Table 5 Estimated prevalence of the different clinical forms of ichthyosis
From: Prevalence of inherited ichthyosis in France: a study using capture-recapture method
Clinical forms of ichthyosis | Number of recorded patients (percentage)* | Estimated prevalence per million people (95% confidence interval) |
|---|---|---|
All clinical forms | 116 | 13.3 [10.9 – 17.6] |
Non syndromic ichthyoses (ichthyosis vulgaris excluded) | 99 (85.3) | 11.3 [9.3 – 15.0] |
ARCI | 61 (52.6) | 7.0 [5.7 – 9.2] |
Major variants: | ||
Lamellar ichthyosis | 39 (33.6) | 4.5 [3.7 – 5.9] |
Congenital ichthyosiform erythroderma | 17 (14.7) | 1.9 [1.6 – 2.6] |
Minor variants: | ||
Bathing suit ichthyosis | 4 (3.4) | 0.46 [0.38 – 0.61] |
Self healing collodion baby | 1 (0.9) | 0.11 [0.09 – 0.15] |
X-linked ichthyosis | 21 (18.1) | 2.4 [2.0 – 3.2] |
Keratinopathic ichthyosis | 10 (8.6) | 1.1 [0.9 – 1.5] |
Other forms | 7 (6.0) | 0.80 [0.66 – 1.06] |
Erythrokeratodermia variabilis | 4 (3.4) | 0.46 [0.38 – 0.61] |
Peeling Skin Disease | 1 (0.9) | 0.11 [0.09 – 0.15] |
KLICK syndrome | 2 (1.7) | 0.23 [0.19 – 0.30] |
Syndromic ichthyoses | 17 (14.7) | 1.9 [1.6 – 2.6] |
Syndromic X-linked ichthyosis | 1 (0.9) | 0.11 [0.09 – 0.15] |
Conradi – Hünermann – Happle syndrome | 2 (1.7) | 0.23 [0.19 – 0.30] |
Netherton syndrome | 7 (6.0) | 0.80 [0.66 – 1.06] |
Trichothiodystrophy | 2 (1.7) | 0.23 [0.19 – 0.30] |
Sjögren – Larsson syndrome | 1 (0.9) | 0.11 [0.09 – 0.15] |
KID syndrome | 3 (2.6) | 0.34 [0.28 – 0.45] |
Neutral lipid storage disease | 1 (0.9) | 0.11 [0.09 – 0.15] |