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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

• Non-bullous congenital ichthyosiform erythroderma (n-CIE) is the primary differential diagnosis. In n-CIE, the scale is thinner, but erythroderma is more pronounced.
• Sjögren-Larsson syndrome
• Chanarin-Dorfman syndrome (neutral lipid storage disease) – Has lamellar ichthyosis at birth, myopathy, and vacuolated leukocytes.
• Tay syndrome (see trichothiodystrophy) – Has generalized ichthyosis, intellectual disability, and a hair shaft abnormality.
• Netherton syndrome – Presents with generalized scaling and erythroderma along with the hair shaft anomaly trichorrhexis invaginata. It is not associated with a collodion phenotype. Ichthyosis linearis circumflexa usually has a double-edge scale but is not seen before 2 years of age.

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