fit what I've experienced.  Rather, my linear ulcers sometimes appear on top of visible blood vessels in areas of thin skin such as ankles and hands, but have also occured on fatty tissue such as upper thighs.  A few bleed slightly as the ulcers break open, reinforcing my belief that these linear and singular skin ulcers are due to vascultiis.  Again, all of this happens without me touching, rubbing, or scratching the skin.

In addition, as I've mentioned, I sometimes get white headed pustules on my extremities as well as painful cystic-type red bumps and scalp ulcers.  Since all of these lesions respond to the same systemic treatments used for oral Behcet's ulcers, I believe they're all part of the same picture.  

Most interesting is that when I tried wearing nicotine patches (I'm a nonsmoker) to control my oral Behcet's ulcers, I also experienced a remission of my skin ulcers.  Stopping the patches resulted in the return of both oral and skin ulcerations.  I developed an allergy to the patches or I would still be using them to keep my mouth and skin clear of lesions.

[maatson]

Skin trauma is not necessarily through injury of known origin, even minor scratching can lead to koebnerization. And pathergy test per-se would not be an index here.
Best wishes.

[behcets]

I have posted photos on my profile page.  

Koebner phenomenon requires a trauma of some sort, and my skin ulcers are spontaneous without any known trauma or scratch.  Koebner is also unlikely because my pathergy tests are negative.  

[maatson]

Oh,Forgot to add the most important here. Koebner phenomenon could explain this.
Is Behçet’s Disease a Kobner Positive Disorder?
Meltem Önder, Murat Oztas and Arzu Aral
Advances in Experimental Medicine and Biology, 2004, Volume 528, Part 8, 333-335, DOI: 10.1007/0-306-48382-3_67

[maatson]

Guess you will have to get it biopsied and especially to rule out  IgA linear bullous dermatosis.