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 are:
- 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).
- 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
- 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.
- 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
- 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.
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