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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]
  1. *Bourgogne area excluded (3 patients).