[CIS PIDD] [cis-pidd] Skin Candida with partial MBL deficiency & Low CD8 effector memory

Sun Jul 12 16:44:20 EDT 2015

Thanks very much to both Juan Carlos Aldave Becerra  and Mikko Seppänen for your valuable suggestions.

Mikko, she is actually from Russia, on sabbatical here. For CMCC, the onset seems late and she does not have endocrine disorders associated with the AIRE mutation, Candida proliferation is intact whereas V. Zoster is reduced and Tetanus absent, I would have expected the opposite. I do realise though that CMCC is a heterogenous disorder both clinically & immunologically. I will try to get the genetic studies done.

Many thanks & Regards,

Stan

From: "Seppänen fi>" <Mikko.Seppanen at hus.fi<mailto:Mikko.Seppanen at hus.fi>>
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Date: Saturday 11 July 2015 at 7:04 AM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: Re: [MARKETING][cis-pidd] Skin Candida with partial MBL deficiency & Low CD8 effector memory

Hi Stan,

I sincerely doubt, check the known CMC genes and an anti-cytokineAb panel? Her ethnicity, from Far East?

The prevalence of that level of MBL2 def is very high in Finland, I never see such a degree of CMC as its manifestation.

In adults, there is a very small percentage of specifically CD8 low individuals and an excess of infections that remain a mystery.

But since this is clear CMC, I definitely would investigate accordingly.

T: Mikko Seppänen, Finland

Stan Ress <stan.ress at uct.ac.za<mailto:stan.ress at uct.ac.za>> kirjoitti 9.7.2015 kello 20.37:

Dear colleagues,

I’d appreciate your opinion on an interesting 38 year-old lady referred to me by her dermatologist for immune evaluation. She has a background history of life long irritable bowel syndrome and frequent gastroenteritis but is generally otherwise well.
Upper endoscopy in 2010 reported erosions ? H. Pylori (but no treatment details). In September 2014 she developed a significant skin rash and Candida was isolated, in March it also involved her face, and her dermatologist treated these with  oral itraconazole and Terbinafine HCL and a facial cream, to which she has responded. Aside from symptoms of bloating and heartburn, there was no history of any other infections or antibiotic use that could precipitate the Candida. Blood tests showed normal FBC, ESR –5, random glucose & HBA1c 5.5, normal full liver functions, and negative HIV. Normal serum Ig A,G,M levels, C3 & C4 & CH50, but partial deficiency of MBL 759 ng/ml (>1000). Coeliac serology negative. IGRA TB T-spot test negative (positive PHA control). Lymphoproliferation to candida was normal, reduced to Varicella Zoster, and absent to Tetanus. Baseline vaccination status indicated sufficient protective IgG to S. Pneumonia, Tetanus Toxoid, C. diphtheriae, and very high levels against B. Pertussis but insufficient titre against H. Influenza. Flow cytometry indicated normal CD3/CD4 but reduced CD8  of 360 /ul (500-900) and NK 191/ul (200-400) with CD4/8 ratio of 2.55:1. B-cell % was 13.5% with total memory B-cells of 20.7%, Naïve B-cells of 73%, and class-switched memory B-cells of 9.48%. CD4/CD45 Naïve & memory T-cells were normal range, as were recent thymic emigrants, central memory helper cells, effector memory helper cells, total naïve cytotoxic cells, and central memory cytotoxic cells. However, effector memory cytotoxic T-cells were low at 2/ul (0-36).

Are the findings of partial reduction in MBL, low CD8+ T-cells and lowish effector memory cytotoxic T-cells enough to explain her susceptibility to Candida with significant skin involvement?

I’d greatly value thoughts about this and any suggestion for further investigations and treatment (Prophylactic itraconazole long term?)

Thanks,

Stan
-- --
Stanley Ress
Specialist Physician & Clinical Immunologist
Emeritus Associate Professor of Medicine, UCT
UCT Private Academic hospital,
Anzio Road, Observatory,
Cape Town, 7925
South Africa
TEL:INTERN. + 2721-4421966 or 4421816
FAX:   "    + 2721-(0)865173095
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email: stan.ress at uct.ac.za<mailto:stan.ress at uct.ac.za>
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