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

[Carla Gianelli]

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.

> Philadelphia, PA 19104

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