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

[Seppänen Mikko]

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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