[CIS PIDD] [cis-pidd] Partial Jacobsen syndrome with HPV-related AINIII and squamous carcinoma- advice on HPV treatment
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Wed Mar 30 23:45:29 EDT 2016
Please Kate Sullivan
Can I enquire, we have access to 1% cidofovir in aqueous cream. Apparently it only lasts 7 days in this form.
would this be in the form you used for your patient or alternative.
Can we expect adverse reactions as she is due to have skin and vulvectomy and removal of SCC anus so we don't want to risk an adverse skin reaction.
Many thanks for your response
Dr Karen Lindsay
Rheumatologist and Immunology Fellow
MBChB BSc MRCP (UK)
From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Thursday, 17 March 2016 1:54 p.m.
To: CIS-PIDD
Subject: Re: [cis-pidd] Partial Jacobsen syndrome with HPV-related AINIII and squamous carcinoma- advice on HPV treatment
We really like topical cidofovir for recalcitrant warts.
Kate Sullivan
Sent from my iPhone
On Mar 16, 2016, at 8:22 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:
Subject: Partial Jacobsen syndrome
Dear All,
I would appreciate any treatment suggestions.
I have a 29 yr old female patient with karyotype confirmed distal chromosomal deletion of no 11. She recently presented to our service with severe anal and genitial warts, with malignant transformation into carcinoma.
She also has eczema like skin lesions.
She has facial appearances that look like Jacobsen syndrome, but with hirsutism.
Previous condylomata treatment includes iquinimod has failed. She has had guardasil vaccinations
Would there be any advice you could offer as to how to manage her condylomata?
Here is her problem list and summary of results.
1. Recurrent severe genitourinary and rectal HPV infection
- Recurrent vaginal and anal condylomata onset age 25 with secondary VIN, CINIII, AINIII with focal invasive squamous cell carcinoma ( and previously resected carcinoma )
-
2. Ch 11 deletion
- Delayed early motor development Possibly delayed primary teeth loss ( age 9 one had to be removed by dentist )
- Early menarche aged seven,
- Acromegalic, hirsute, greasy skin. Testosterone, IGF-1 normal
- No Family History of PID
3. Rash with secondary Staph aureus infection Biopsy reported as chronic spongiotic dermatitis ( this is more eczema like than psoriatic) Now resolved.
Current Medication
Depoprovera
Further Negative or Normal Investigations
Platelets normal morphology
WCC normal and lymphocyte subsets all normal
HIV test negative on two occasions, syphilis serology normal
Immunoglobulins IgG 6.7 (normal pre-op and for many years)
IGF-1 normal
Prolactin normal
TFT normal
HbA1c normal
Testosterone normal
Many thanks in advance for your advice
Dr Karen Lindsay
Rheumatologist and Immunology Fellow
MBChB BSc MRCP (UK)
Immunology dept
Auckland City Hospital
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