[CIS PIDD] Progressive Fibrosis

[Sullivan, Kathleen]

Could this be one of the IgG4 mediated diseases?
On Sep 19, 2012, at 10:05 AM, Richard Wasserman wrote:


> 

> I was contacted by a 69 y/o physician who presented with gastroenteritis five years ago but was thought to have cholecystitis. At lap cholecystectomy he was found to have multiple fibrous adhesions from ascending colon to abominal wall. There were two subsequent releases of adhesions with Path diagnosis of fibrous tissue w/o neoplasm or serositis. Two years later there was an open right colectomy for colonic inertia with a diagnosis of fibrosed appendix.

> 

>  

> Post cholecystectomy he developed bilateral forearm lichen planus.

> 

>  

> Extensive evaluation was unrevealing except for SS-A >8 and a minor salivary biopsy that showed grade Grade III siladenitis. MGUS showed a small 0.24 M-spike & kappa light chains that normalized with prednisone or methotrexate used to treat fibrosis.

> 

>  

> CYP2D6 deficiency.

> 

>  

> His major ongoing problems:  Debilitating abdominal pain worsened by movement. 2 1/2-yr colectomy scar swelling and severe "neuropathic-type" pains with CT & US evidence of intra-abdominal fibrous adhesions to liver w/ irregular liver edge suggesting ongoing intraperitoneal fibrosis. He has been treated with plaquenil, colchicine, methotrexate and prednisone without benefit. He has experienced improvement after treatment with doxycycline.

> 

>  

> PMH: life-long protracted healing requiring months to resolve simple local tissue injury such as insect bites and minor cuts. Sites of more significant tissue injury (operative scars) experience periodic (pain and inflammation every three to five weeks).

> 

>  

> Any thoughts?

> 

>  

> He has had extensive evaluations by specialists both in Dallas and at two major medical centers out of town. If anyone is interested in seeing this gentleman, he is willing to travel. He asked that I help identify a physician who would be willing to evaluate him.

> 

>    

> Thank you,

> 

> Richard Wasserman,

> 

> Dallas

> 

> 

> -- 

> Richard L. Wasserman, MD, PhD

> DallasAllergyImmunology

> 7777 Forest Lane, Suite B-332

> Dallas, Texas 75230

> Office (972) 566-7788

> Fax (972) 566-8837

> Cell (214) 697-7211

> The CIS-PIDD listserv is supported by:

> Clinical Immunology Society - The science & practice of human immunology

> 

> P: +1.414.224.8095

> E: info at clinimmsoc.org

> 

> Not a member of CIS? Please visit www.clinimmsoc.org to join!


Kate Sullivan, MD PhD
Professor of Pediatrics
ARC 1216 Immunology CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363


-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/pipermail/pagid/attachments/20120919/86b21c92/attachment.htm>