[CIS-PAGID] Lichen Planus RE: Good's syndrome

[Nelson, Robert P Jr]

Jack,
Yes, I'm worried too.  Organ function OK but has some retroperitoneal nodes--will biopsy and share the results--thanks much.  Bob

Robert P. Nelson Jr., MD
Professor of Medicine and Pediatrics
Divisions of Hematology/Oncology
535 Barnhill Dr. Ste 473
Indianapolis, IN  46202
Telephone: 317-948-1186
E-mail: ronelson at iupui.edu
pager:  317-312-1773

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Routes, John
Sent: Thursday, March 08, 2012 4:45 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Lichen Planus RE: Good's syndrome

Bob
I am worried that this may be a more systemic problem-----were granuloma described? Does the patient have lung or liver disease? Would be surprised if pentosan worked for this given the described infiltrate and may need some form of immunosuppression----low dose cyclosporine has been used in IC in patients with rheum disease.
Jack
 
John M. Routes, MD
Chief, Section of Allergy and Clinical Immunology Co-Director, Clinical and Translational Science Institute of Southeast WI Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics Department of Pediatrics Children's Hospital of Wisconsin Medical College of Wisconsin
9000 W. Wisconsin Ave.
Milwaukee, WI  53226-4874
 
Phone: 414-456-4802; 414-266-6997
Fax: 414-456-6487 (Clinical)
Fax: 414-456-6323 (Laboratory)
Email: jroutes at mcw.edu
 




> From: "Nelson, Robert P Jr" <ronelson at iupui.edu>

> Reply-To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>

> Date: Thu, 8 Mar 2012 11:33:18 -0600

> To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>

> Subject: Re: [CIS-PAGID] Lichen Planus RE:  Good's syndrome

> 

> Overactive bladder function, frequency, urgency.  Urology currently 

> trying pentosan polysulfate.  Bob

> 

> Robert P. Nelson Jr., MD

> Professor of Medicine and Pediatrics

> Divisions of Hematology/Oncology

> 535 Barnhill Dr. Ste 473

> Indianapolis, IN  46202

> Telephone: 317-948-1186

> E-mail: ronelson at iupui.edu

> pager:  317-312-1773

> 

> -----Original Message-----

> From: pagid-bounces at list.clinimmsoc.org 

> [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Routes, John

> Sent: Thursday, March 08, 2012 11:40 AM

> To: pagid at list.clinimmsoc.org

> Subject: Re: [CIS-PAGID] Lichen Planus RE: Good's syndrome

> 

> Bob

> this doesn't look like the typical biopsy with interstitial 

> cystitis---what are the symptoms?

> Jack

> 

> John M. Routes, MD

> Chief, Section of Allergy and Clinical Immunology Co-Director, 

> Clinical and Translational Science Institute of Southeast WI Professor 

> of Pediatrics, Medicine, Microbiology and Molecular Genetics 

> Department of Pediatrics Children's Hospital of Wisconsin Medical 

> College of Wisconsin

> 9000 W. Wisconsin Ave.

> Milwaukee, WI  53226-4874

> 

> Phone: 414-456-4802; 414-266-6997

> Fax: 414-456-6487 (Clinical)

> Fax: 414-456-6323 (Laboratory)

> Email: jroutes at mcw.edu

> 

> 

> 

> 

> ________________________________

> From: "Nelson, Robert P Jr" <ronelson at iupui.edu>

> Reply-To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>

> Date: Thu, 8 Mar 2012 08:29:08 -0600

> To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>

> Subject: Re: [CIS-PAGID] Lichen Planus RE:  Good's syndrome

> 

> I have a male patients with CVID and interstitial cystitis.  Biopsy 

> reveals intense chronic cystitis (lymphoid follicular formation, 

> histiocytes, eosinophils), no cancer.  Any thoughts regarding your 

> collective experience and management consideration would be appreciated.

> Thanks.

> Bob

> 

> 

> Robert P. Nelson Jr., MD

> Professor of Medicine and Pediatrics

> Divisions of Hematology/Oncology

> 535 Barnhill Dr. Ste 473

> Indianapolis, IN  46202

> Telephone: 317-948-1186

> E-mail: ronelson at iupui.edu <mailto:ronelson at iupui.edu>

> pager:  317-312-1773

> 

> 

> From: pagid-bounces at list.clinimmsoc.org 

> [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Paris, Kenneth

> Sent: Thursday, March 08, 2012 8:55 AM

> To: pagid at list.clinimmsoc.org

> Subject: [CIS-PAGID] Lichen Planus RE: Good's syndrome

> 

> 

> Mikko,

> 

> 

> 

> At the ACAAI meeting in 2011 we reported a patient with classic Good 

> Syndrome who developed debilitating oral, vaginal (and eventually 

> cutaneous) lichen planus.  She had complained of oral "ulcers" which caused extreme discomfort.

