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Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

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Dermatitis herpetiformis in siblings.

N Chmurova¹, Z Parnicka, D Svecova

¹Department of Dermatovenerology, Faculty of Medicine, Comenius University, Mickiewiczova 13, SK-813 69 Bratislava 1, Slovakia. nada.chmuroav@centrum.sk

Bratislavske Lekarske Listy

|March 4, 2008

Summary

Dermatitis herpetiformis (DH) can present differently in families, even in sisters. This case highlights the genetic link of DH to autoimmune disorders and the HLA-DQB1*0201 allele.

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Area of Science:

Immunodermatology
Genetics
Autoimmunity

Background:

Dermatitis herpetiformis (DH) is a chronic autoimmune blistering skin condition.
DH is strongly associated with celiac disease (CD) and specific HLA alleles.

Observation:

Two sisters presented with DH, but with differing clinical courses and comorbidities.
One sister had DH, CD, and autoimmune thyroid disease, while the other had only DH.
The sisters shared homozygous HLA-DQB1*0201 alleles.

Findings:

Confirms the association between DH and other autoimmune disorders.
Reinforces the established link between DH and the HLA-DQB1*0201 allele.
Suggests a potential familial predisposition to DH, challenging its general classification.

Implications:

Highlights the importance of HLA typing in understanding DH pathogenesis.
Suggests that genetic factors may play a more significant role in DH familial occurrence.
Emphasizes the need for comprehensive screening for associated autoimmune conditions in familial DH cases.