Dermatitis Herpetiformis

 

Epidemiology:

    • M > F
    • 20s and 30s

 

Common sites:

    • Extensor surfaces, elbows, knees, upper back, buttocks

 

Clinical features:

    • (extremely pruritic) papules, vesicles, occasional bullae
    • erythematous, often urticarial base
    • herpetiformis refers to tendency of vesicles to group
    • association with celiac disease
    • responds to gluten-free diet

 

Histologic features:

    • fibrin and neutrophils at the tips of papillae (microabscesses)
    • vacuolization of overlying basal cells
    • dermoepidermal separations, from minute at papillae, to blister formation
    • eosinophils in older lesions

 

Immunophenotype:

Marker:

Sensitivity:

Specificity:

IgA (granular at tips of papillae)

 

 

 

Molecular features:

    •  

 

Other features:

    • major association with Celiac
    • responds to a gluten-free diet
    • IgA anti-gliadin antibodes deposited on anchoring fibrils (reticulin)
    • Histocompatibility types HLA-B8 and HLA-DRw3 are higher risk

 

References:

    • Robbins 2005