Centro Studi GISED

Dermatitis herpetiformis (DH) is a rare skin inflammatory disease with a chronic-relapsing course, characterized by strongly itching polymorphic lesions (1). DH is considered to be the specific phenotypic cutaneous expression of a gluten-sensitive enteropathy similar to celiachy (C) (2,3).

The Dermatology clinic of the University of Florence, in collaboration with Centro Studi GISED, organized a specific project on DH.

The aims of the study were:

• To identify the diagnostic modalities observed in Italy
• To investigate possible diseases related to DH (insulin-dependent diabetes mellitus, Hashimoto's thyroiditis, Sjögren syndrome, Down syndrome, IgA deficiency, Multiple sclerosis, primary biliary cirrhosis and other epatopathies, nephropaties, Lupus erythematosus, vitiligo).
• To demonstrate the actual association Celiachy/Psoriasis, in consideration of the results of a retrospective study recently published by an Italian-Swedish group underlining that celiac patients have an absolute risk of future psoriasis equal to 135/100,000 person-years (4).
• To identify and define possible clinical profiles, histopathologically and/or immunopathologically atypical

Diagnostic protocol for DE

The following criteria are essential for a correct diagnosis:

• Clinical - outset characterized by papulo-erythematous and erythematous-pomphoid figured lesions, followed by vesiculobollous elements associated to scratching-derived lesions, symmetrically localized on the extensor surface of limbs (elbow in 90% of cases), gluteus and nape.
• Histopathology - presence of neutrophil granulocytes and fibrin (microabscesses) near the apex of dermal papillae.
• Direct immuno-fluorescence (gold standard) - IgA micro-granular deposits (near the apex of dermal papillae or along the whole dermo-epidermal junction (DEJ) with "enhancement" at the apex of the papillae) on perilesional cutis samples.
• Serology - IgA anti tTG (tissular transglutaminase or transglutaminase-2); anti-endomysium antibodies (to be tested only in doubtful cases and in reference centres); anti-gliadin antibodies (significant only in children up to two years old); anti-gliadin deaminated peptides antibodies (1).

1. Caproni M, Antiga E, Melani L, Fabbri P. Guidelines for the diagnosis and treatment of dermatitis herpetiformis. J Eur Acad Dermatol 2009;23:633-8
2. Garioch JJ, Lewis HM, Sargent SA, Leonard JN, Fry L. 25 years' experience of a gluten-free diet in the treatment of dermatitis herpetiformis. Br J Dermatol 1994;131:541-5.
3. Hervonen K, Karell K, Holopainen P, Collin P, Partanen J, Reunala T. Concordance of dermatitis herpetiformis and celiac disease in monozygous twins. J Invest Dermatol 2000;115:990-3
4. Ludvigsson JF, Lindelöf B, Zingone F, Ciacci C. Psoriasis in a nationwide cohort study of patient with celiac disease. J Invest Dermatol 2011;131:2010-6.

Results

The study confirmed some of the most relevant data regarding DH that have been previously reported in the literature. In addition, it was found a reduced diagnostic delay in females with respect to males, possibly related to the higher sensitivity of serologic testing in females with DH compared to males. Finally, the study demonstrated that intestinal involvement could be severe in patients with DH and that females should be tested for thyroiditis.

The results were published here:

Antiga E, Bonciolini V, Cazzaniga S, et al. Female Patients with Dermatitis Herpetiformis Show a Reduced Diagnostic Delay and Have Higher Sensitivity Rates at Autoantibody Testing for Celiac Disease. Biomed Res Int. 2019;2019:6307035.

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