Abstract
Celiac disease (CD) is an immune-mediated enteropathy caused by gluten ingestion, affecting approximately 1% of the worldwide population. Extraintestinal symptoms may be present as the first signs of CD, years before the CD diagnosis is made. A great variety of extraintestinal manifestations may be associated with CD. Cutaneous manifestations represent the main extraintestinal manifestations, with dermatitis herpetiformis being the most common in patients with CD. In adults, it has been demonstrated that the role of a gluten-free diet is crucial not only for the recovery of signs and symptoms associated with CD but also for cutaneous manifestations, which often improve after gluten avoidance. In children with CD, the association with skin disorders is well documented regarding dermatitis herpetiformis, but studies considering other dermatological conditions, such as psoriasis and atopic dermatitis, are few. The prevalence and manifestations of dermatological disorders in celiac children are often different from those in adults, explaining the gap between these populations. In addition, the therapeutic role of a gluten-free diet in the improvement in skin alterations is not fully understood in children and in adult population except for dermatitis herpetiformis. Therefore, cutaneous CD symptoms need to be known and recognized by physicians despite their specialties to improve early CD diagnosis, which is critical for a better prognosis. This review describes the current scientific evidence on skin manifestations associated with CD in the pediatric population.
Highlights
Celiac disease (CD) is a systemic immune-mediated condition characterized by an aberrant response to wheat gliadins and other cereal prolamins that causes small intestinal enteropathy and a wide range of symptoms in genetically susceptible individuals [1]
Feirgyuthreem1.aD, uerrtmicaatriitaisl pHlearqpueetsi,foprampiusl.e(sA, g) rEoruyptheedmvaetsoiculse,spaanpdublalirs,taenrsdavsessoicciualtoesduws iltehsiionntseninseait1c4hyaenadr othldercehfoilrde wfoiltlhowAetodpbicy Dereorsmioantist,isexacnodridatiiaognnso, sainsdofhCypeleirapcigdmiseeanstea.ti(oBn,.C) a magnification of the Dermatitis herpetiformis (DH) shows a typical polymorphism consisting of erythema, urticarial plaques, papules, grouped vesicles and blisters associated with intense itch and followed by erosions, excoriations, and hyperpSiegrmumentIagtAio-nc.lass antibodies against TG2, the autoantigen of CD, frequently circulate in undiagnosed patients with DH and should always be kept in mind in clinical practice [42]
Skin manifestations are frequently associated with CD in both adults and children
Summary
Celiac disease (CD) is a systemic immune-mediated condition characterized by an aberrant response to wheat gliadins and other cereal prolamins that causes small intestinal enteropathy and a wide range of symptoms in genetically susceptible individuals [1]. A higher number of asymptomatic cases together with an increase in the number of “non classical” presentations, including extraintestinal symptoms such as iron deficiency anemia, altered bone metabolism, short stature, elevated liver enzymes and skin manifestations, have been detected by targeted screening of at-risk groups [3]. CD patients present an increased risk for autoimmune disorders compared to healthy controls, with a prevalence ranging from 14% to 27% [5,6]. A higher prevalence of CD has been recognized in patients with a number of autoimmune disorders, warranting an active screening only in a few diseases, such as type I diabetes mellitus [7]. There are some hypotheses related to this association: the shared genetic features between CD and the other immune-mediated disorders, the alteration of the intestinal barrier, and a similar environment trigger [8]. We aim to improve the early recognition of CD-associated skin manifestations, recommending active case finding for CD and reducing the risk of diagnostic delay
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