[CIS PIDD] [cis-pidd] CVID with hidradenitis suppurativa+ acne conglobata and recurrent boils

[Yeşim Yılmaz Demirdağ]

Hi Dr. Seppanen,
I had a similar patient, a 46-yr-old morbidly obese man with CVID. He was
initially on IM then IVIG since age 16 yrs of age. His weight ranged
between 130 and 150 kgs. He had recurrent pneumonia due to low trough IgG
levels ( 200s) , he also had recurrent skin abscesses in the groin area and
legs, sometimes below the breasts. They were all MRSA +. I increased IVIG
to 60gr q2 wks to have a trough level > 800 mg/dl, 6-wk course of Doxy, and
I also recommended twice weekly bleach baths which we were recommending to
our MRSA + atopic dermatitis patients at that time. I am not sure what
worked, but after a couple of weeks he never had abscesses anymore.
Best,
Yeshim

Yesim Yilmaz Demirdag, MD
Columbia University Medical Center
3959 Broadway Rm 107N
New York, NY 10032
phone: (212) 305 2300





On Tue, Dec 3, 2013 at 2:47 AM, Seppänen Mikko <Mikko.Seppanen at hus.fi>wrote:


>  Dear Carla and Dewton,

>

>

>

> many thanks for the advice. Sadly, all (!) these have been attempted…. our

> dermatologists have consulted their colleagues all over Finland and

> Scandinavia - to no avail. Antibiotics with immunomodulatory properties

> have thus not helped either.

>

>

>

> The patient has bipolar disorder, a very gentle man with few social

> contacts and for obvious reasons often depressed. He tries to lose weight

> and usually succeeds to drop off 10 kg before the next crisis or manic

> period starts (not too bad, control with drugs is quite good)… And he does

> know very well (he is actually quite intelligent and also has excellent

> social skills) that he should lose weight…

>

>

>

> TNF blockers would seem logical and seem to work for severe HS in immune

> competent. I was hoping that someone had encountered a similar patient ad

> found them to be safe/ tell me if they are not…

>

> I personally am quite reluctant to use either of the TNF blockers (numbers

> 1-2 infiximab, numbers 3-4 etanercept), since:

>

>

>

> 1)...the last CVIDs patient we put on TNF-blocking agent due to

> granulomatous, severe and disfiguring  skin lesions (those did respond)

> after 6 months developed CMV colitis (3rd such adult CVID in Finland I know

> of, 2 previous ones fatal), got it suppressed with early dg and

> ganciclovir. He then developed smoldering—full blown HLH, and we last week

> found B-nHL in the liver.... He is in WES (= is it truly “normal” CVID)?

> And had his 1st chemo just last week. Let us hope he survives.

>

>  2) The one before, due to skin granulomas (I think he may have some form

> of yet unidentified CID or a minimum of LOCID, is in WES presently as well)

> developed severe axonal polyneuropathy due to vasculitis that manifested

> after 3-4 months of treatment (a known, rare side effect). He is in

> Neurology in Tampere University Hospital, and receives high dose

> immunomodulatory IVIg+ MMF, recovering a little, bound to wheelchair and in

> agony with neuropathy.

>

>  3-4) I have had therapeutically non-responsive CVIDs patients: 2

> patients with severe "MbC" (or should we call it CVID-IBD?) and fistulas

> (the other ended up in colectomy and permanent stoma, though in classic

> MbCrohn not very wise, does well, the other seems to be doing relatively

> well with conventional therapy for time being).

>

>

>

> So (since they are adults? different genetic background?) I am not too

> eager to use TNF blockers…. However, present situation is quite

> unacceptable. And one cannot help feeling deeply empathic despite his

> excess weight.

>

>

>

>

>

> Mikko Seppänen, MD PhD

>

>

>

>

>

>

>

>

>

>

>

> *Lähettäjä:* Carla Gianelli [mailto:gianellicarla at gmail.com]

> *Lähetetty:* 3. joulukuuta 2013 9:23

> *Vastaanottaja:* CIS-PIDD

> *Aihe:* Re: [cis-pidd] CVID with hidradenitis suppurativa+ acne

> conglobata and recurrent boils

>

>

>

> Etarnecept has  better results  than infliximab  (25mg twice a week s.c.)

>

>

>

> Alternative treatment to be consider with his dermatologist : CO2 laser +

> healing by second intention / láser Smoothbeam ( diode laser . 1.450 nm) till

> the patient loose weight .

