Atrophia maculosa varioliformis cutis with extrahepatic biliary atresia

A Case of Atrophia Maculosa Varioliformis Cutis

“…The etiology of AMVC is unknown, although a familial occurrence has previously been reported 2,3 , which suggests that AMVC is an inherited disorder rather than a response to an environmental insult. Cases associated with extrahepatic biliary atresia 4 and pachydermodactyly 5 have been described, but these associations may be coincidental. The diagnosis of AMVC is mainly made on a clinical basis, with patients having facial atrophic lesions with sharply defined edges and an absence of erythema, induration, pigmentary changes or a honeycombed pattern.…”

A Case of Atrophia Maculosa Varioliformis Cutis

“…In the literature, associated diseases include extrahepatic biliary atresia 8 , pachydermodactyly 9 and keratosis pilaris atrophicans 10 . However, there were no associated diseases for our case.…”

Familial atrophia maculosa varioliformis cutis: case report and pedigree analysis

“…Only 19 cases have been reported since 1918. We reviewed these previous reports and summarized the clinical and pathological features of AMVC 1–12 . There are nine males and 10 females among the 19 patients.…”