Atrophoderma of Pasini and Pierini Differential Diagnoses

Updated: May 03, 2018
  • Author: Sarah Jane Adams, MD; Chief Editor: Dirk M Elston, MD  more...
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DDx

Diagnostic Considerations

Late-stage morphea, systematized epidermal nevus, postinflammatory hyperpigmentation secondary to herpes zoster, and linear atrophoderma of Moulin may resemble idiopathic atrophoderma of Pasini and Pierini. Many consider idiopathic atrophoderma of Pasini and Pierini to be an atrophic stage of morphea.

Since the original description, much debate has occurred regarding whether idiopathic atrophoderma of Pasini and Pierini is a distinct entity or an atrophic, nonindurated variant of morphea, despite differences in the origin, development, and outcome of the lesions. The existence of a relationship between idiopathic atrophoderma of Pasini and Pierini and morphea is supported by the striking clinical and histological similarities seen at sites of regressing plaques of morphea. Morphea characteristically begins as a discrete circumscribed, erythematous-to-sclerotic plaque, often with a white center and characteristic peripheral lilac rim. Idiopathic atrophoderma of Pasini and Pierini lacks sclerosis, and lesions commonly coalesce over time. There may be a moth-eaten appearance that is not consistent with morphea.

From a distance, the skin depression in lichen sclerosis et atrophicus, anetoderma, or resolving panniculitis may resemble a stage of idiopathic atrophoderma of Pasini and Pierini, but these conditions lack the pigmentation seen in persons with idiopathic atrophoderma of Pasini and Pierini. Anetoderma, also a dermal atrophic process, is easily differentiated by palpation and, with histology studies, loss of dermal elastic fibers.

Typical lesions of morphea and idiopathic atrophoderma of Pasini and Pierini may appear in different or adjacent areas on the same individual. These conditions may occur simultaneously or one may precede the other. This observation may support the hypothesis that idiopathic atrophoderma of Pasini and Pierini and morphea are variations in response to the same abnormality.

The diagnosis of linear atrophoderma of Moulin requires that the acquired hyperpigmented linear atrophoderma follows Blaschko lines. However, a 2016 paper suggests that this entity may simply represent a linear variant of atrophoderma of Pasini and Pierini. [13]

Differential Diagnoses