PhD Scientific Days 2022

Clinical Medicine VI. (Poster discussion will take place on the terrace of the room during the Coffee Break)

Selective IgA deficiency in dermatitis herpetiformis

Előadó neve

Dr. Malkovics, Tamás Ákos

Előadó munkahelye

Department of Dermatology, Venereology and Dermatooncology

Előadó telefonszáma

+36205827780

Előadó e-mail címe

malkovics.tamas_akos@med.semmelweis-univ.hu

Az előadás címe

Selective IgA deficiency in dermatitis herpetiformis

Szerző(k) neve és munkahelye

Dr. Tamás Ákos Malkovics, Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest
Prof. Dr. Miklós Sárdy, Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest

Bemutatás módja

Poszter

Szekció

Clinical Medicine VI. (Poster discussion will take place on the terrace of the room during the Coffee Break)

Language of the presentation

Hungarian

Preferred session

Clinical Medicine

Összefoglaló szövege

Introduction
Dermatitis herpetiformis, also known as Duhring’s disease, is a gluten-induced, bullous autoimmune skin disease, associated with celiac disease of various severity. The association between celiac disease and selective IgA deficiency (total serum IgA less than 0.7 g/L without the deficiency of other Ig classes) has been previously known for decades. 2-3% of celiac disease patients are reported to be IgA deficient which is a 10 to 15 times higher prevalence than that in the normal population. The prevalence of selective IgA deficiency in dermatitis herpetiformis has not been well determined.

Aims
Our aim was the determination of the prevalence of selective IgA deficiency in dermatitis herpetiformis and its impact on the diagnostic procedure. We also present two cases of our 75 patients with both selective IgA deficiency and dermatitis herpetiformis.

Methods
The study was carried out prospectively in 1 year at the Department of Dermatology, Venereology and Dermatooncology, Semmelweis University between May 2021 – May 2022. Serum IgA levels of 75 dermatitis herpetiformis patients were prospectively evaluated via routine laboratory procedure. The diagnosis of the patients was established between 1979 and 2022.

Results
2/75 (2.66%) of our patients had lower serum IgA levels, selective IgA deficiency. Further, one of our patients (a 35-year-old woman) had selective IgA deficiency (0.6 g/L) in 2000, but in 2021, her total serum IgA level was normal (1.39 g/L).

Conclusion
Our results raise the attention to the coincidence of gluten sensitivity and primary immunodeficiency. Dermatitis herpetiformis can occur with lower IgA levels but pathognomic role of immune complexes formed by transglutaminase 3 (TG3) and anti-TG3 IgA in the skin seems to be essential for the development of the disease. For this reason, development of dermatitis herpetiformis in case of complete lack of IgA immunoglobulins does not seem to be possible; to date no such case has been presented. Even in patients with selective IgA deficiency, direct immunofluorescence microscopy is specific for the disease. Virtually all cases of dermatitis herpetiformis can be detected by direct immunofluorescence microscopy which is the gold standard method of the diagnosis.

Funding
Semmelweis 250+ Kiválósági PhD Ösztöndíj: EFOP-3.6.3-VEKOP-16-2017-00009

University and Doctoral School

Semmelweis University, Károly Rácz Doctoral School of Clinical Medicine

Supervisor

Prof. Dr. Miklós Sárdy

Publication of my abstract

I do not give consent to the publication of my abstract on the website of the congress.

Kind

Szabad

Status

elfogadva

Accepted presentation method

poszter

Előadás fájl jóváhagyás

nem rendelkezett róla

Előadó

6111

Start

11:00

End

11:05

Authors (legacy)

Dr. Tamás Ákos Malkovics, Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest
Prof. Dr. Miklós Sárdy, Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest