Synonym: Duhring's disease.
■ Dermatitis herpetiformis is characterized by a symmetric extremely pruritic papulovesicular eruption on the extensor extremities (Fig. 6A).
■ A diffuse "herpetiform" distribution of lesions that are preceded by stinging sensation can also be seen (Fig. 6B). The mean age of patients is 40.
■ Dermatitis herpetiformis is associated with gluten-sensitive enteropathy in up to 90% of patients, but most patients are asymptomatic.
Histology (Figs. 6D and E):
■ Infiltration of the papillary tips with neutrophils (micro-abscesses), diagnostic of dermatitis herpetiformis
Bullous lupus erythematosus; linear IgA bullous disease. A
rare variant of vasculitis, pustular vasculitis, has not only papillary dermal pustules, but also vasculitis involving the papillary dermal vessels.
■ DIF: granular IgA in papillary tips on perilesional skin of dermatitis herpetiformis (Fig. 6C)
■ IIF: circulating IgA antibodies to papillary tip immuno-reactants not seen
MUCOUS MEMBRANE PEMPHIGOID/CICATRICIAL PEMPHIGOID
■ Circulating antibodies to endomysium of smooth muscle, gliadin, and thyroid microsomal proteins are present, but are probably not directly pathogenic.
■ IgA deposits in skin promote neutrophil recruitment to basement membrane zone, where proteases are released and degrade the basement membrane zone proteins, leading to blister formation.
Location on extensor elbows,
Exposed areas likely to be traumatized
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