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Atrophia maculosa varioliformis cutis in Adult
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Atrophia maculosa varioliformis cutis in Adult

Contributors: Boya Abudu MD, MPH, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Atrophia maculosa varioliformis cutis (AMVC) is an extremely rare idiopathic atrophic disorder characterized by the spontaneous appearance of scar-like depressions without preceding inflammation or trauma.

The onset of disease usually occurs during young adulthood, but may be seen in early childhood, and is slowly progressive. Lesions are asymptomatic and appear as well-demarcated round, linear, or varioliform (smallpox-like) scars that vary in size from 0.2-2 cm. The cheeks are affected most frequently. The temples and extrafacial sites such as the neck, back, and upper extremities can be involved.

The majority of cases occur spontaneously, although a few familial cases have been reported. A minority of patients have reported very mild acne at the time of AMVC onset. AMVC has been reported in one patient with extrahepatic biliary atresia, one with pachydermodactyly, and one with keratosis pilaris atrophicans. These associations may be coincidental.

Codes

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

SNOMEDCT:
238841008 – Atrophic scarring of cheeks

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Last Reviewed:06/14/2019
Last Updated:06/14/2019
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Atrophia maculosa varioliformis cutis in Adult
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Atrophia maculosa varioliformis cutis : Forehead, Cheeks
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