dermatits herpetiformis Flashcards
what granular deposits are duagnostix
■ Achronic, recurrent, intensely pruritic eruption occurring symmetrically on the extremities and the trunk.
■ Consists o tiny vesicles, papules, and urticarial plaques that are arranged in groups.
■ Associated with gluten-sensitive enteropathy (GSE).
■ Characterized histologically by papillary collection o neutrophils.
■ Granular IgAdeposits in paralesional or normal skin are diagnostic.
■ Responds to sul a drugs and, to a lesser extent, a gluten- ree diet
age
gender
AGEOFONSET Mostcommonat30to40years; may occur in children.
SEX Male: emale ratio is 2:1
precipitating factor
Symptomso enprecedetheappearanceo skin lesionsby8to12h.Ingestiono iodidesand overload o gluten are exacerbating actors
skin lesions
Erythematouspapulesorwheal- like plaques (Fig. 6-17); tiny rm-topped vesicles, sometimes hemorrhagic
predilection
Extensor areas—elbows (Fig. 6-18), knees. Strikingly symmetrical. Buttocks, scapular, and sacral areas (Figs. 6-18 and 6-19). Here, o en in a “butter y” ashion. Scalp, ace, and hairline
HLA types assoc to
IMMUNOGENETICS Association with HLA-B8, HLA-DR, and HLA-DQ
IF
granular igA Deposits
c3
c5
tx
SYSTEMICTHERAPY Dapsone. 100 to 150 mg daily, with gradual reduction to as low as 50 mg twiceaweek.Dramaticresponse,o enwithin hours. Obtain a glucose-6-phosphate dehydro- genase level be ore starting sul ones; obtain methemoglobin levels in the initial 2 weeks and
ollow blood counts care ully.
Sulfapyridine. 1 to 1.5 g/d, with plenty o u- ids, i dapsone contraindicated or not tolerated. Monitor or casts in urine and kidney unction. DIET A gluten- ree diet may suppress the disease or allow reduction in the dosage of dapsone or sul apyridine, but response is very slow.