Familial atrophia maculosa varioliformis cutis: An ultrastructural study

Atrophia maculosa varioliformis cutis is a rare and distinctive form of idi opathic facial macular noninflammatory atrophy that may rarely be observed in members of the same family. We describe two brothers, ages 14 and 16 yea rs, with spontaneously appearing, asymptomatic, varioliform and linear atro phic lesions. Their past medical history was positive for varicella occurri ng in childhood without residual facial scarring. Routine laboratory invest igations and screening for circulating autoantibodies were negative. Both p atients were concordant for HLA A2 and DQ4.1. Routine and ultrastructural h istologic examination of a punch biopsy specimen showed the presence of sca rce, small, fragmented elastic fibers and compact collagen bundles associat ed with hypertrophic fibroblasts in the dermis, Our patients remained clini cally stable, untreated, over a 2-year follow-up period. No long-term follo w-up data have previously been reported.