Typically, DH can be characterized as an intensely itchy skin eruption distinguished by the formation of small papules or vesicles. The patient may perceive having red bumps and blisters. The symptoms of intense burning, stinging and itching cannot be overemphasized. It is common for DH lesions to be symmetrically distributed on the extensor surfaces of the body: over the elbows, knees, buttocks, scalp, posterior, neck, sacral (lower end of the spinal column) and shoulder areas. The face and facial hairline are occasionally affected. And rarely, the lesions occur within the mouth. Although its severity may vary, it persists indefinitely and is a lifelong condition. There are only a few patients in whom the disease remits for long periods of time.

The lesions may first appear as discolorations and next consist of small bumps called papules and small blisters called vesicles. These lesions tend to occur in groups much like the lesions of Herpes, hence the term herpetiformis, meaning "like Herpes." Because of the intense itching, the lesions are often scratched; these scratched areas will then develop crusts and often then heal with changes in color, either those of hypopigmentation or hyperpigmentation. Most DH patients can usually predict the location of a new lesion as much as 8 to 12 hours before the actual onset because of the symptoms of localized burning and itching. Though the blisters tend to come out in crops, all stages can be seen at any one time. The blisters may take from 7 to 10 days to lose their itching and burning sensations and then begin to crust. They are rarely inflamed and don't contain pus. Although there are recorded cases of the lesions occurring on the lips and within the mouth, it is extremely unusual for lesions to occur on mucous membranes (soft tissues at body openings) in other areas of the body.

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