Background
Idiopathic atrophoderma of Pasini and Pierini (IAPP) is a form of dermal atrophy that manifests as single or multiple sharply demarcated, hyperpigmented, non-indurated patches. These patches are marked by a slight depression of the skin with an abrupt edge, creating a "cliff-drop" border, and are usually located on the backs of adolescents or young adults. The lesions may be discrete or confluent. The affected skin appears thinned and discolored, but the consistency and feel of the affected skin remains normal.
In 1923, Pasini [1] described a case of pigmentary atrophoderma that was both clinically and histologically unique from any known atrophy, including localized scleroderma, under the name progressive idiopathic atrophoderma. In 1936, in Argentina, Pierini and Vivoli [2] extensively studied and defined the condition and its possible link to morphea. In 1958, the disorder was first introduced into the American dermatologic literature by Canizares et al, [3] who reviewed Pierini’s findings and renamed it idiopathic atrophoderma of Pasini and Pierini. Canizares et al believed that idiopathic atrophoderma of Pasini and Pierini differed sufficiently from morphea to classify it as a distinct entity.
Since then, reports of the co-occurrence of morphea and occasionally lichen sclerosus et atrophicus with idiopathic atrophoderma of Pasini and Pierini suggest a close relationship between idiopathic atrophoderma of Pasini and Pierini and morphea. In 2000, Yokoyama et al [4] reported that skin glycosaminoglycans extracted from idiopathic atrophoderma of Pasini and Pierini lesions are different from those in typical morphea lesions.
Pathophysiology
The cause of atrophoderma of Pasini and Pierini is not known. The pathophysiologic events that cause the discrete lesions seen clinically, as well as the timing of their appearance, are also unknown. Some authors have suggested a role for infection with Borrelia burgdorferi.
Etiology
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 [5] 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.
Epidemiology
Frequency
United States
The exact incidence of idiopathic atrophoderma of Pasini and Pierini is not known. The small number of cases reported may reflect its asymptomatic nature rather than its true incidence.
International
Idiopathic atrophoderma of Pasini and Pierini is more frequently reported in Europe than in North America, paralleling the incidence of acrodermatitis chronica atrophicans and suggesting involvement of European Borrelia strains.
Race
Idiopathic atrophoderma of Pasini and Pierini is more common in whites and is rarely reported in blacks or Asians.
Sex
Idiopathic atrophoderma of Pasini and Pierini is more frequently encountered in women than in men, with a ratio of 6:1.
Age
Idiopathic atrophoderma of Pasini and Pierini usually begins insidiously in individuals during the second or third decade of life. However, it has been described in individuals as young as 7 years and as old as 66 years. Four reports of congenital atrophoderma have been described in the literature. [6, 7, 8, 9]
Prognosis
Idiopathic atrophoderma of Pasini and Pierini has no known effect on overall health. The condition often runs a protracted course over 10-20 years and may self-resolve.
Patient Education
Excessive sun exposure may cause the more deeply pigmented lesional skin to become darker, resulting in further uneven discoloration of the skin. This may be perceived as a cosmetic problem.
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Early lesion demonstrating diagnostic "cliff-drop" border to atrophy. Courtesy of Joe Eastern, MD.
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Older lesion showing typical pigmentation and classic "cliff-drop" border. Courtesy of Joe Eastern, MD.
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Single ovoid patch of atrophoderma on the back of a young adult female.
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Atrophic hyperpigmented patch with characteristic “cliff-drop” borders.
