P33 A lichenoid eruption and severe eczema in association with IMAGe syndrome

Abstract

Abstract IMAGe syndrome is a rare genetic disorder. IMAGe is an acronym for; (1)Intrauterine restricted growthMetaphyseal dysplasiaCongenital adrenal hypoplasiaGenitourinary abnormalities in males (cryptorchidism, micropenis, and hypospadias) Diagnosis is established by molecular genetic testing with heterozygous CDKN1C pathogenic mutation or a proband with suggestive findings in the PCNA (proliferating cell nuclear antigen)- binding domain of the maternally expressed allele. Additional features include characteristic facies (frontal bossing), developmental delay, immunodeficiency, osteoporosis and scoliosis. Severe, early onset eczema has been described in association with this syndrome. (2) We present the case of a 15-year-old boy with proven IMAGe syndrome, longstanding history of atopic dermatitis, and new onset lichenoid eruption on the forearms.