Familial atrophia maculosa varioliformis cutis

Atrophia maculosa varioliformis cutis (AVMC) was first described by Heidingsfeld in 1918, as a rarely reported form of idiopathic macular atrophy on the cheek. 1 It is characterized, clinically, by shallow, sharply demarcated depressions in various shapes. Extrahepatic biliary atresia 2 and pachydermodactyly 3 have been the only conditions associated with AMVC reported in the past 80 years. Although keratosis pilaris is a common skin disorder, it is related to other idiopathic atrophic conditions considered in the differential diagnosis of AMVC, namely keratosis pilaris atrophicans. 4 However, the two associations may be coincidental. We observed a patient with keratosis pilaris, and her brother and an unrelated young man, whose findings led to a diagnosis of AMVC.