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Pathology - Dermatitis herpetiformis
Definition: An immunobullous skin disorder marked by severe pruritus, papules, and vesicles, granular IgA deposition in the papillary dermis, and a significant correlation with celiac disease.
Epidemiology • Uncommon. The condition can afflict individuals of any age, while the highest frequency occurs in young people aged 20 to 40 years. Males are impacted twice as frequently as females. This illness is very prevalent in Northern Europe and Ireland. Up to 90% of individuals exhibit indications of celiac disease, although this may be asymptomatic.
Aetiology mediator. • IgA transglutaminase antibodies generated in the gastrointestinal tract seem to be pivotal.
Pathogenesis • IgA transglutaminase antibodies interact with transglutaminase enzymes present in the skin. The fixation of complement induces chemotaxis of neutrophils into the papillary dermis. • Enzymes secreted by neutrophils induce blister development.
Presentation • The rash consists of clusters of papules and vesicles that are highly pruritic. • Preferred locations are the shoulders, back, buttocks, elbows, and knees.
Histopathology Biopsies from first lesions reveal aggregates of neutrophils within the papillary dermis (papillary dermal microabscesses). Biopsies from established lesions reveal a subepidermal blister abundant in neutrophils. Immunofluorescence Direct immunofluorescence of perilesional skin demonstrates granular deposition of IgA in the papillary dermis.
Prognosis: The condition is typically chronic and persistent; nevertheless, it exhibits a significant response to the medication dapsone.
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