Dermatology - Dominant Ichthyosis Vulgaris
Dominant ichthyosis is a hereditary condition that follows an autosomal dominant pattern, meaning it is passed down from one generation to the next. It typically starts between the ages of 3 and 12 months and is characterized by a generally moderate dryness of the skin, known as xerosis, with the most noticeable scaling occurring on the lower legs. Dominant ichthyosis frequently co-occurs with atopy, and dryness and itching are exacerbated during the winter season. A significant number of individuals experience cosmetic issues, especially in cases of severe hyperkeratosis. More than half of the patients also exhibit atopy. Abnormalities Xerosis, which refers to dry skin, can present with fine, powdery scaling. However, it can also manifest as larger scales that are firmly attached and arranged in a fish-scale pattern. The condition involves widespread and scattered areas of the body, particularly the shins, arms, and back. It also affects the buttocks and outer thighs. However, it does not affect the armpits, inner elbows, and back of the knees. Typically, the face is unaffected, although the cheeks and forehead may sometimes be involved. Keratosis pilaris is a condition characterized by the presence of small, spiky, thickened bumps around hair follicles. These bumps have a normal skin color and can be seen in clusters or spread out across the body. They are most commonly found on the outer surfaces of the arms and legs, but can also appear on the cheeks during childhood. The hands and feet are often unaffected, but there is an increased prominence of palmoplantar marks (hyperlinear). The diagnosis is typically made based on clinical observations, with the presence of aberrant keratohyalin granules being visible under electron microscopy. The differential diagnosis include acquired ichthyoses as well as all other types of ichthyosis. Advise patients to maintain skin moisture by soaking in a bath and then using petrolatum. Urea-based lotions, which retain moisture in the outermost layer of the skin, are also beneficial. Propylene glycol, with a concentration of 44-60% in water, along with 6% salicylic acid in propylene glycol and alcohol under plastic occlusion (with caution with hypersalicism), α-hydroxy acids such as lactic acid or glycolic acid, and preparations containing urea (2-10%) have also been found to be beneficial. Intermittent therapy with isotretinoin and acitretin is highly beneficial for severe instances. However, it is crucial to closely monitor for any signs of toxicity.
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