[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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