Autosomal dominant ichthyosis vulgaris

Published on 05/03/2015 by admin

Filed under Dermatology

Last modified 22/04/2025

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Autosomal dominant
ichthyosis vulgaris

DDx Ref       91220

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Ichthyosis vulgaris can have a cracked pavement appearance, and can be inflamed, especially in winter.

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The scales are stuck on and will slough off and renew again. Emollients with lactic acid can help alleviate the dry appearance of ichthyosis vulgaris.

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Large, dark, platelike scales characterized this ichthyosis, which is X-linked ichthyosis. Ichthyosis vulgaris can look like this, although usually the scales are not as dark.

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Dried scaling skin of ichthyosis. X-linked ichthyosis vulgaris is more evident in winter. The thick scale may disappear in the summer.

DESCRIPTION

A disorder of keratinization characterized by dry, rectangular scales resembling a cracked pavement; these scales appear most prominently on the extensor extremities. Associated with the atopic diathesis in 50% of cases.

HISTORY

• Onset in early to middle childhood. • Condition may improve with age or may persist throughout life. • Scales more noticeable and often pruritic in the winter, when humidity is low. • Autosomal dominant; 1 in 300 have the disorder. • May be confused clinically with X-linked ichthyosis. • Differential diagnosis: dry skin, acquired ichthyosis as a manifestation of HIV, malignancy, drugs, autoimmune disorders, X-linked ichthyosis.

PHYSICAL FINDINGS

• Findings often mild. • Dry, small, rectangular scales appear on the extensor extremities. Lower extremities, particularly the anterior shins, often more noticeably affected. • Affected skin has the appearance of cracked pavement or fish scales. • Characteristically spares the flexor surfaces. • Usually asymptomatic but may become pruritic or chapped in winter. • Palmar creases may be accentuated. • Keratosis pilaris may also be present. • Scaling rarely involves entire cutaneous surface. • Scaling of the skin results from the retention of scale rather than increased proliferation. • No tests are routinely indicated, unless there is a question of X-linked ichthyosis. • Skin biopsy is rarely performed; when performed, it shows in a subset of patients a decreased or an absent granular layer.

TREATMENT

• Often improves with age; infrequently, it resolves completely. • Increased environmental humidity and warmth often result in resolution or improvement. • Regular application of moisturizing cream or lotion decreases pruritus and improves skin appearance. • Optimal time for applying moisturizer is immediately after bathing and hydrating the skin. • Emollients containing lactic acid, urea, or alphahydroxy acids help treat severe dryness and scaling. • Ammonium lactate 12% (Lac-Hydrin, Amlactin) lotion or cream very effective when applied daily.