Bazex-Dupre-Christopos Syndrome, Scar, Atrophoderma - DermAtlas

Contributor

Andrea Zaenglein, MD

Diagnosis

Bazex-Dupre-Christopos syndrome scar atrophoderma

Body Site

hand

Age

40 years

Pigmentation

light

Organization

grouped, clustered

Color

white/hypo

Morphology

depressed (atrophy, loss of soft tissue,scar)

Pattern

symmetric acral (centripetal - extremity predominant)

Comments

This family has multiple members with the typical changes of Basex-Dupre-Christol syndrome including follicular atrophoderma, congenital hypotrichosis, and early onset of basal cell carcinomas. The father, his 2 brothers and sister, and both of his children were affected. They have had multiple cysts on the face since birth, coarse slow growing hair, ice-pick scars on the tops of the hands, and basal cell carcinomas beginning at a young age. The disorder is presumed to be inherited as an X-linked dominant trait and maps to chromosome Xq24-q27.

Description

pitted follicular scars

Image Added

1/7/2007 10:04:52

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Other Resources

PubMed Medline Plus Medscape

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