Beirana PA, Ortiz ÁM

Language: Spanish
References: 21
Page: 39-49
PDF size: 207.06 Kb.

ABSTRACT

The term ichthyosis is use to describe a cornification disorder. The congenital Ichthyosis are: 1) Ichthyosis vulgaris (IV) 2) X-linked Ichthyosis (ILX) 3) recessive ichthyoses a) lamellar Ichthyosis (IL) and b) non-bullous congenital ichthyosiform erythroderma (IECnoB). The two first types are the most common. 1) IV is due by a filaggrin disorder, the manifestations appear in the first year of life, with fine scaling, on the extensor surfaces of extremities and trunk, while the face and flexures are spared. Extracutaneous manifestations are absent, and its associated to atopy in and 50 percent. 2) ILX : is due to the absence of esteroid sulphatase enzyme (SE). Presents generalized, polygonal and dark brown scales, on the extensor surfaces of extremities, the most cases improved during the summer. The extracutaneous manifestations are corneal opacities and cryptorchidism. The direct biochemical diagnoses is made by determining the SE serum deficiency in the mother and the patient. The «collodion baby» term is use to describe an initial presentation of ichthyosis like LI or EICnoB; this refers to a collodion-like membrane that covers the baby at birth its associated to severe eclabium and ectropion. 3) congenital recessive ichthyoses a) IL: begins in the first month of life with generalized scale, large, dark, polygonal, some fissures, palmoplantar keratoderma and alopecia can be present, without extracutaneous manifestations; last throughout life. B) EICnoB: the patient borns like collodion baby and after shedding the membrane the baby has intense erythema with finer and white generalized scales without blister formation. The palms and soles are hyperkeratotic, hypoplasia of nasal and auricular cartilage and scarring alopecia may be seen. There is not a curative treatment but we can use some treatments like: alpha-hydroxy acids, salicylic acid, propylene glycol, topical and systemic retinoids.

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