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November 1998

Pigmented Purpura Over the Lower Extremities

Arch Dermatol. 1998;134(11):1477-1482. doi:

First described in 1896 by Majocchi,1 and later introduced to the American literature in 1915 by MacKee,2 purpura annularis telangiectodes of Majocchi is an uncommon pigmented purpuric eruption that is seen most frequently in adolescent girls. Clinically, the disease is characterized by symmetrical telangiectatic, purpuric, and atrophic patches with a predilection for the lower extremities and buttocks. The distribution may extend to the trunk and upper extremities, however. Classically, the lesions progress in 3 stages. The first, or telangiectatic, stage, is characterized by millet seed–sized bright-red macules that partially fade on pressure. Perifollicular involvement is common but not constant. During the second, or purpuric-pigmentary, stage, these areas assume a darker, yellowish rust color. The initial macules coalesce and enlarge to form annular patches ranging from 2 to 20 mm in diameter that do not fade on pressure. While primarily annular, these patches may be linear, macular, stellate, or serpentine in shape. In the third, or atrophic, stage, the patches progress to form a smooth depigmented and atrophic center with alopecia, surrounded by a halo of hyperpigmentation.3 The complete cycle is observed in only a certain percentage of cases, with the third stage being the least reliable; however, the first stage is constant.3 The patient may experience only 1 outbreak, but relapses and remissions are common.4 Symptoms, if present, are mild to moderate pruritus.5 The results of hematologic studies, including bleeding time, clotting time, and thrombocyte count, are invariably normal.6