Question

A Child is born, covered with a thick membranous coat, what could be the possible diagnosis ?

• A. Lamellar ichthyosis
• B. X-linked ichthyosis
• C. Ichthyosis Vulgaris
• D. Ichthyosis acquista

Answer 1

The Correct Option is A

The condition where a child is born covered with a thick membranous coat is known as a collodion membrane. This is a characteristic feature of Lamellar ichthyosis.

Lamellar ichthyosis is a rare genetic disorder that falls under the broad category of autosomal recessive congenital ichthyoses (ARCI). It is characterized by the abnormal development of the outer layer of the skin, leading to the formation of a thick, plate-like scale over the skin surface.

The presence of a collodion membrane at birth suggests that the skin fails to develop its normal barrier function, which is a hallmark of Lamellar ichthyosis. After birth, the collodion membrane typically dries and peels off, revealing the underlying ichthyosic skin, which might be dry, scaly, and can have a shiny appearance.

In contrast, the other options listed have different characteristics:

• X-linked ichthyosis: Usually manifests later with brownish scales, particularly affecting males due to its genetic linkage.
• Ichthyosis Vulgaris: Characterized by mild, fine scaling and is mostly noticed after birth, not associated with a membrane.
• Ichthyosis acquista: Also known as acquired ichthyosis, it presents due to systemic illnesses rather than congenital causes. It is also not present at birth with a membranous covering.

Based on this information, the clear diagnosis given the presence of a thick membranous coat at birth is Lamellar ichthyosis.