> She had significant weight loss (>30lbs).  Topical medications  were 

> not helpful, but systemic steroids caused improvement.  This raised 

> the suspicion of other dermatologic disorders including LP.  Diagnosis 

> of LP was confirmed with biopsy.

> 

> 

> 

> Since we did not consider oral steroids a longterm treatment, we 

> consulted a medical dermatologist to help with decisions regarding 

> immunomodulators.  Our patient has now been on Soriatane (Acitretin) 

> for 6 months with excellent results (near complete remission of the 

> oral and vaginal lesions).  She is not of childbearing age.  She uses 

> a tetracaine lollipop for some burning sensation she continues to have 

> at times, but the lesions are gone.  She has regained her weight.

> 

> 

> 

> Hope the suggestion helps for your patient.

> 

> 

> 

> Ken

> 

> 

> 

> Kenneth Paris MD, MPH

> 

> Assistant Professor of Pediatrics

> 

> A/I Fellowship Training Program Director

> 

> Division of Allergy and Immunology

> 

> LSU Health Sciences Center

> 

> Children's Hospital of New Orleans

> 

> 

> 

> Mail:

> 

> 200 Henry Clay Avenue

> 

> Children's Hospital

> 

> Research Institute for Children 4th Floor

> 

> New Orleans, LA 70118

> 

> Phone:  504-896-9589

> 

> Fax:  504-896-9311

> 

> Email: kparis at lsuhsc.edu <mailto:kparis at lsuhsc.edu>

> 

> 

> 

> The information contained in this e-mail is privileged and 

> confidential and is intended only for the use of the addressee(s) 

> indicated above. Use or disclosure of information e-mailed in error is 

> respectfully prohibited. If you have received this e-mail in error, 

> please contact the sender and immediately delete the original message or you may call 504-896-9589. Thank you.

> 

> 

> ________________________________

> 

> From: pagid-bounces at list.clinimmsoc.org on behalf of Seppänen Mikko

> Sent: Thu 3/8/2012 6:33 AM

> To: 'pagid at list.clinimmsoc.org'

> Subject: Re: [CIS-PAGID] Good's syndrome

> 

> I have 3 in follow up, none of them has T-lymphopenias nor reduced 

> mitogen responses (thus a subgroup, not representative of the whole 

> diseases), and their IgG troughs are kept above 10 g/l (11-13), and 

> none of them needs any prophylaxis. One had to go through FESS first, 

> but after that has been doing fine.

> 

> However, one of them has severe oral mucosal lichen, causing scarring 

> and a lot of discomfort and I + dentists have been completely helpless with that.

> 

> mikko

> 

> __________________________________________________

> Mikko Seppänen, MD, PhD

> Specialist in Internal Medicine and Infectious Diseases Senior 

> Consultant, Physician in charge (PIDD) EM(E)A Expert, PIDDs and 

> Intravenous Immunoglobulin Therapy

> 

> Immunodeficiency Unit

> Division of Infectious Diseases

> Department of Medicine

> Helsinki University Central Hospital

> Hospital District of Helsinki and Uusimaa Aurora Hospital, Ward 4-2 

> and Outpatient Clinic P.O.Box 348

> FI-00029 HUS, Helsinki

> FINLAND

> phone +358 9 47175923, fax +358 9 47175945 

> _________________________________________

> 

> 

> Lähettäjä: pagid-bounces at list.clinimmsoc.org 

> [mailto:pagid-bounces at list.clinimmsoc.org] Puolesta Terri Tarrant

> Lähetetty: 8. maaliskuuta 2012 13:59

> Vastaanottaja: pagid at list.clinimmsoc.org

> Aihe: [CIS-PAGID] Good's syndrome

> 

> 

> I have a patient with newly diagnosed Good's syndrome with resected 

> thymoma, severe hypogammaglobulinemia, absent CD19+ B cells, and 

> recurrent sinopulmonary infections over the last 5-7 years.  There has 

> been no documentation of severe viral, fungal, or opportunistic 

> infections, and I have just started the patient on IgG replacement.  I 

> wanted to query the group if you use prophylaxis in these patients, and if so, with what.

> 

> 

> 

> Many thanks for your thoughts,

> 

> Terri

> 

> --

> 

> Terri Tarrant, MD

> 

> Assistant Professor of Medicine

> 

> Thurston Arthritis Research Center

> 

> Lineberger Cancer Center Member

> 

> CB # 7280, 3300 Manning Dr.

> 

> Chapel Hill, NC 27599

> 

> (919) 843-4727

> 

> http://tarc.med.unc.edu/tarrant_welcome.php

> 

>