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Anetoderma is characterized by circumscribed oval macules with overlying wrinkled skin that is slightly depressed or bulges outwards. Skin biopsy shows a decrease of elastic dermal fibers. It may not be associated with an underlying disease (primary anetoderma) or may be related to many dermatoses (secondary anetoderma).

We report a 7-year-old girl who presented at birth with yellowish brown papules on the upper trunk, neck and head, which within days evolved to yellowish orange papules. A skin biopsy was carried out and the presence of an histiocytic infiltrate with foam cells and Touton cells in the dermis that were CD68+, factor XIII+ and S-100−, confirmed the diagnosis of Juvenile Xanthogranuloma (JXG). After 4 years the lesions began to evolve to asymptomatic oval and round atrophic skin areas. Histopathologic evaluation showed decrease of elastic fibers in the dermis, diagnostic of anetoderma.

The mechanisms of anetoderma are unknown. Although many different dermatoses have been associated with anetoderma we have only found two reported cases of anetoderma and JXG.