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

[Routes, John]

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