Familial Atrophia Maculosa Varioliformis Cutis: An Ultrastructural Study

: Atrophia maculosa varioliformis cutis is a rare and distinctive form of idiopathic facial macular noninflammatory atrophy that may rarely be observed in members of the same family. We describe two brothers, ages 14 and 16 years, with spontaneously appearing, asymptomatic, varioliform and linear at...

Full description

Saved in:
Bibliographic Details
Published in:Pediatric dermatology 2001-05, Vol.18 (3), p.230-233
Main Authors: Dall'Oglio, Federica, Nasca, Maria Rita, Taparelli, Francesca, Bacchelli, Barbara, Micali, Giuseppe
Format: Article
Language:English
Subjects:
Adolescent
Atrophy
Biological and medical sciences
Biopsy, Needle
Dermatology
Facial Dermatoses - genetics
Facial Dermatoses - pathology
Humans
Male
Medical sciences
Skin - pathology
Skin - ultrastructure
Skin involvement in other diseases. Miscellaneous. General aspects
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:: Atrophia maculosa varioliformis cutis is a rare and distinctive form of idiopathic facial macular noninflammatory atrophy that may rarely be observed in members of the same family. We describe two brothers, ages 14 and 16 years, with spontaneously appearing, asymptomatic, varioliform and linear atrophic lesions. Their past medical history was positive for varicella occurring in childhood without residual facial scarring. Routine laboratory investigations and screening for circulating autoantibodies were negative. Both patients were concordant for HLA A2 and DQ4.1. Routine and ultrastructural histologic examination of a punch biopsy specimen showed the presence of scarce, small, fragmented elastic fibers and compact collagen bundles associated with hypertrophic fibroblasts in the dermis. Our patients remained clinically stable, untreated, over a 2‐year follow‐up period. No long‐term follow‐up data have previously been reported.
ISSN:0736-8046
1525-1470
DOI:10.1046/j.1525-1470.2001.018003230.x