[CIS-PAGID] Lichen Planus in Good's syndrome

[Seppänen Mikko]

Dear Kenneth and Tony,

many thanks! As a summary, acitretin and thalidomide against severe Good's sdr associated mucosal lichen has worked in single cases.

For our pt's mucosal lichen we have attempted:
topical triamcinolone (best thus far) + Mycostatin (and at times other topical antimicrobial meds likse Corsodyl, Kenacort-T and symptomatic topical Xylocain)

no / rapidly waning response:
systemic steroid (partial response, waned when dose below 15mg/d, unacceptable side effects), thalidomide (mild positive effect, severe fatigue as side effect, stopped), topical tacrolimus (partial response, waned), pentoxyphyllin, dapsone, doxycycline,
He has secondary dry mouth caused by lichen and chronic periodontitis that is very treatment resistant as well.

For his trunk pityriasis lichenoides summer helped and has not been a problem lately, nails affected as well.

Summary: we will most likely try acitretin next.

Many thanks again, I sure hope this works....

mikko

________________________________
Lähettäjä: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] Puolesta Infante, Anthony J
Lähetetty: 8. maaliskuuta 2012 16:49
Vastaanottaja: pagid at list.clinimmsoc.org
Aihe: Re: [CIS-PAGID] Lichen Planus RE: Good's syndrome

I have a young lady with hypogamma (not Good syndrome) and severe lichen planus who has responded very well to thalidomide as a last ditch treatment. She has primary amenorrhea and is on BCPs.

Tony Infante
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Paris, Kenneth
Sent: Thursday, March 08, 2012 7:55 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

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