Review Question - QID 103798

The dermatologic lesion pictured is most consistent with dermatitis herpetiformis (DH). DH is associated with gluten sensitivity, and is a skin manifestation of celiac disease.

DH is a dermatologic disease in which patients develop pruritic inflammatory papules and vesicles typically over the extensor surfaces of the forearms. The lesions can also appear on the knees, scalp, and buttocks. The cause is usually a gluten sensitivity. Skin biopsies will reveal subepidermal IgA deposits and neutrophilic inflammation in the dermis. Treatment is dapsone and a gluten-free diet.

Pelkowski and Viera describe the diagnosis and management of celiac disease, an autoimmune disorder of the gastrointestinal tract caused by gluten intolerance. Celiac disease is characterized by chronic inflammation of the small intestine, leading to atrophy of the villi and malabsorption. In addition to dermatitis herpetiformis, other extraintestinal manifestations of celiac disease include iron deficiency anemia, decreased bone mineral density, and neuropathy.

Krishnareddy et al. performed a cross-sectional study comparing patients presenting only with DH versus those presenting with DH in the context of celiac disease. Small bowel biopsies revealed that patients who presented with both celiac disease and DH were more likely to have severe pathology than those presenting only with DH.

Figure A shows typical DH lesions on the extensor surfaces of the forearms.

Incorrect Answers:
Answer 1: Crohn's disease is associated with erythema nodosum.
Answer 2: Ulcerative colitis is associated with pyoderma gangrenosum.
Answer 4: Juvenile rheumatoid arthritis, Still's disease, is associated with a salmon-pink macular rash usually on the trunk.
Answer 5: Patients with SLE may have a number of different skin conditions. A commonly tested rash is the "butterfly" or malar rash on their face.