Ichthyin Related Autosomal Recessive Congenital Ichthyosis (ARCII) is one of the several forms of hereditary ichthyosis, a dermatological condition characterized by dry, thickened, scaly, or flaky skin. Compared to other ichthyotic conditions, such as Harlequin ichthyosis, ARCII is a less severe condition, and is characterized by non-bullous congenital ichthyoisform erythroderma (NCIE). Children affected with this condition are often born encased in a taut, shiny, translucent or opaque membrane (collodion membrane), which heals by itself in a few days or weeks. Once the membrane is shed, the underlying skin appears red with generalized fine whitish scaling and larger brownish scaling on the buttocks, necks, and legs. Other features seen in affected patients include palmoplantar keratoderma, painful fissures and digital contractures. Some patients with ARCII may show a lamellar form of ichtyosis.
ARCI can be easily diagnosed in neonates by its physical manifestation. Molecular genetic testing, however, is needed to confirm the Ichthyin related form of the condition. Since the condition is self healing in most cases, management in neonates consists of preventing infection, and treating any if they occur. Topical creams and ointments are used to keep the skin hydrated. Since corneal drying is a common complication, patients may require corneal lubrication with artificial tears or ointments. Patients may need regular physical examinations to detect any infection or skin involvement.