[CIS PIDD] [cis-pidd] Good Syndrome

Paris, Kenneth kparis at lsuhsc.edu
Wed Aug 28 18:14:50 EDT 2013


Joe,



We follow a similar patient, and had a poster at one of the annual meetings a few years back (it's been reported previously). She was diagnosed with LP via oral mucosal biopsy (she had cutaneous, oral and vaginal lesions) and is currently managed with Soriatane (oral retinoid). Her biggest problem with the LP was the debilitating pain from her oral lesions. This caused significant weight loss due to loss of appetite. Since starting Soriatane, things are significantly better but not completely resolved. Her oral lesions are improved, but are persistent. Not sure if your patient has oral LP, or if their weight loss is due to decreased intake like we saw in our patient. I prescribe tetracaine lollipops for her oral pain/burning, and she claims they help. Her cutaneous symptoms are completely resolved.



My other Good Syndrome patient was diagnosed here at our center due to persistent esophageal candidiasis - that was her only presenting symptom. Post thymectomy, her candidiasis improved significantly.



Maybe some other people have similar experiences with their patients. We enlisted the help of an excellent medical dermatologist for management of the LP, but continue to take care of the immunologic deficits ourselves.



Best regards,



Ken





Kenneth Paris MD MPH

Associate Professor of Pediatrics

Program Director, A/I Fellowship Program

Co-Director, JM Center for Primary Immunodeficiencies

LSU Health Sciences Center and Children's Hospital



Phone: 504-896-9589

Fax: 504-896-9311



Mail:

Children's Hospital New Orleans

Division of Allergy and Immunology

200 Henry Clay Avenue

New Orleans, LA 70118



________________________________
From: Church, Joseph [JChurch at chla.usc.edu]
Sent: Wednesday, August 28, 2013 4:45 PM
To: CIS-PIDD
Subject: [cis-pidd] Good Syndrome

Colleagues:

Today I was asked about a 55yo man with Good syndrome, complicated by lichen planus, alopecia, thrush and progressive weight loss.

Any thoughts as to who I might recommend as a second opinion?

Joe Church
Children’s Hospital Los Angeles



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