CP-202 Safety and efectiveness of topical 10% n-acetylcysteine in 5% urea o/w emulsion for congenital lamellar ichtyosis and epidermolytic ichtyosis in children

Background Treatment of ichthyosis is based on disease severity, although a permanent cure may not yet be possible. Treatment options for ichthyosis include topical formulations (classically emollient creams, ointments, keratolytic agents and bath oils) and oral retinoids. Purpose To determine safety and efectiveness of topical 10% N-acetylcysteine (NAC) in 5% urea emulsion by two cases of congenital lamellar ichthyosis and four cases of epidermolytic ichthyosis. Material and methods Case 1 and 2: 9 and 12 years old patients with congenital lamellar ichtyosis, extended cutaneus xerosis with dark and medium-sized flakes at upper and lower limbs and sides. One of them with moderate affectation at skin folds. Cases 3, 4, 5 y 6: 16, 17 y 18 months and 10 years old patients with epidermolytic ichthyosis (1, 7 and 9 exon mutation of KRT10 gene). Case 3 presents denudated areas at gluteus, trunk and lower limbs, with subsequent healing and keratotic appearance. Case 4 presents big denudated areas at thorax and white keratotic appearance at palm and at sole, progressing to totally body erosion and completely denudation. Case 5 and 6 presents hyperkeratotic lesion at upper and lower limbs. All patients have been previously treated with emollient creams and ointments. Results It was decided to apply topical 10% NAC in 5% urea emulsion at one limb two times a day for 6 weeks and compare its efficacy to that of an emollient prescription of vaseline, paraffin and glycerol. For sensitive areas (palm, sole and face) the concentration was modified to 5% NAC in 5% urea emulsion presenting better tolerance. The first four cases presented clinical improvement and reduction of the hyperkeratotic lesion without side effects, therefore was treated all the body surface area. Case 5 interrupted the treatment after a month due to a lack of answer and started oral acitretin treatment. Case 6 stopped the treatment because of the emulsion’s unpleasant smell. Conclusion Topical 10% N- acetylcysteine in 5% urea emulsion seems to be an effective and safety option to reduce hyperkeratotic lesion when emollient creams and ointments aren’t effective and before use systemic treatments which could increase the risk of side effects. References and/or Acknowledgements N/A No conflict of interest.