Intensely itchy, chronic papulovesicular eruption distributed symmetrically on extensor surfaces.
Characterized histologically by dermal papillary collections of neutrophils.
Granular immunoglobulin A deposits in normal-appearing skin are diagnostic.
Epidermal transglutaminases appear to be the dominant autoantigens.
Most, if not all, dermatitis herpetiformis patients have an associated gluten-sensitive enteropathy.
The rash responds rapidly to dapsone therapy and, in many patients, to strict adherence to a gluten-free diet.
In 1884, Louis Duhring first described the clinical features and natural history of a polymorphous pruritic disorder that he called dermatitis herpetiformis (DH); however, the critical elements in the pathogenesis of DH remained unknown until the 1960s.1 In 1888, Brocq described patients with a very similar disorder and called it dermatite polymorphe prurigineuse.2 In addition, he analyzed Duhring’s report and excluded several types of patients from the diagnosis. Since 1888, several important discoveries have been made. In 1940, Costello3 demonstrated the efficacy of sulfapyridine in the treatment of DH. In early 1960s, Pierard and Whimster4 and MacVicar et al5 found that early lesions of DH are characterized by neutrophilic microabscesses in the dermal papillae. In 1967, Cormane6 found that the skin of DH patients contained granular immunoglobulin deposits in dermal papillary tips, and in 1969, van der Meer7 extended these studies and found that the most regularly detected immunoglobulin (Ig) deposited in DH is IgA. The association between DH and intestinal abnormalities was first observed by Marks et al8 in 1966. Fry et al9 and Shuster et al10 identified the intestinal findings as a gluten-sensitive enteropathy. In 1973, Fry et al11 demonstrated that strict adherence to a gluten-free diet would improve the skin disease as well as reverse the intestinal abnormality, as occurs in celiac disease. Katz et al12 identified a strong association between DH and certain histocompatibility antigens in 1972. In 1979, Jablonska and Chorzelski13 distinguished those patients with linear IgA deposits from those with granular IgA deposits and defined a distinct entity. In 1999, Dieterich et al14 identified antibodies to tissue transglutaminases in the sera from DH patients. Distinguishing between various types of transglutaminases enabled Sárdy et al,15 in 2002, to demonstrate that epidermal transglutaminase (eTG) is the dominant autoantigen in DH. In 2016, Görög et al16 demonstrated circulating transglutaminase 3–IgA immune complexes in DH patients.
The prevalence of DH in various white populations varies between 10 in 100,000 persons and 75 in 100,000 persons.17-19 The male-to-female ratio ranges from 1.1 to 1 to 1.5 to 1. It may start at any age, including in childhood; however, the second, third, and fourth decades of life are the most common ages. After presentation, DH persists indefinitely in most patients, although with varying severity.
Patients with DH have an associated gluten-sensitive enteropathy (celiac disease) that is usually asymptomatic.