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Atrophoderma in Child
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Atrophoderma in Child

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Contributors: Chris G. Adigun MD, Jeffrey D. Bernhard MD
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Synopsis

Atrophoderma, also known as idiopathic atrophoderma of Pasini and Pierini (IAPP), is a form of dermal atrophy that is thought to lie within the spectrum of morphea. The disorder is characterized by brown to gray oval, round, or irregularly shaped smooth atrophic depressions that fall below the level of the skin with a well-demarcated, sharply sloping border. This distinctive sloping border is often described as a "cliff drop" border, usually 2-8 mm deep, forming an inverted plateau. Lesions occur most often on the trunk, most commonly the lumbo-sacral region of the back.

Individual lesions are asymptomatic and may be single or multiple. Individual lesions range in size from a few millimeters to several centimeters but may coalesce to form a lesion that measures greater than 20 cm in diameter. The color change may contribute to an optical illusion that accentuates the depression. Initially, lesions are localized, before progressing to involve other areas over months to years before stabilizing. The condition occurs disproportionately in females, usually in the second and third decades. However, it has been reported in infancy and old age.

Whether IAPP is a nonsclerotic, atrophic variant of morphea or a distinct entity is still hotly debated. Linear atrophoderma of Moulin is a related condition that follows the lines of Blaschko. The etiology of IAPP is unknown, although it has been theorized that cells with an aberrant T-cell phenotype may be the cause of collagen fiber destruction. The role of Borrelia burgdorferi in the pathogenesis of this entity is highly controversial.

Codes

ICD10CM:
L90.9 – Atrophic disorder of skin, unspecified

SNOMEDCT:
399979006 – Atrophoderma

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Last Updated: 09/08/2017
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Atrophoderma in Child
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Atrophoderma : Atrophy, Primarily truncal, Scattered many
Clinical image of Atrophoderma
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