A case of lamellar ichthyosis = A case of lamellar ichthyosis

다국어 초록 (Multilingual Abstract)

Lamellar ichthyosis is an autosomal recessive disorder that is apparent at birth and is present throughout life. The newborn is born encased in a collodion membrane that sheds within 10-14 days, resulting in generalized scaling with variable redness of the skin. Although lamellar ichthyosis is not life threatening, it is quite disfiguring and causes considerable psychological stress to affected patients. A 16-year-old man presented with diffuse thick scales since childhood. He had no family history or past history of the disease. On examination, ectropion and large brownish plate-like scales on the whole body were observed. His palms and soles showed hyperkeratosis with some fissures. He had a history of allergic rhinitis and a mild to moderate conductive type of hearing loss. A skin biopsy from his left thigh showed epidermal acanthosis with a markedly thickened compact stratum corneum. He was treated with emollients containing hyaluronic acid and petrolatum. After treatment, his symptoms improved significantly. As far we know, there is no case report of lamellar ichthyosis in Korean literature. Therefore, we report a rare case of lamellar ichthyosis.