>

>

>

> Best regards

>

>

>

> 2013/12/2 Dewton Vasconcelos <dmvascon at usp.br>

>

> Due to the fact that I am at the department of dermatology I usually see

> patients with very severe acne and several patients with hydrosadenitis

> suppurativa (without CVID).

>

> Among these patients, besides surgical therapy, we usually treat with

> prolonged antibiotics (doxycycline 100 mg bid, or a macrolide

> (erythromycin, clarithromycin or azythromycin) or even dapsone, with

> interesting response in several patients.

> There is a trend to relapse after suspension of the therapy, so we treat

> some patients with a vitamin A analog (acitretin or isotretinoin) with good

> response and prolonged periods free of disease.

> It is important to try to convince your patient to lose weight, as obesity

> is associated with worsening of the HA symptoms and signs.

>

> I have never used TNF blockers but seems to me an interesting idea (as you

> noted, maybe a little bit dangerous because of continuous infections).

>

> Good luck with your patient,

>

> Dewton

>

> Dewton de Moraes Vasconcelos, MD, PhD

>

> Primary Immunodeficiencies Outpatient Unit ADEE3003

>

> Lab. of Medical Investigation Unit 56

>

> University of São Paulo School of Medicine

>

> Elie Haddad wrote:

>

>  Same for me (n = 1), although the patient did not have CVID.

>

> Elie

>

>

>

>

>

> Elie Haddad, MD, PhD,

>

> Professor of Pediatrics, University of Montreal,

>

> Head, Pediatric Immunology and Rheumatology Division,

>

> CHU Sainte-Justine, 3175 Cote Sainte-Catherine

>

> Montreal, QC, H3T 1C5, Canada

>

> Ph: 1 514 345 4713

>

> fax: 1 514 345 4897

>

> e-mail: elie.haddad at umontreal.ca

>

>

>

>

>

>

>

>

>

> Le 2013-12-02 à 06:26, Sullivan, Kathleen a écrit :

>

>

>

>  I've been impressed wit TNF inhibitors (n=2).

>

>

>

> Kate

>

> On Dec 2, 2013, at 3:58 AM, Seppänen Mikko wrote:

>

>

>

>   Dear all,

>

>

>

> I had today at the office a 35 y old male, whom I have seen for 8 years at

> my office. He was sent for consultation due to recurrent boils, and the

> extent was atypical for his earlier diagnoses hidradenitis suppurativa

> (HS) and acne conglobata (AC). Had been symptomatic for 2 years.

>

>

>

> He was first diagnosed with low IgG (appr. 4g/L), and borderline low

> anti-PnP responses, then he progressed in 1-2 years to clear CVID,

> EUROClass B+smB-21norm, now has IgA 0.15 g/L, IgM <0.10, IgG 8.0 g/L. CD19+

> 0.42, CD4 0.582, CD8 0.24, NK/CD16+/56+ 0.16. HRCT clean, no respiratory

> infections.

>

>

>

> IgGRT has been tough, since he is obese (185cm, 135 kg) and his individual

> consumption is extremely high. He is now on Gammanorm 140 and 160ml

> alternatingly every other week (the highest absolute dose ever in my

> practice). Per/kg makes only 0.71 g/kg/month.

>

> He does not loose it to stools, nor to urine, nor is his spleen large, nor

> is there any other obvious reason outside the skin condition. If not this

> high an IgGRT: he is in sepsis in ICU.

>

>

>

> I will switch to Hizentra (0.88 g/kg/month) now, just to try something

> (the dose is the largest he wants to take, and he is not eager for daily

> push).

>

>

>

> He clearly has HS and AC as well. Every conventional therapy for these has

> been tried, conservative+operative treatments to the max, and still he has

> almost all the time suppurating boil somewhere. They used to be restricted

> to inguinal and axillar areas, now he has had twice scrotal abscess in 2

> years (each time to hospital and 1-2 weeks of i.v.ab, operative therapy),

> has presently suppurating boils in gluteal, preauricular regions, in

> external ear etc. etc.

>

>

>

> The only mode of therapy not tried is biologicals, partly due to CVID and

> scanty experience in this setting and partly since he has bacterial

> culture+ boils ......continuously. Ab prophylaxis only selects multi-R

> strains, will not do any good.

>

>

>

> Any suggestions? I would be grateful for fresh ideas..... TNF

> blockers????????

>

>

>

> Yours respectfully,

>

>

>

> Mikko

>

>

>

> Mikko Seppänen, MD PhD

>

> Finland/HUCH

>

>

>

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>

> Kate Sullivan, MD PhD

> Professor of Pediatrics

> ARC 1216 Immunology CHOP

> 3615 Civic Center Blvd.

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