(Classic lamellar Ichthyosis, Congenital lamellar Ichthyosis , LI)

Very rare form of congenital ichthyosis. Autosomal recessive transmission. In 30% of cases, the mutation concerns the TGM1 (14q11) gene that codes for transglutaminase1 in keratinocytes. In general, clinical picture of collodion baby at birth, more rarely  of ichthyosiform erythroderma. After the loss of the 'collodion' film, the skin becomes more or less erythematous and covered with scals of variable color and size. Often: ectropion, alopecia, eclabion, intolerance to heat. Sometimes intense pruritus.

Anesthetic implications: 

difficulties for peripheral venous access. Difficulties to secure catheters and tracheal intubation tubes as hyperkeratosis reduces the stickiness of dressings. Risk of hypo - and hyperthermia. Eye protection (ectropion). Risk of difficult manual ventilation or intubation. Malnutrition is common (caloric loss).

References : 

• Chiavérini C. 
  Ichtyoses génétiques. 
  Ann Dermatol Vénéréol 2009 ; 136 : 923-34.
• Hedge HV, Annigeri VM, Pai VV. 
  Anesthetic challenges in lamellar ichtyosis. 
  Pediatr Anesth 2012; 22: 492-4.  
• Wu IC, Yao Q.
  Lamellar ichtyosis.
  JAMA Dermatology 2023; 159:210

Updated: February 2023