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General Reference ⬇

Nejm 1983;308:816

Pathophys and Cause ⬆ ⬇

Cause:Genetic association in 90% (HLA B8 and DRw3—Ann IM 1982;97:105) but not clinically hereditary

Pathophys:IgA deposition at dermal/epidermal junction, because is allergic in etiology (J Am Acad Derm 1992;27:209, Ann IM 1982;97:105)

Epidemiology ⬆ ⬇

Associated with Hashimoto's thyroiditis, hypothyroidism, hyperthyroidism, and thyroid nodules in 50% (Ann IM 1985;102:194); pregnancy; hypoparathyroidism

Males > females

Signs and Symptoms ⬆ ⬇

Sx:Debilitating itch and rash; precipitated by UV, steroids, and infection (Ann IM 1984;100:677)

Si:Umbilicated, grouped vesicles, r/o other viral (H. simplex, H. zoster, variola, varicella)

Complications ⬆ ⬇

Lymphomas

r/o pustular psoriasis, often indistinguishable even histologically (Ann IM 1984;100:677)

Lab and Xray ⬆ ⬇

Lab: Path:Small bowel bx shows sprue-like pathology even though asymptomatic; skin bx shows IgA at dermal-epidermal junction

Treatment ⬆

Rx:

Gluten-free diet, rice and oats ok (Nejm 1997;337:1884) but takes 2 years to help

Sulfapyridine 4 gm po qd

Dapsone 100 mg po qd; inhibits polys chemotaxis; adverse effects: G6PD deficiency anemia precipitation, severe peripheral neuropathies