Atrophoderma of Pasini and Pierini Clinical Presentation
- Author: Sarah Jane Adams, MD; Chief Editor: Dirk M Elston, MD more...
Idiopathic atrophoderma of Pasini and Pierini is typically a benign, asymptomatic condition. The disorder usually begins during adolescence or early adulthood with a slightly erythematous lesion appearing on the trunk, commonly on the back or lumbosacral region, followed in frequency by the chest, arms, and abdomen.
At first, only a single lesion may be present, but more often, multiple lesions 1-12 cm in diameter are present. Within 1-2 weeks, these lesions become hyperpigmented, appearing slate-gray to brown. Initially, localized lesions may slowly spread for months to years before becoming quiescent. Discrete new lesions may appear for 10-20 years, and old lesions may slowly enlarge, giving the skin a moth-eaten appearance. Transformation to generalized morphea has not been observed.
Lesions are single or multiple, irregularly round or ovoid patches, varying in size from a few centimeters to large areas across the trunk. A predilection for the legs has also been reported. The face, hands, and feet are usually spared. Distribution is often symmetric and bilateral; however, reports have described solely unilateral or zosteriform distribution. A rare, generalized, and lenticular variant has also been documented. Lesions have traditionally been described as hyperpigmented; however, Saleh et al described a retrospective study of 16 Lebanese patients in whom lesions were more hypopigmented (56%) and skin-colored (25%).
The lesions are usually asymptomatic and lack inflammation. The skin lesions may coalesce over time to form large, irregular, pigmented areas. The areas are usually brown but may have a blue hue.
Eventually, the pigmentation lightens, and the involved skin becomes depressed below the level of the surrounding skin. This change produces the characteristic, sharply defined "cliff-drop" borders, ranging from 1-8 mm in depth, although they can have a gradual slant. Close examination of the skin with side lighting demonstrates the unique cliff-drop border, giving the impression of an inverted plateau. Multiple lesions may have an appearance similar to Swiss cheese.
Note the images below.
The skin surrounding the patches appears normal. No erythema or lilac ring, as in morphea, is observed. Once indentation occurs, the lesions may become quiescent, stopping any further enlargement. The surface of the skin feels and appears normal, and no induration or sclerosis is noted.
In the late stages, superficial blood vessels may be visible in the depressed patches. No palpable difference can be felt between normal and affected skin. White, shiny sclerodermatous induration is sometimes seen in the central part of the lesions, and concurrent but separate characteristic plaques of morphea may be noted, supporting a possible relationship between these disorders.
Idiopathic atrophoderma of Pasini and Pierini may represent a late atrophic stage of morphea.
Some findings suggest that the spirochete B burgdorferi may be involved in the pathogenesis of some cases of idiopathic atrophoderma of Pasini and Pierini. Buechner and Rufi studied the sera of 26 patients with typical lesions. Ten (53%) of the 26 patients had immunoglobulin G anti–B burgdorferi antibodies, and 6 (14%) of control subjects had these antibodies. No immunoglobulin M antibodies were found.
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