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.
Lefevre et al. (2004) studied 23 patients with congenital ichthyosis from 14 consanguineous families. Some of the families originated from Algeria (8) and Syria (1). Fourteen of the patients (60%) were born as 'collodion babies' except for the patients from four Algerian families who presented a clinical picture resembling nonbullous ichthyosiform erythroderma (NCIE). They presented with generalized ichthyosis with erythema, fine whitish scaling on the face and trunk, and larger brownish scaling on the neck, buttocks, and legs; the remainder of the patients had a lamellar-like phenotype. Patients with the NCIE phenotype showed hyperkeratosis, thickening of the stratum corneum and moderate acanthosis and parakeratosis, with a normal or slightly prominent granular layer and a mild dermal perivascular lymphocytic infiltrate, whereas patients with a lamellar phenotype had significant orthohyperkeratosis, moderate acanthosis, a normal or reduced granular layer, and mild papillomatosis with dilatation of dermal capillaries. All the patients had palmoplantar keratoderma, often yellowish with fissures, and some clubbing of nails.
A survey performed by Bastaki et al. (1992) between 1985 and 1989 reported the overall incidence of genodermatosis in Kuwait Maternity Hospital to be 0.26 per 1000 livebirths. More specifically, the incidence of ichthyosis was 0.11 per 1000 livebirths in Kuwait during the over-mentioned period of five years.
[Bastaki L, Al-Awadi A, Naguib KK. Incidence of genodermatosis among the neonates in Kuwait Maternity Hospital: 1985 to 1989 survey report, Kuwait Medical Genetics Centre, 1992, Kuwait.]
[See: Algeria > Lefevre et al., 2004]