Erythrodermia Ichtyosis Formis Congenital - Case Report

Abstract

Non-bullous congenital ichthyosi-form erythroderma is an autosomal recessive congenital keratinization disorder. Materials and Methods: We present the only one registered case of congenital non-bullous ichthyosiform erythroderma in North Macedonia. Our patient now is a 29-year old man from Gostivar. He was born with low birth weight and a low Apgar score (5/7). After birth, the skin was so cracked, that there were bleeding in certain places. Furthermore, the entire body, especially the head, was covered with yellow-green scales of varying size, which were firmly attached to the substrate. He does not provide information about a similar or same illness in the family. The ophthalmologist detected ectropion on the eyes, congenital chronic dacryocystitis, and conjunctivitis. His nails are very fragile. Often, pruritus occurs due to dryness of the skin. Meanwhile, flexion contractures on the 4th and 5th fingers of the right arm are noticed. In addition, they are with reduced function. Little is known about the oral manifestations of this disorder. Conclusion: The prognosis is variable. The disease has a strong impact on the quality of life due to altered physical appearance, problematic symptoms and treatment restrictions Key words: Congenital; Recessive; Non-Bullous Congenital Ichthyosiform Erythroderma; Oral Manifestations of Itchthyosi